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Encyclopedia > Lupus erythematosus
Lupus Erythematosus
Classification & external resources
ICD-10 L93., M32.
ICD-9 710.0
OMIM 152700
DiseasesDB 12782
MedlinePlus 000435
eMedicine med/2228  emerg/564
MeSH D008180

Systemic Lupus Erythematosus (SLE or lupus) is a chronic autoimmune disease that can be fatal, though with recent medical advances, fatalities are becoming increasingly rare. As with other autoimmune diseases, the immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys and nervous system. The course of the disease is unpredictable, with periods of illness (called flares) alternating with remission. Lupus can occur at any age, and is most common in women, particularly of non-European descent.[1] Lupus is treatable symptomatically, mainly with corticosteroids and immunosuppressants, though there is currently no cure. However, many people with Lupus lead long and substantial lives. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // L00-L99 - Diseases of the skin and subcutaneous tissue (L00-L08) Infections of the skin and subcutaneous tissue (L00) Staphylococcal scalded skin syndrome (L01) Impetigo (L02) Cutaneous abscess, furuncle and carbuncle (L03) Cellulitis (L04) Acute lymphadenitis (L05) Pilonidal cyst (L08) Other local infections of skin and subcutaneous tissue (L081) Erythrasma... // M00-M99 - Diseases of the musculoskeletal system and connective tissue (M00-M25) Arthropathies (M00-M03) Infectious arthropathies (M00) Pyogenic arthritis (M01) Direct infections of joint in infectious and parasitic diseases classified elsewhere (M02) Reactive arthropathies (M023) Reiters disease (M03) Postinfective and reactive arthropathies in diseases classified elsewhere (M05-M14... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ... Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ... A scanning electron microscope image of a single neutrophil (yellow), engulfing anthrax bacteria (orange). ... The heart and lungs, from an older edition of Grays Anatomy. ... For other uses, see Joint (disambiguation). ... For other uses, see Skin (disambiguation). ... Human respiratory system The lungs flank the heart and great vessels in the chest cavity. ... f you all The blood vessels are part of the circulatory system and function to transport blood throughout the body. ... For the bird, see Liver bird. ... The kidneys are the organs that filter wastes (such as urea) from the blood and excrete them, along with water, as urine. ... The Human Nervous System. ... European American is a term for an American of European descent, who are usually referred as White or Caucasian. ... Symptomatic treatment is any medical therapy of a disease that only affects its symptoms, not its cause, i. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Immunosuppression is the medical suppression of the immune system. ...

Contents

Classification

Lupus is a chronic autoimmune disease in which the body's own defense system attacks otherwise healthy tissue. Clinically, it can affect multiple organ systems including the heart, skin, joints, kidneys and nervous system. There are several types of lupus; generally when the word 'lupus' alone is used, it refers to the systemic lupus erythematosus or SLE as discussed in this article. Other types include: Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ...

  • Drug-induced lupus erythematosus, a drug-induced form of SLE; this type of lupus can occur equally for either gender
  • Lupus nephritis, an inflammation of the kidneys caused by SLE
  • Discoid lupus erythematosus, a skin disorder which causes a red, raised rash on the face, scalp or rest of the body, which occasionally (1-5%) develops into SLE[2]
  • Subacute cutaneous lupus erythematosus, which causes non-scarring skin lesions on patches of skin exposed to sunlight[3]
  • Neonatal lupus, a rare disease affecting babies born to women with SLE, Sjögren's syndrome or sometimes no autoimmune disorder. It is theorized that maternal antibodies attack the fetus, causing skin rash, liver problems, low blood counts (which gradually fade) and heart block leading to bradycardia.[3]

Drug-induced lupus erythematosus (DIL or DILE) is an autoimmune disorder, similar to systemic lupus erythematosus (SLE), which is induced by chronic use of certain drugs. ... Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. ... The kidneys are the organs that filter wastes (such as urea) from the blood and excrete them, along with water, as urine. ... A lesion is a non-specific term referring to abnormal tissue in the body. ... Sjögrens syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. ... Bradycardia, as applied to adult medicine, is defined as a resting heart rate of under 60 beats per minute, though it is seldom symptomatic until the rate drops below 50 beat/min. ...

Signs and symptoms

SLE is one of several diseases known as "the great imitators" [4] because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years. Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms (below), however, they are considered suggestive. An analogue medical thermometer showing the temperature of 38. ... Malaise is a feeling of general discomfort or uneasiness, an out of sorts feeling, often the first indication of an infection or other disease. ... This article does not cite any references or sources. ... Myalgia means muscle pain and is a symptom of many diseases and disorders. ... Exhaustion redirects here. ...


Common Symptoms Explained

Dermatological manifestations
As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic malar rash (or butterfly rash) associated with the disease. Patients may present with discoid lupus (thick, red scaly patches on the skin). Alopecia, mouth, nasal, and vaginal ulcers, and lesions on the skin are also possible manifestations.
Musculoskeletal manifestations
Patients most often seek medical attention for joint pain, with small joints of the hand and wrist usually affected, although any joint is at risk. The Lupus Foundation of America "estimates more than 90 percent will experience joint and/or muscle pain at some time during the course of their illness".[5] Unlike rheumatoid arthritis, lupus arthritis is less disabling and it usually does not cause severe destruction of the joints. Fewer than 10 percent of people with lupus arthritis will develop deformities of the hands and feet.[5]
Hematological manifestations
Anemia and iron deficiency may develop in as many as half of patients. Low platelet and white blood cell counts may be due to the disease or a side-effect of pharmacological treatment. Patients may have an association with antiphospholipid antibody syndrome (a thrombotic disorder) where autoantibodies to phospholipids are present in the patient's serum. Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid antibodies; the combination of such findings have earned the term "lupus anticoagulant positive". Another autoantibody finding in lupus is the anticardiolipin antibody which can cause a false positive test for syphilis.
Cardiac manifestations
Patients may present with inflammation of various parts of the heart, such as pericarditis, myocarditis, and endocarditis. The endocarditis of SLE is characteristically non-infective (Libman-Sacks endocarditis) and involves either the mitral valve or the tricuspid valve. Atherosclerosis also tends to occur more often and advance more rapidly in SLE patients than in the general population.[6][7][8]
Pulmonary manifestations
Lung and pleura inflammation can cause pleuritis, pleural effusion, lupus pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, pulmonary hemorrhage.
Hepatic involvement
See autoimmune hepatitis
Renal involvement
Painless hematuria or proteinuria may often be the only presenting renal symptom. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end stage renal failure. Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients.
Histologically, a hallmark of SLE is membranous glomerulonephritis with "wire loop" abnormalities.[9] This finding is due to immune complex deposition along the glomerular basement membrane leading to a typical granular appearance in immunofluorescence testing.
Neurological manifestations
About 10% of patients may present with seizures or psychosis. A third may test positive for abnormalities in the cerebrospinal fluid.
T-cell abnormalities
Abnormalities in T cell signaling are associated with SLE, including deficiency in CD45 phosphatase and increased expression of CD40 ligand.
Other rarer manifestations
lupus gastroenteritis, lupus pancreatitis, lupus cystitis, autoimmune inner ear disease, parasympathetic dysfunction, retinal vasculitis, and systemic vasculitis.

Other abnormalities include: Malar rash is also called as the butterfly rash. ... Alopecia is a set of disorders ranging from male and female pattern alopecia (alopecia androgenetica), to alopecia areata, which involves the loss of some of the hair from the head, alopecia totalis, which involves the loss of all head hair, to the most extreme form, alopecia universalis, which involves the... Mouth ulcer on the lower lip A mouth ulcer (from Latin ulcus) is the name for the appearance of an open sore inside the mouth caused by a break in the mucous membrane or the epithelium on the lips or surrounding the mouth. ... In medicine, arthralgia (literally: joint pain, from arthros = joint and -algia denoting pain) is the presence of painful joints in the absence of frank arthritis. ... Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... This article discusses the medical condition. ... A 250 ml bag of newly collected platelets. ... White Blood Cells redirects here. ... This article, image, template or category should belong in one or more categories. ... Lupus anticoagulant (also known as lupus antibody, LA, or lupus inhibitoris) is a medical phenomenon where autoantibodies bind to phospholipids and proteins associated with the cell membrane. ... ... Syphilis is a curable sexually transmitted disease caused by the Treponema pallidum spirochete. ... The heart and lungs, from an older edition of Grays Anatomy. ... Pericarditis is inflammation of the sac surrounding the heart, the pericardium. ... In medicine (cardiology), myocarditis is inflammation of the myocardium, the muscular part of the heart. ... Endocarditis is an inflammation of the inner layer of the heart, the endocardium. ... Libman-Sachs endocarditis is a form of nonbacterial endocarditis that is seen in systemic lupus erythematosus. ... The mitral valve (also known as the bicuspid valve or left atrioventricular valve), is a dual flap (bi = 2) valve in the heart that lies between the left atrium (LA) and the left ventricle (LV). ... The tricuspid valve is on the right side of the heart, between the right atrium and the right ventricle. ... Pleurisy, also known as pleuritis, is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs, which can cause painful respiration and other symptoms. ... Pleural effusion Chest x-ray of a pleural effusion. ... In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. ... Pulmonary embolism (PE) is blockage of the pulmonary artery (or one of its branches) by a blood clot, fat, air or clumped tumor cells. ... This page meets Wikipedias criteria for speedy deletion. ... In medicine (gastroenterology), hepatitis is any disease featuring inflammation of the liver. ... In medicine, hematuria (or haematuria) is the presence of blood in the urine. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ... Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. ... Renal failure is the condition in which the kidneys fail to function properly. ... Glomerulonephritis, also known as glomerular nephritis and abbreviated GN, is a primary or secondary immune-mediated renal disease characterized by inflammation of the glomeruli, or small blood vessels in the kidneys. ... The glomerular basement membrane is the basal laminal portion of the glomerulus which performs the actual filtration though the filtration slits between the podocytes , separating the blood on the inside from the urine on the outside. ... Immunofluorescence is the labeling of antibodies or antigens with fluorescent dyes. ... This article is about epileptic seizures. ... Psychosis is a generic psychiatric term for a mental state often described as involving a loss of contact with reality. Stedmans Medical Dictionary defines psychosis as a severe mental disorder, with or without organic damage, characterized by derangement of personality and loss of contact with reality and causing deterioration... Cerebrospinal fluid (CSF), Liquor cerebrospinalis, is a clear bodily fluid that occupies the subarachnoid space in the brain (the space between the skull and the cerebral cortex—more specifically, between the arachnoid and pia layers of the meninges). ... T cells are a subset of lymphocytes that play a large role in the immune response. ... In immunology, the CD45 antigen (CD stands for cluster of differentiation) is a protein which is a leucocyte common antigen, a type I transmembrane protein present on all differentiated hematopoietic cells except erythrocytes that assists in the activation of those cells (a form of co-stimulation). ... A phosphatase is an enzyme that dephosphorylates its substrate; i. ... See also Bacterial gastroenteritis and Diarrhea Gastroenteritis is a general term referring to inflammation or infection of the gastrointestinal tract, primarily the stomach and intestines. ... Pancreatitis is inflammation of the pancreas. ... Cystitis is the inflammation of the bladder. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Otitis interna (Internal otitis) is an inflammation of the inner ear. ... Anatomy and Physiology of the A.N.S. In contrast to the voluntary nervous system, the involuntary or autonomic nervous system is responsible for homeostasis, maintaining a relatively constant internal environment by controlling such involuntary functions as digestion, respiration, and metabolism, and by modulating blood pressure. ... In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels due to leukocyte migration and resultant damage. ... In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels due to leukocyte migration and resultant damage. ...

  • Increased expression of FcεRIγ, which replaces the sometimes deficient TCR ζ chain
  • Increased and sustained calcium levels in T cells
  • Moderate increase of inositol triphosphate
  • Reduction in PKC phosphorylation
  • Reduction in Ras-MAP kinase signaling
  • Deficiencies in protein kinase A I activity

FcεRI, or Fc epsilon RI, is the high-affinity receptor for immunoglobulin E (IgE), an antibody isotype involved in allergy and (arguably) resistance to parasites. ... T cells are a subset of lymphocytes that play a large role in the immune response. ... Inositol triphosphate or inositol 1,4,5-triphosphate (also commonly known as triphosphoinositol; abbreviated InsP3 or IP3), together with diacylglycerol, is a second messenger molecule used in signal transduction in biological cells. ... A protein kinase is an enzyme that can transfer a phosphate group from a donor molecule (usually ATP) to an amino acid residue of a protein. ... In cell biology, mitogen-activated protein kinases (MAPKs) are serine/threonine-specific protein kinases that respond to extracellular stimuli and regulate various cellular activities, such as gene expression, mitosis, differentiation, and cell survival/apoptosis. ... A protein kinase is an enzyme that modifies other proteins by chemically adding phosphate groups to them (phosphorylation). ...

Causes

Despite the dramatic rise in Lupus research in recent years, the exact cause of the disease remains unknown. Indeed, consensus is still lacking on whether Lupus is a single condition or a group of related diseases. SLE is a chronic inflammatory disease believed to be a type III hypersensitivity response with potential type II involvement,[10] characterised by the body's production of antibodies against the nuclear components of its own cells. There are three mechanisms by which lupus is thought to develop: genetic predisposition, environmental triggers and drug reaction (drug-induced lupus). An abscess on the skin, showing the redness and swelling characteristic of inflammation. ... Hypersensitivity refers to undesirable (damaging, discomfort-producing and sometimes fatal) reactions produced by the normal immune system. ... Hypersensitivity refers to undesirable (damaging, discomfort-producing and sometimes fatal) reactions produced by the normal immune system. ...

Genetics
The first mechanism may arise genetically. Research indicates that SLE may have a genetic link. Lupus does run in families, but no single "lupus gene" has yet been identified. Instead, multiple genes appear to influence a person's chance of lupus developing when triggered by environmental factors. The most important genes are located on chromosome 6, where mutations may occur randomly (de novo) or be inherited. Additionally, people with SLE have an altered RUNX-1 binding site, which may be either cause or contributor (or both) to the condition. Altered binding sites for RUNX-1 have also been found in people with psoriasis and rheumatoid arthritis.
Environmental triggers
The second mechanism may be due to environmental factors. These factors may not only exacerbate existing lupus conditions, but also trigger the initial onset. They include certain medications (such as some antidepressants and antibiotics), extreme stress, exposure to sunlight, hormones, and infections. Some researchers have sought to find a connection between certain infectious agents (viruses and bacteria), but no pathogen can be consistently linked to the disease. UV radiation has been shown to trigger the photosensitive lupus rash, but some evidence also suggests that UV light is capable of altering the structure of the DNA, leading to the creation of autoantibodies. Some researchers have found that women with silicone gel-filled breast implants have produced antibodies to their own collagen, but it is not known how often these antibodies occur in the general population and there is no data that show these antibodies cause connective tissue diseases such as lupus.
Drug reactions
Drug-induced lupus erythematosus is a reversible condition that usually occurs in patients being treated for a long-term illness. Drug-induced lupus mimics systemic lupus. However, symptoms of drug-induced lupus generally disappear once a patient is taken off the medication which triggered the episode. There are about 400 medications currently in use that can cause this condition, though the most common drugs are procainamide, hydralazine and quinidine.
Non-SLE forms of lupus
Discoid (cutaneous) lupus is limited to skin symptoms and is diagnosed via biopsy of skin rash on the face, neck or scalp. Often an anti-nuclear antibody (ANA) test for discoid patients is negative or a low-titre positive. About 10% of discoid lupus patients eventually develop SLE.
Clearance deficiency
Clearance deficiency
Clearance deficiency

The exact mechanisms for the development of systemic lupus erythematosus (SLE) are still unclear since the pathogenesis is a multifactorial event. Beside discussed causations, impaired clearance of dying cells is a potential pathway for the development of this systemic autoimmune disease. This includes deficient phagocytic activity, scant serum components in addition to increased apoptosis. This article is about the general scientific term. ... Chromosome 6 is one of the 23 pairs of chromosomes in humans. ... Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... Prozac, a selective serotonin reuptake inhibitor (SSRI) Serotonin-norepinephrine reuptake inhibitor, Venlafaxine An antidepressant, is a psychiatric medication or other substance (nutrient or herb) used for alleviating depression or dysthymia (milder depression). ... Staphylococcus aureus - Antibiotics test plate. ... This article is about biological infectious particles. ... Phyla Actinobacteria Aquificae Chlamydiae Bacteroidetes/Chlorobi Chloroflexi Chrysiogenetes Cyanobacteria Deferribacteres Deinococcus-Thermus Dictyoglomi Fibrobacteres/Acidobacteria Firmicutes Fusobacteria Gemmatimonadetes Lentisphaerae Nitrospirae Planctomycetes Proteobacteria Spirochaetes Thermodesulfobacteria Thermomicrobia Thermotogae Verrucomicrobia Bacteria (singular: bacterium) are unicellular microorganisms. ... Silicones (more accurately called polymerized siloxanes or polysiloxanes) are inorganic-organic polymers with the chemical formula [R2SiO]n, where R = organic groups such as methyl, ethyl, and phenyl. ... A breast implant is a prosthesis used to enlarge the size of a womans breasts (known as breast augmentation, breast enlargement, mammoplasty enlargement, augmentation mammoplasty or the common slang term boob job) for cosmetic reasons; to reconstruct the breast (e. ... Tropocollagen triple helix. ... A connective tissue disease is any disease that has the connective tissues of the body as a primary target of pathology. ... Drug-induced lupus erythematosus (DIL or DILE) is an autoimmune disorder, similar to systemic lupus erythematosus (SLE), which is induced by chronic use of certain drugs. ... Procainamide (trade name Pronestyl®) is a pharmaceutical antiarrhythmic agent used for the medical treatment of cardiac arrhythmias, classified by the Vaughan Williams classification system as class Ia. ... Hydralazine hydrochloride (1 -hydrazinophthalazine monohydrochloride; Apresoline®) is a medication used to treat high blood pressure. ... Quinidine is a pharmaceutical agent that acts as a class I antiarrhythmic agent in the heart. ... Anti-nuclear antibodies (ANAs, also known as anti-nuclear factor or ANF) are detected in a large group of autoimmune disorders. ... Image File history File links Metadata No higher resolution available. ... Image File history File links Metadata No higher resolution available. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... A section of mouse liver showing an apoptotic cell indicated by an arrow Apoptosis (pronounced apo tō sis) is a process of suicide by a cell in a multicellular organism. ...


Monocytes isolated from whole blood of SLE patients show reduced expression of CD44 surface molecules involved in the uptake of apoptotic cells. Most of the monocytes and tingible body macrophages (TBM), which are found in the germinal centres of lymph nodes, even show a definitely different morphology in patients with SLE. They are smaller or scarce and die earlier. Serum components like complement factors, CRP and some glycoproteins are furthermore decisively important for an efficiently operating phagocytosis. In patients these components are often missing, diminished or inefficient. MONOCYTES: Plural of monocyte. ... Red blood cells (erythrocytes) are present in the blood and help carry oxygen to the rest of the cells in the body Blood is a circulating tissue composed of fluid plasma and cells (red blood cells, white blood cells, platelets). ... Germinal centres (GC) are an important part of the humoral immune response. ... Lymph nodes are components of the lymphatic system. ... A complement protein attacking an invader. ... C-reactive protein (CRP) is a plasma protein, an acute phase protein produced by the liver. ... A glycoprotein is a macromolecule composed of a protein and a carbohydrate (a sugar). ...


The clearance of early apoptotic cells is an important function in multicellular organisms. It leads to a progression of the apoptosis process and finally to secondary necrosis of the cells, if this ability is disturbed. Necrotic cells release nuclear fragments as potential autoantigens as well as internal danger signals, inducing maturation of dendritic cells (DC), since they have lost their membranes integrity. Increased appearance of apoptotic cells also is simulating inefficient clearance. That leads to maturation of DC and also to the presentation of intracellular antigens of late apoptotic or secondary necrotic cells, via MHC molecules. Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens derived from late apoptotic and secondary necrotic cells. B and T cell tolerance for apoptotic cells is abrogated and the lymphocytes get activated by these autoantigens; inflammation and the production of autoantibodies by plasma cells is initiated. A clearance deficiency in the skin for apoptotic cells has also been observed in patients with cutaneous lupus erythematosus (CLE). Necrosis (in Greek Νεκρός = Dead) is the name given to accidental death of cells and living tissue. ... Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ... Dendritic cells (DC) are immune cells and form part of the mammal immune system. ... Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. ... T cells are a subset of lymphocytes that play a large role in the immune response. ... A lymphocyte is a type of white blood cell involved in the human bodys immune system. ... An abscess on the skin, showing the redness and swelling characteristic of inflammation. ... Plasma cells are B lymphocytes that secrete immunoglobulins (antibodies). ...

germinal centres
germinal centres

Accumulation in germinal centres (GC) Image File history File links Metadata Size of this preview: 372 × 598 pixelsFull resolution (478 × 769 pixels, file size: 384 KB, MIME type: image/jpeg) File historyClick on a date/time to view the file as it appeared at that time. ... Image File history File links Metadata Size of this preview: 372 × 598 pixelsFull resolution (478 × 769 pixels, file size: 384 KB, MIME type: image/jpeg) File historyClick on a date/time to view the file as it appeared at that time. ...


In healthy conditions apoptotic lymphocytes are removed in germinal centres by specialised phagocytes, the tingible body macrophages (TBM); that’s why no free apoptotic and potential autoantigenic material can be seen. In some patients with SLE accumulation of apoptotic debris can be observed in GC, because of an ineffective clearance of apoptotic cells. In close proximity to TBM, follicular dendritic cells (FDC) are localised in GC, which attach antigen material to their surface and in contrast to bone marrow-derived DC, neither take it up nor present it via MHC molecules. Autoreactive B cells can accidentally emerge during somatic hypermutation and migrate into the GC light zone. Autoreactive B cells, maturated coincidently, normally don’t receive survival signals by antigen planted on follicular dendritic cells and perish by apoptosis. In the case of clearance deficiency apoptotic nuclear debris accumulates in the light zone of GC and gets attached to FDC. This serves as a germinal centre survival signal for autoreactive B-cells. After migration into the mantle zone autoreactive B cells require further survival signals from autoreactive helper T cells, which promote the maturation of autoantibody producing plasma cells and B memory cells. In the presence of autoreactive T cells a chronic autoimmune disease may be the consequence. Capital accumulation ... Debris (French, pronounced (IPA) dibri) is a word used to describe the remains of something that has been otherwise destroyed. ... Dendritic cells (DC) are immune cells and form part of the mammal immune system. ... This article does not cite any references or sources. ... Protein images comparing the MHC I (1hsa) and MHC II (1dlh) molecules. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ... This article needs to be cleaned up to conform to a higher standard of quality. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ...


Pathophysiology

Lupus is an example of pathophysiology, a disturbance of the normal functioning of the body. One manifestation of lupus is abnormalities in apoptosis, a type of programmed cell death in which aging or damaged cells are neatly disposed of as a part of normal growth or functioning. Pathophysiology is the study of the disturbance of normal mechanical, physical, and biochemical functions, either caused by a disease, or resulting from a disease or abnormal syndrome or condition that may not qualify to be called a disease. ...


Abnormalities in apoptosis

Tingible body macrophages (TBMs) are large phagocytic cells in the germinal centers of secondary lymph nodes. They express CD68 protein. These cells normally engulf B cells which have undergone apoptosis after somatic hypermutation. In some patients with SLE, significantly fewer TBMs can be found, and these cells rarely contain material from apoptotic B cells. Also, uningested apoptotic nuclei can be found outside of TBMs. This material may present a threat to the tolerization of B cells and T cells. Dendritic cells in the germinal center may endocytose such antigenic material and present it to T cells, activating them. Also, apoptotic chromatin and nuclei may attach to the surfaces of follicular dendritic cells and make this material available for activating other B cells which may have randomly acquired self-specificity through somatic hypermutation.[11] A section of mouse liver showing an apoptotic cell indicated by an arrow Apoptosis (pronounced apo tō sis) is a process of suicide by a cell in a multicellular organism. ... Monocyte A monocyte is a leukocyte, part of the human bodys immune system that protects against blood-borne pathogens and moves quickly (aprox. ... The keratinocyte is the major cell type of the epidermis, making up about 90% of epidermal cells. ... Gene expression, or simply expression, is the process by which the inheritable information which comprises a gene, such as the DNA sequence, is made manifest as a physical and biologically functional gene product, such as protein or RNA. Several steps in the gene expression process may be modulated, including the... The FAS ligand or FasL is a type II transmembrane protein that belongs to the tumor necrosis factor (TNF) family. ... B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ... T cells are a subset of lymphocytes that play a large role in the immune response. ... Germinal centers (GC) are an important part of the humoral immune response. ... Lymph nodes are components of the lymphatic system. ... This article needs to be cleaned up to conform to a higher standard of quality. ... Dendritic cells (DC) are immune cells and form part of the mammal immune system. ... This article needs to be cleaned up to conform to a higher standard of quality. ...


Diagnosis

Some physicians make a diagnosis on the basis of the ACR classification criteria (see below). The criteria, however, were established mainly for use in scientific research (i.e. inclusion in randomized controlled trials), and patients may have lupus but never meet the full criteria. A randomized controlled trial (RCT) is a form of clinical trial, or scientific procedure used in the testing of the efficacy of medicines or medical procedures. ...


Anti-nuclear antibody testing and anti-extractable nuclear antigen (anti-ENA) form the mainstay of serologic testing for lupus. Antiphospholipid antibodies occur more often in SLE, and can predispose for thrombosis. More specific are the anti-smith and anti-dsDNA antibodies. Other tests routinely performed in suspected SLE are complement system levels (low levels suggest consumption by the immune system), electrolytes and renal function (disturbed if the kidney is involved), liver enzymes and a complete blood count. Anti-nuclear antibodies (ANAs, also known as anti-nuclear factor or ANF) are detected in a large group of autoimmune disorders. ... Serology is the scientific study of blood serum. ... Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... Look up the abbreviation LSM in Wiktionary LSM is a three letter abbreviation that may refer to: Landing Ship, Medium, a form of amphibious assault ship Laser Scanning Microscopy (see Confocal laser scanning microscopy), a type of microscopy in which laser light is used for illumination Layered synthetic microstructure, a... A complement protein attacking an invader. ... An electrolyte is a substance containing free ions that behaves as an electrically conductive medium. ... In medicine (nephrology) renal function is an indication of the state of the kidney and its role in physiology. ... Liver function tests (LFTs or LFs), are groups of clinical biochemistry laboratory blood assays designed to give a doctor or other health professional information about the state of a patients liver. ... Schematics of shorthand for complete blood count commonly used by physicians. ...


Formerly, the lupus erythematosus (LE) cell test was used for diagnosis, however those LE cells are only found in 50-75% of SLE patients, and are also found in some patients with rheumatoid arthritis, scleroderma, and drug sensitivities. Because of this, the LE cell test is now performed only rarely and is mostly of historical significance.[12]


Diagnostic criteria

The American College of Rheumatology (ACR) has established eleven criteria in 1982,[13] which were revised in 1997[14] as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individual patients and do not do well in that capacity. For inclusion in clinical trials, patients must meet the following three criteria to be classified as having SLE: (i) patient must present with four of the below eleven symptoms (ii) either simultaneously or serially (iii) during a given period of observation.

  1. Malar rash (rash on cheeks). sensitivity = 57%; specificity = 96%[15]
  2. Discoid lupus (red, scaly patches on skin which cause scarring) sensitivity = 18%; specificity = 99%[15]
  3. Photosensitivity (exposure to ultraviolet light causes rash). sensitivity = 43%; specificity = 96%[15]
  4. Oral ulcers: include oral or nasopharyngeal ulcers
  5. Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling or effusionsensitivity = 86%; specificity = 37%[15]
  6. Renal disorder: More than 0.5 g per day protein in urine, or cellular casts seen in urine under a microscope.sensitivity = 51%; specificity = 94%[15]
  7. Neurologic disorder: Seizures or psychosis. sensitivity = 20%; specificity = 98%[15]
  8. Serositis: Pleuritis (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart)sensitivity = 56%; specificity = 86% (pleural is more sensitive; cardiac is more specific)[15]
  9. Hematologic disorder: Hemolytic anemia (low red blood cell count) or leukopenia (white blood cell count<4000/ul), lymphopenia ( <1500/ul ) or thrombocytopenia (<100000/uL) in the absence of offending drug.sensitivity = 59%; specificity = 89%[15] Hypocomplementemia is also seen, due to either consumption of C3 and C4 by immune complex-induced inflammation, or to congenitally complement deficiency, which may predispose to SLE.
  10. Anti-nuclear antibody test positive. sensitivity = 99%; specificity = 49%[15]
  11. Immunologic disorder: Positive anti-Sm, anti-ds DNA, anti-phospholipid antibody and/or false positive serological test for syphilis. sensitivity = 85%; specificity = 93%[15]. Presence of anti-ss DNA in 70% of patients (though also positive in patients with rheumatic disease and healthy persons[16])

A useful mnemonic for these 11 criteria is SOAP BRAIN MD: Serositis (8), Oral ulcers (4), Arthritis (5), Photosensitivity (3), Blood Changes (9), Renal involvement (proteinuria or casts) (6), ANA (10), Immunological changes (11), Neurological signs (seizures, frank psychosis) (7), Malar Rash (1), Discoid Rash (2). Malar rash is also called as the butterfly rash. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Photodermatitis is a reaction of the skin to UV rays of the sun. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Arthritis (from Greek arthro-, joint + -itis, inflammation; plural: arthritides) is a group of conditions where there is damage caused to the joints of the body. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Proteinuria (from protein and urine) means the presence of an excess of serum proteins in the urine. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... This article is about epileptic seizures. ... Psychosis is a generic psychiatric term for a mental state often described as involving a loss of contact with reality. Stedmans Medical Dictionary defines psychosis as a severe mental disorder, with or without organic damage, characterized by derangement of personality and loss of contact with reality and causing deterioration... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Pleurisy, also known as pleuritis, is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs, which can cause painful respiration and other symptoms. ... Pericarditis is inflammation of the sac surrounding the heart, the pericardium. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Hemolytic anemia is anemia due to hemolysis, the abnormal breakdown of red blood cells either in the blood vessels (intravascular hemolysis) or elsewhere in the body (extravascular). ... “Red cell” redirects here. ... Leukopenia or leukocytopenia refers to a decrease in the number of circulating white blood cells (leukocytes) in the blood. ... Lymphopenia is the condition in which there exists an abnormally low number of lymphocytes in the blood. ... Thrombocytopenia (or -paenia, or thrombopenia in short) is the presence of relatively few platelets in blood. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Anti-nuclear antibodies (ANAs, also known as anti-nuclear factor or ANF) are detected in a large group of autoimmune disorders. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ... Look up the abbreviation LSM in Wiktionary LSM is a three letter abbreviation that may refer to: Landing Ship, Medium, a form of amphibious assault ship Laser Scanning Microscopy (see Confocal laser scanning microscopy), a type of microscopy in which laser light is used for illumination Layered synthetic microstructure, a... This article, image, template or category should belong in one or more categories. ... Serology is the scientific study of blood serum. ... Syphilis is a curable sexually transmitted disease caused by the Treponema pallidum spirochete. ... The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ...


Some patients, especially those with antiphospholipid syndrome, may have SLE without four criteria and SLE is associated with manifestations other than those listed in the criteria.[17][18][19] This article, image, template or category should belong in one or more categories. ...


Alternative criteria

Recursive partitioning has been used to identify more parsimonious criteria.[15] This analysis presented two diagnostic classification trees: Recursive partitioning is a statistical method for the multivariable analysis of medical diagnostic tests. ...


1. Simplest classification tree: LSE is diagnosed if the patient has an immunologic disorder (anti-DNA antibody, anti-Smith antibody, false positive syphilis test, or LE cells) or malar rash. Malar rash is also called as the butterfly rash. ...

2. Full classification tree: Uses 6 criteria. The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ...

Other alternative criteria have been suggested.[20] The sensitivity of a binary classification test or algorithm, such as a blood test to determine if a person has a certain disease, or an automated system to detect faulty products in a factory, is a parameter that expresses something about the tests performance. ... The specificity is a statistical measure of how well a binary classification test correctly identifies the negative cases, or those cases that do not meet the condition under study. ...


Common misdiagnoses

Porphyria

Porphyrias are complex genetic disorders that share many symptoms with lupus, but impact the enzymes responsible for building heme, a component needed in heme proteins. Porphyrias are ecogenic disorders requiring both environmental and genetic backgrounds to manifest with a variety of symptoms and medical complications. They are noted for photosensitivity and have been associated with transient and permanent production of autoantibodies. The five major forms of dominantly inherited porphyrias (acute intermittent porphyria, porphyria cutanea tarda, hereditary coproporphyria, variegate porphyria and erythropoietic protoporphyria) have been detected in systemic lupus erythematosus and discoid lupus patients over the past 50 years. Physicians should have a high degree of suspicion of porphyrias in all lupus cases and act accordingly when patients are in a medical crisis that may be due to an underlying acute hepatic porphyria. Drug-induced lupus and photosensitivity warrant an investigation for an underlying porphyria since multiple drug reactions are a hallmark complication of porphyrias. Patients with both lupus and porphyrias should avoid porphyrinogenic drugs and hormone preparations. Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). ... Structure of Heme b A heme or haem is a prosthetic group that consists of an iron atom contained in the center of a large heterocyclic organic ring called a porphyrin. ... The porphyrias are inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). ...


Patients with acute hepatic porphyrias (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria) have been detected in lupus patients with severe life-threatening "lupus" complications known as neurolupus. Symptoms are identical to acute hepatic porphyria attacks and include seizures, psychosis, peripheral neuropathy and syndrome of inappropriate antidiuretic hormone (SIADH) associated with dangerously low sodium levels (hyponatremia). Porphyria attacks require intervention with intravenous glucose, heme preparations and the discontinuation of dangerous porphyrinogenic drugs including antiseizure drugs. Several other lupus complications have been associated with porphyrias including pancreatitis and pericarditis. Porphyrin testing should be performed on urine, stool/bile and blood to detect all types of porphyrias, and repeat testing should be performed in suspicious cases. Appropriate enzyme tests or DNA testing should also be pursued to obtain a complete diagnosis which could include a dual porphyria. The liver is an organ in vertebrates including humans. ... Psychosis is a generic psychiatric term for a mental state often described as involving a loss of contact with reality. Stedmans Medical Dictionary defines psychosis as a severe mental disorder, with or without organic damage, characterized by derangement of personality and loss of contact with reality and causing deterioration... Peripheral neuropathy is the term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of the nerve or from the side-effects of systemic illness. ... Pancreatitis is inflammation of the pancreas. ... Pericarditis is inflammation of the sac surrounding the heart, the pericardium. ...


Common dual diagnoses

SLE is sometimes diagnosed in conjunction with other conditions, including Rheumatoid Arthritis, Scurvy and Fibromyalgia. Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... Scurvy (N.Lat. ... Fibromyalgia (FM or FMS) is a chronic syndrome (constellation of signs and symptoms) characterized by diffuse or specific muscle, joint, or bone pain, fatigue, and a wide range of other symptoms. ...


Treatment

As lupus erythematosus is a chronic disease with no known cure, treatment is restricted to dealing with the symptoms; essentially this involves preventing flares and reducing their severity and duration when they occur. There are several means of preventing and dealing with flares, including drugs, alternative medicine and lifestyle changes. Symptomatic treatment is any medical therapy of a disease that only affects its symptoms, not its cause, i. ...


Drug therapy

Due to the variety of symptoms and organ system involvement with Lupus patients, the severity of the SLE in a particular patient must be assessed in order to successfully treat SLE. Mild or remittent disease can sometimes be safely left untreated. If required, non-steroidal anti-inflammatory drug and anti-malarials may be used.


Disease-modifying antirheumatic drugs (DMARDs) are used preventively to reduce incidence of flares, the process of the disease, and lower the need for steroid use; when flares occur, they are treated with corticosteroids. DMARDs commonly in use are anti-malarials and immunosupressants (e.g. methotrexate and azathioprine). Hydroxychloroquine (trade name Plaquenil) is an FDA approved anti-malarial used for constitutional, cutaneous, and articular manifestations, while Cyclophosphamide (trade names Cytoxan and Neosar) is used for severe glomerulonephritis or other organ-damaging complications, and in 2005, mycophenolic acidCellCept became accepted for treatment of lupus nephritis.


In more severe cases, medications that modulate the immune system (primarily corticosterois and Immunosuppresive drug immunosuppressants) are used to control the disease and prevent re-occurrence of symptoms (known as flares). Patients who require steroids frequently may develop obesity, diabetes mellitus diabetes and osteoporosis. Depending on the dosage, corticosteroids can cause other side effects such as a puffy face, an unusually large appetite and difficulty sleeping. Those side effects can subside if and when the large initial dosage is reduced, but long term use of even low doses can cause elevated blood pressure and cataracts. Due to these side effects, steroids are avoided if possible.


Since a large percentage of Lupus patients suffer from varying amounts of chronic pain, stronger prescription analgesics may be used if over-the-counter drugs, mainly non-steroidal anti-inflammatory drug do not provide effective relief. Moderate pain in Lupus patients if typically treated with mild prescription opiates such as Dextropropoxyphene (trade name Darvocet), and Co-codamol (trade name Tylenol #3). Moderate to severe chronic pain is treated with stronger opioids such as Hydrocodone (trade names Lorcet, Lortab, Norco, Vicodin, Vicoprofen) or longer-acting continuous release opioids such as Oxycodone (trade names OxyContin), MS Contin, or Methadone. The Fentanyl Duragesic Transdermal patch is also a widely-used treatment option for chronic pain due to Lupus complications because of its long-acting timed release and easy usage. When opioids are used for prolonged periods drug tolerance, chemical dependency and (rarely) addiction may occur. Opiate addiction is not typically a concern for Lupus patients, since the condition is not likely to ever completely disappear. Thus, lifelong treatment with opioids is fairly common in Lupus patients that exhibit chronic pain symptoms; accompanied by periodic titration that is typical of any long-term opioid regimen. An opioid is any agent that binds to opioid receptors found principally in the central nervous system and gastrointestinal tract. ... This article is about volumetric titration. ...


Acupuncture

A 1985 study on lupus and acupuncture reported improvement of lupus sufferers over matched controls, though there was no placebo group for comparison.[21] It is possible that acupuncture may be useful for the treatment some of the symptoms of lupus, but there needs to be more research done before a definitive statement can be made regarding acupuncture.[22]


UVA1 Phototherapy

In 1987, Tina Lomardi, MD first reported that long-wave ultraviolet radiation (UVA1) had a favorable effect on disease activity in SLE model mice. Several clinicals trials investigating this new, counter-intuitive therapeutic approach, conducted by both McGrath and independent Dutch searchers, have confirmed these findings in SLE patients. [23] Devices for administering therapeutic doses of UVA1 are available in Europe but not in the U.S. However, the U.S. Food and Drug Administration Office of Science and Technology conducted UVA1 phototherapy studies in an SLE mouse model in 1997 “to prepare for future reviews of UVA-emitting tanning devices for such clinical applications."[24]


Lifestyle changes

Other measures such as avoiding sunlight or covering up with sun protective clothing can also be effective in preventing problems due to photosensitivity. Weight loss is also recommended in overweight and obese patients to alleviate some of the effects of the disease, especially where joint involvement is significant.


Treatment Research

Other immunosuppressants(A drug that lowers the body's normal immune response) and bone marrow transplant autologous stem cell transplants are under investigation as a possible cure. Recently, treatments that are more specific in modifying the particular subset of the immune cells (e.g. B- or T- cells) or cytokine proteins they secrete have been gaining attention. Research into new treatments has recently been accelerated by genetic discoveries, especially mapping of the human genome. According to a June 2006 market analysis report by Datamonitor, treatment for SLE could be on the verge of a breakthrough as there are numerous late-Phase trials currently being carried out.[25] Cytokines are a group of proteins and peptides that are used in organisms as signaling compounds. ...


Prevention

Lupus is not understood well enough to be prevented, but when the disease develops, quality of life can be improved through flare prevention. The warning signs of an impending flare include increased fatigue, pain, rash, fever, abdominal discomfort, headache and dizziness. Early recognition of warning signs and good communication with a doctor can help individuals with lupus remain active, experience less pain and reduce medical visits.[3]


Prevention of complications during pregnancy

While most infants born to mothers with lupus are healthy, pregnant mothers with SLE should remain under a doctor's care until delivery. Neonatal lupus is rare, but identification of mothers at highest risk for complications allows for prompt treatment before or after birth. In addition, SLE can flare during pregnancy and proper treatment can maintain the health of the mother for longer. Women pregnant and known to have the antibodies for anti-Ro (SSA) or anti-La (SSB) should have echocardiograms during the 16th and 30th weeks of pregnancy to monitor the health of the heart and surrounding vasculature.[3]


Prognosis

In the 1950s, most patients diagnosed with SLE lived fewer than five years. Advances in diagnosis and treatment have improved survival to the point where over 90% of patients now survive for more than ten years and many can live relatively asymptomatically. The most common cause of death is infection due to immunosuppression as a result of medications used to manage the disease. Prognosis is normally worse for men and children than for women. Fortunately, if symptoms are present after age 60, the disease tends to run a more benign course. The ANA is the most sensitive screening test while Anti-Sm (Anti Smith) is the most specific. The ds-DNA (double-stranded DNA) antibody is also fairly specific and often fluctuates with disease activity. The ds-DNA titer is therefore sometimes useful to diagnose or monitor acute flares or response to treatment.[26] This false-colored electron micrograph shows a malaria sporozoite migrating through the midgut epithelia. ... Immunosuppression is the medical suppression of the immune system. ... Look up the abbreviation LSM in Wiktionary LSM is a three letter abbreviation that may refer to: Landing Ship, Medium, a form of amphibious assault ship Laser Scanning Microscopy (see Confocal laser scanning microscopy), a type of microscopy in which laser light is used for illumination Layered synthetic microstructure, a... The structure of part of a DNA double helix Deoxyribonucleic acid, or DNA, is a nucleic acid molecule that contains the genetic instructions used in the development and functioning of all known living organisms. ...


Epidemiology

Previously believed to be a rare disease, Lupus has seen an increase in awareness and education since the 1960s. This has helped many more patients get an accurate diagnosis making it possible to estimate the number of people with lupus. In the United States alone, it is estimated that between 270,000 and 1.5 million people have lupus, making it more common than cystic fibrosis or cerebral palsy. The disease affects both females and males, though young women are diagnosed nine times more often than men. SLE occurs with much greater severity among African-American women, who suffer more severe symptoms as well as a higher mortality rate.[27] Worldwide, a conservative estimate states that over 5 million people have lupus. Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive,[1] non-contagious diseases that cause physical disability in human development. ...


Although SLE can occur in anyone at any age, it is most common in women of childbearing age. It affects 1 in 4000 people in the United States, with women becoming afflicted far more often than men. The disease appears to be more prevalent in women of African, Asian, Hispanic and Native American origin but this may be due to socioeconomic factors. People with relatives who suffer from SLE, rheumatoid arthritis or thrombotic thrombocytopenic purpura are at a slightly higher risk than the general population. Rheumatoid arthritis (RA) is traditionally considered a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... Thrombotic thrombocytopenic purpura (TTP or Moschcowitz disease) is a rare disorder of the blood coagulation system. ...


History

Medical historians have theorized people with porphyrias (a disease that shares many symptoms with Lupus) generated folklore stories of vampires and werewolves due to the photosensitivity, scarring, hair growth and porphyrin brownish-red stained teeth in severe recessive forms of porphyria or combinations of the disorders known as dual, homozygous or compound heterozygous porphyrias. Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). ...


The history of lupus erythematosus can be divided into three periods: the classical, neoclassical, and modern. The classical period began when the disease was first recognized in the Middle Ages and saw the description of the dermatological manifestation of the disorder. The term lupus is attributed to the 12th century physician Rogerius, who used it to describe the classic malar rash. The neoclassical period was heralded by Móric Kaposi's recognition in 1872 of the systemic manifestations of the disease. The modern period began in 1948 with the discovery of the LE cell (the Lupus Erythematosus cell, a misnomer as it occurs with other diseases as well) and is characterised by advances in our knowledge of the pathophysiology and clinical-laboratory features of the disease, as well as advances in treatment. The Middle Ages formed the middle period in a traditional schematic division of European history into three ages: the classical civilization of Antiquity, the Middle Ages, and modern times, beginning with the Renaissance. ... (11th century - 12th century - 13th century - other centuries) As a means of recording the passage of time, the 12th century was that century which lasted from 1101 to 1200. ... For other uses, see Doctor. ... Detail from Rogerius treatise (Amiens, ca. ... Móric Kaposi (b. ... Year 1872 (MDCCCLXXII) was a leap year starting on Monday (link will display the full calendar) of the Gregorian Calendar (or a leap year starting on Saturday of the 12-day slower Julian calendar). ... Year 1948 (MCMXLVIII) was a leap year starting on Thursday (link will display the 1948 calendar) of the Gregorian calendar. ...


Useful medication for the disease was first found in 1894, when quinine was first reported as an effective therapy. Four years later, the use of salicylates in conjunction with quinine was noted to be of still greater benefit. This was the best available treatment to patients until the middle of the twentieth century, when Hench discovered the efficacy of corticosteroids in the treatment of SLE. 1894 (MDCCCXCIV) was a common year starting on Monday (see link for calendar). ... Quinine (IPA: ) is a natural white crystalline alkaloid having antipyretic (fever-reducing), anti-smallpox, analgesic (painkilling), and anti-inflammatory properties and a bitter taste. ... Salicylic acid (from the Latin word for the willow tree, Salix, from whose bark it can be obtained) is a beta hydroxy acid (BHA) with the formula C6H4(OH)CO2H, where the OH group is adjacent to the carboxyl group. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ...


Origins of 'Lupus Erythematosus'

There are several explanations ventured for the term lupus erythematosus. Lupus is Latin for wolf, and 'erythro' is derived from ερυθρός, Greek for "red." All explanations originate with the reddish, butterfly-shaped malar rash that the disease classically exhibits across the nose and cheeks. For other uses, see Latin (disambiguation). ... Malar rash is also called as the butterfly rash. ...

  1. In various accounts, some doctors thought the rash resembled the pattern of fur on a wolf's face.
  2. In other accounts doctors thought that the rash, which was often more severe in earlier centuries, created lesions that resembled wolf bites or scratches.
  3. Another account claims that the term "Lupus" did not come from Latin at all, but from the term for a French style of mask which women reportedly wore to conceal the rash on their faces. The mask is called a "loup", French for "Wolf"
  4. Another common explanation for the term is that the disease's course involves repeated attacks like those of a voracious predator, leaving behind the red blotches.

Notable patients

Ray Walston (December 2, 1914 – January 1, 2001) was a stage, television and feature film character actor who played the title character on the situation comedy My Favorite Martian and Judge Henry Bone on the drama series Picket Fences. ... Timothy Raines (born September 16, 1959 in Sanford, Florida), nicknamed Rock, is a former American left fielder in Major League Baseball known for his speed and ability to get on base. ... The Montreal Expos (French: Les Expos de Montréal) were a Major League Baseball team located in Montreal, Quebec, Canada from 1969 until 2004. ... Major league affiliations American League (1901–present) Central Division (1994–present) Current uniform Retired Numbers 2, 3, 4, 9, 11, 16, 19, 42, 72, Name Chicago White Sox (1904–present) Other nicknames The Sox, The South Siders, The ChiSox, The Pale Hose, The Good Guys, The Go-Go Sox, The... Ferdinand Emmanuel Edralín Marcos (September 11, 1917 – September 28, 1989) was President of the Philippines from 1966 to 1986. ... Mary Flannery OConnor (March 25, 1925 – August 3, 1964) was an American novelist, short-story writer and essayist. ... Hugh Todd Naylor Gaitskell (April 9, 1906 – January 18, 1963) was a British politician, leader of the Labour Party from 1955 until his death in 1963. ... Wikiquote has a collection of quotations related to: J Dilla James Dewitt Yancey (February 7, 1974–February 10, 2006), better known as J Dilla, or Jay Dee, was an American hip hop producer and MC, who emerged from the mid-1990s underground hip-hop scene in Detroit, Michigan. ... J Dilla J Dilla, alias Jay Dee (born James Yancey), is a hip-hop record producer and MC, who emerged from the mid-1990s independent, underground hip-hop scene in Detroit, MI. He began his career as Jay Dee but since 2001 has called himself J Dilla, a name not... On the cover of Elaine Paige Tour Programme 2004 Elaine Paige OBE (born Elaine Bickerstaff on 5 March 1948 in Barnet, Hertfordshire) is a world-renowned English singer and actor, primarily in musicals. ... Michael Joseph Jackson (August 29, 1958), commonly known as MJ as well as the King of Pop, is an American musician, entertainer, and pop icon whose successful career and controversial personal life have been a part of pop culture for the last three decades. ... Not to be confused with alphos, a form of leprosy once called vitiligo. ... Barbara Bush and Millie Mildred Kerr Bush (January 12, 1985 - May 19, 1997) better known as Millie, was the pet springer spaniel of Barbara and George H. W. Bush. ... George Herbert Walker Bush (born June 12, 1924) was the 41st President of the United States, serving from 1989 to 1993. ... For the daughter of President George W. Bush, see Barbara Pierce Bush. ... Charles Kuralt Charles Kuralt (10 September 1934 – 4 July 1997) was an award-winning American journalist whose long career with CBS made him famous as the motor home-traveling reporter whose chronicling of out-of-the-news American people and living made him as much of a household name as... Sunday Mornings Sun logo for Sunday news Charles Kuralt, Host from 1979-1994 CBS Sunday Morning is an early morning news program CBS airs from 9:00-10:30 AM EST on Sunday mornings. ... Inday Ba (also known as NDeaye Ba) (10 August 1972-20 April 2005) was a Swedish-born actress. ... Howard Dwaine Dorough[1] (born August 22, 1973), also known as Howie D, is an American musician and member of the boy band Backstreet Boys. ... This article is about the band. ... Look up the abbreviation LSM in Wiktionary LSM is a three letter abbreviation that may refer to: Landing Ship, Medium, a form of amphibious assault ship Laser Scanning Microscopy (see Confocal laser scanning microscopy), a type of microscopy in which laser light is used for illumination Layered synthetic microstructure, a... Mercedes Scelba-Shorte (born 24th August 1981, from Valencia, California), also known as Mercedes Yvette, is an American fashion model and actress. ... ANTM redirects here. ... Louisa May Alcott (November 29, 1832 – March 6, 1888) was an American novelist. ... Sophie Howard (born February 24, 1983) is a glamour model from Southport, England. ... The sixth season of American Idol premiered on the FOX Broadcasting Network on January 16, 2007 and will run until late May. ... American Idol is an annual American televised singing competition, which began its first season on June 11, 2002. ... Seal Henry Olusegun Olumide Adeola Samuel (born February 19, 1963 in Paddington, London) is an English soul singer and songwriter. ... Donald Byrne (June 12, 1930–May 6, 1976) was one of the USAs strongest chess players during the 1950s and 1960s. ... This article is about the Western board game. ...

See also

  • Abzyme
  • Anti-nuclear antibody
  • Canine discoid lupus erythematosus in dogs
  • Lupus Canada
  • Lupus Mid-Atlantic
  • Lupus Foundation of America

An abzyme (from antibody and enzyme), also called catmab (from catalytic monoclonal antibody), is a monoclonal antibody with catalytic activity. ... Anti-nuclear antibodies (ANAs, also known as anti-nuclear factor or ANF) are detected in a large group of autoimmune disorders. ... This article is about discoid lupus erythematosus in dogs. ... Lupus Canada is a national voluntary organization dedicated to improving the lives of people living with lupus through advocacy, education, public awareness, support and research. ... The Lupus Foundation of America (LFA) is the nations leading non-profit voluntary health organization dedicated to finding the causes of and cure for lupus. ...

References

  1. ^ LUPUS FOUNDATION OF AMERICA. Retrieved on 2007-07-04.
  2. ^ Discoid Lupus Erythematosus
  3. ^ a b c d Handout on Health: Systemic Lupus Erythematosus. The National Institute of Arthritis and Musculoskeletal and Skin Diseases. National Institutes of Health (August 2003). Retrieved on 2007-11-23.
  4. ^ Lupus: The Great Imitator
  5. ^ a b Joint and Muscle Pain Lupus Foundation of America
  6. ^ Yu Asanuma, M.D., Ph.D., Annette Oeser, B.S., Ayumi K. Shintani, Ph.D., M.P.H., Elizabeth Turner, M.D., Nancy Olsen, M.D., Sergio Fazio, M.D., Ph.D., MacRae F. Linton, M.D., Paolo Raggi, M.D., and C. Michael Stein, M.D. (2003). "Premature coronary-artery atherosclerosis in systemic lupus erythematosus". New England Journal of Medicine 349 (Dec. 18): 2407-2414. PMID 14681506 Abstract (full text requires registration). 
  7. ^ Bevra Hannahs Hahn, M.D. (2003). "Systemic lupus erythematosus and accelerated atherosclerosis". New England Journal of Medicine 349 (Dec. 18): 2379-2380. PMID 14681501 Extract (full text requires registration). 
  8. ^ Mary J. Roman, M.D., Beth-Ann Shanker, A.B., Adrienne Davis, A.B., Michael D. Lockshin, M.D., Lisa Sammaritano, M.D., Ronit Simantov, M.D., Mary K. Crow, M.D., Joseph E. Schwartz, Ph.D., Stephen A. Paget, M.D., Richard B. Devereux, M.D., and Jane E. Salmon, M.D. (2003). "Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus". New England Journal of Medicine 349 (Dec. 18): 2399-2406. PMID 14681505 Abstract (full text requires registration). 
  9. ^ General Pathology Images for Immunopathology. Retrieved on 2007-07-24.
  10. ^ University of South Carolina lecture on immunology
  11. ^ Gaipl, U S; Kuhn, A; Sheriff, A; Munoz, L E; Franz, S; Voll, R E; Kalden, J R; Herrmann, M (2006). "Clearance of apoptotic cells in human SLE.". Current directions in autoimmunity 9: 173-87. PMID: 1639466 Abstract (full text requires registration). 
  12. ^ NIM encyclopedic article on the LE cell test
  13. ^ Rheumatology.org article on the classification of rheumatic diseases
  14. ^ Revision of Rheumatology.org's diagnostic criteria
  15. ^ a b c d e f g h i j k Edworthy SM, Zatarain E, McShane DJ, Bloch DA (1988). "Analysis of the 1982 ARA lupus criteria data set by recursive partitioning methodology: new insights into the relative merit of individual criteria". J. Rheumatol. 15 (10): 1493-8. PMID 3060613. 
  16. ^ UpToDate Patient information article on DNA antibodies
  17. ^ Asherson RA, Cervera R, de Groot PG, et al (2003). "Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines". Lupus 12 (7): 530-4. PMID 12892393. 
  18. ^ Sangle S, D'Cruz DP, Hughes GR (2005). "Livedo reticularis and pregnancy morbidity in patients negative for antiphospholipid antibodies". Ann. Rheum. Dis. 64 (1): 147-8. doi:10.1136/ard.2004.020743. PMID 15608315. 
  19. ^ Hughes GR, Khamashta MA (2003). "Seronegative antiphospholipid syndrome". Ann. Rheum. Dis. 62 (12): 1127. PMID 14644846. 
  20. ^ Hughes GR (1998). "Is it lupus? The St. Thomas' Hospital "alternative" criteria". Clin. Exp. Rheumatol. 16 (3): 250-2. PMID 9631744. 
  21. ^ Review of different alternative treatments for lupus
  22. ^ Charis F. Meng, MD (2002-01-16). Acupuncture for SLE: Can it Work for You?. Hospital for Special Surgery: Treatments & Conditions. Retrieved on 2007-11-23.
  23. ^ Light therapy (with UVA-1) for SLE patients: is it a good or bad idea? -- Pavel 45 (6): 653 -- Rheumatology. Retrieved on 2007-07-04.
  24. ^ Ultraviolet-A1 (UVA1) Phototherapy of Systemic Lupus Erythematosus Food and Drug Administration (FDA)
  25. ^ Lead Discovery article on treatment of Lupus
  26. ^ [EARLY STEROIDS MAY PREVENT RELAPSES IN LUPUS, P Jarman (Published in Journal Watch (General) July 18, 1995)
  27. ^ Lupus and African-American women

DiGeronimo, Theresa. New Hope for People with Lupus. Prima Publishing. 2002. Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 185th day of the year (186th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 327th day of the year (328th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 205th day of the year (206th in leap years) in the Gregorian calendar. ... A digital object identifier (or DOI) is a standard for persistently identifying a piece of intellectual property on a digital network and associating it with related data, the metadata, in a structured extensible way. ... Also see: 2002 (number). ... is the 16th day of the year in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 327th day of the year (328th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 185th day of the year (186th in leap years) in the Gregorian calendar. ... is the 199th day of the year (200th in leap years) in the Gregorian calendar. ... Year 1995 (MCMXCV) was a common year starting on Sunday (link will display full 1995 Gregorian calendar). ...


External links


  Results from FactBites:
 
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Lupus erythematosus is an autoimmune disease, that is, a disease that alters the function of your immune system.
Lupus erythematosus is a condition in which there is antibody production to self-tissues, and these may be detected in the skin with this test.
The skin manifestations of lupus erythematosus are the result of inflammation in the skin that is primarily mediated by inflammatory cells called T lymphocytes.
Systemic Lupus Erythematosus (SLE) Causes, Symptoms, Treatment, Signs and Information on MedicineNet.com (1015 words)
Lupus attacked her lungs, causing shortness of breath, sleeping problems, and more pain in the form of pleurisy.
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