Lupus erythematosus (also known as systemic lupus erythematosus - SLE) is an autoimmune disorder in which antibodies are created against the patient's own DNA. It classically presents with a butterfly-shaped malar rash, causing a wolf-like appearance (Lupus is Latin for wolf). Despite this very clear etymology, it has been hypothesized that lupus erythematosus may be one of the diseases that may have inspired the myth of the werewolf. Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Wikipedia does not yet have an article with this exact name. ... DNA replication Deoxyribonucleic acid (DNA) is a nucleic acid which is capable of carrying genetic instructions for the biological development of all cellular forms of life and many viruses. ... For the manga by Tsukasa Hôjô, see Rash. ... Binomial name Canis lupus Linnaeus, 1758 The Wolf or Grey Wolf (Canis lupus) is a mammal of the Canidae family and the ancestor of the domestic dog. ... Latin - Wikipedia /**/ @import /skins/monobook/IE50Fixes. ... A werewolf in folklore and mythology is a person who changes into a wolf, either by purposefully using magic in some manner or by being placed under a curse. ...

Signs and symptoms

Common initial and chronic complaints are fever, malaise, myalgias, fatigue and weight loss. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE, but when occurring in conjunction with other signs and symptoms, they are considered suggestive. Fever is also the name of an album by Kylie Minogue. ... Malaise is a term used to refer to a general state of discomfort, tiredness, or illness. ... Myalgia means muscle pain and is a symptom of many diseases and disorders. ... Fatigue is a feeling of excessive tiredness or lethargy, with a desire to rest, perhaps to sleep. ...

Dermatological manifestations

As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), but only 30% to 50% suffer the classic malar (or ?butterfly?) rash associated with the disease. Patients may present with discoid lupus, thick red scaly patches on the skin. Alopecia, mouth and vaginal ulcers and lesions on the skin are also possible manifestations. Baldness (formally alopecia) is the state of lacking hair where it usually would grow, especially on the head. ... A mouth ulcer or canker sore is a painful open sore inside the mouth caused by a break in the mucous membrane. ...

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. Unlike rheumatoid arthritis, SLE arthropathy is not usually destructive of bone, however, deformities caused by the disease may become irreversible in as many as 20% of patients. This article is about a joint in zootomical anatomy. ... Rheumatoid arthritis (RA) is a chronic, inflammatory autoimmune disorder that causes the immune system to attack the joints. ... Grays illustration of a human femur, a typically recognized bone. ...

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. Anemia ( American English) or anaemia ( Commonwealth English), which literally means without blood, is a lack of red blood cells and/or hemoglobin. ... A 250 ml bag of newly collected platelets. ... White Blood Cells is also the name of a White Stripes album. ...

Cardiac manifestations

Patients may present with inflammation of various parts of the heart: pericarditis, myocarditis and endocarditis. There may be immune complexes on the mitral valve of the heart, where bacteria can accumulate. Atherosclerosis also tends to occur more often and advance more rapidly in SLE patients than in the general population. This page is about the muscular organ, the Heart. ... Pericarditis is inflammation of 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. ... Hypersensitivity is the name given to a state in which an immune response damages the bodys own tissues. ... The mitral valve is a valve in the heart that lies between the left atrium (LA) and the left ventricle (LV). ... This page is about the muscular organ, the Heart. ... Atherosclerosis is a disease of arterial blood vessels. ...

Renal involvement

Painless hematuria or proteinuria may often be the only presenting renal symptom. Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients. In medicine, hematuria 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. ... Renal failure is when the kidneys fail to function properly. ...

Neurological manifestations

About 10% of patients may present with seizures or psychosis. A third may test positive for abnormalities in the cerebrospinal fluid. This article is about the medical condition. ... Psychosis is a psychiatric classification for a mental state in which the perception of reality is distorted. ... Cerebrospinal fluid, CSF in short, is the clear fluid that occupies the subarachnoid space (the space between the skull and cortex of the brain). ...

Diagnosis

Some physicians make a diagnosis on the basis of the ARC classification criteria (see below). The criteria, however, were established mainly for use in scientific research (i.e. inclusion in randomised controlled trials), and patients may have lupus despite never meeting the criteria. A randomized controlled trial (RCT) is a form of clinical trial, or scientific procedure used in the testing of the efficacy of medicine, used because of its record of reliability. ...

Antinuclear 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 is the anti-smith antibody. 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 full blood count. Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are detected in a large group of autoimmune disorders. ... Antiphospholipid syndrome, or antiphospholipid antibody syndrome, is a disorder of coagulation, and causes thrombosis in both arteries and veins, as well as recurrent miscarriage. ... Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... The complement system is a complex biochemical cascade of the immune system, leading to cytolysis, chemotaxis, opsonization and inflammation, it can mark pathogens for phagocytosis. ... An electrolyte is a substance which dissociates free ions when dissolved (or molten), to produce 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. ... A full blood count (FBC) or complete blood count (CBC) is a test requested by a doctor or other medical professional that gives information about the cells in a patients blood. ...

Classification

The American College of Rheumatology has established eleven criteria in 1982[1] (http://www.rheumatology.org/publications/classification/index.asp?aud=mem), which were revised in 1997[2] (http://www.rheumatology.org/publications/classification/SLE/1982SLEupdate.asp?aud=mem), 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. A patient must present with four of the eleven criteria, either simultaneously or serially, during a given period of observation, to be classified as having SLE - for the purposes of inclusion in clinical trials.

1. Malar rash (rash on cheeks)
2. Discoid lupus (red, scaly patches on skin which cause scarring)
3. Photosensitivity (adverse reaction to sunlight)
4. Mouth ulcers
5. Arthritis
6. More than 0.5g per day protein in urine, or cellular casts seen in urine under a microscope.
7. Seizures or psychosis
8. Pleuritis (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart)
9. Hemolytic anemia (low red blood cell count), leukopenia (low white blood cell count), lymphopenia (low lymphocyte count) or thrombocytopenia (low platelet count)
10. Anti-DNA antibody, anti-Sm antibody or false positive serological test for syphilis or antiphospholipid antibody positivity
11. Positive fluorescence antinuclear antibody test (positive ANA)

Some patients may have SLE without four criteria and SLE is associated with manifestations other than those listed in the criteria. Dr Graham R.V. Hughes, an authority on lupus in the UK, has published "alternative criteria" to diagnose SLE[3] (http://www.lupus-support.org.uk) in 1982. Arthritis (from Greek arthro-, joint + -itis, inflammation) is a group of conditions that affect the health of the bone joints in the body. ... This article is about the medical condition. ... Psychosis is a psychiatric classification for a mental state in which the perception of reality is distorted. ... ... Pericarditis is inflammation of the pericardium. ... Human red blood cells Red blood cells are the most common type of blood cell and are the vertebrate bodys principal means of delivering oxygen to body tissues via the blood. ... Leukopenia, low white blood count is seen in: aplastic anemia pancytopenia Categories: Stub ... Lymphopenia is the condition in which there exists an abnormally low number of lymphocytes in the blood. ... A lymphocyte is a type of white blood cell involved in the human bodys immune system. ... Thrombocytopenia (or -paenia, or thrombopenia in short) is the presence of relatively few platelets in blood. ... Schematic of antibody binding to an antigen An antibody is a protein used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... Serology is a medical blood test to detect the presence of antibodies against a microorganism. ... Depression-era U.S. poster advocating early syphilis treatment Syphilis (historically called lues) is a sexually transmitted disease (STD) that is caused by a spirochaete bacterium, Treponema pallidum. ... Antiphospholipid syndrome, or antiphospholipid antibody syndrome, is a disorder of coagulation, and causes thrombosis in both arteries and veins, as well as recurrent miscarriage. ... Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are detected in a large group of autoimmune disorders. ...

Pathophysiology

Causes

The exact cause of the disease is unknown, and there is no consensus on whether it is a single condition or a group of related diseases. SLE is a chronic inflammatory disease believed to be a type III hypersensitivity response, which is characterised by the body's production of antibodies against the nuclear components of its own cells. 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. Certain medications (such as some antidepressants) and environmental factors (such as exposure to sunlight) have been found to exacerbate symptoms. Inflammation is the first response of the immune system to infection or irritation and may be referred to as the innate cascade. ... Hypersensitivity is the name given to a state in which an immune response damages the bodys own tissues. ... A common alternate meaning of virus is computer virus. ... Phyla/Divisions Actinobacteria Aquificae Bacteroidetes/Chlorobi Chlamydiae/Verrucomicrobia Chloroflexi Chrysiogenetes Cyanobacteria Deferribacteres Deinococcus_Thermus Dictyoglomi Fibrobacteres/Acidobacteria Firmicutes Fusobacteria Gemmatimonadetes Nitrospirae Omnibacteria Planctomycetes Proteobacteria Spirochaetes Thermodesulfobacteria Thermomicrobia Thermotogae Bacteria (singular, bacterium) are a major group of living organisms. ... Antidepressant - Wikipedia /**/ @import /skins/monobook/IE50Fixes. ...

Treatment

SLE is a chronic disease with no cure. There are, however, some medications, such as corticosteroids and immunosuppressants which can control the disease and prevent flares. Measures such as avoiding sunlight (to prevent problems due to photosensitivity) may also have some effect. 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. ...

Epidemiology

Although SLE can occur in anyone at any age, it is most common in women of childbearing age. It affects one in 3000 people in the United States, with women suffering five to nine times more often than men. The disease appears to be more prevalent in women of African and Hispanic origin but this may be due to socioeconomic factors. People with relatives who suffer from SLE, rheumatoid arthritis or thrombotic thrombocytopenic purpura are at higher risk than the general population. Rheumatoid arthritis (RA) is 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 that in most cases arises from the deficiency or inhibition of the enzyme responsible for cleaving von Willebrand factor. ...

Prognosis

In the 1950s, most patients diagnosed with SLE lived less 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 and if symptoms present after age 60, the disease tends to run a more benign course. In medicine, infectious disease or communicable disease is disease caused by a biological agent (e. ...

History

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 recognised in the Middle Ages and saw the description of the dermatological manifestation of the disorder. The term Lupus is attributed to the thirteenth century physician Rogerius, who used it to describe the classic malar rash. The neoclassical period was heralded by Moritz Kohn 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 (although use of these cells as diagnostic indicators has now been largely abandoned) and is characterised by advances in our knowledge of the pathopysiology 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. ... Events January - April January 2 - Brigham Young, is arrested for bigamy (25 wives). ...

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 conjuction with quinine was noted to be of still greater benefit. This was the best available to patients until the middle of the twentieth century when Hench discovered the efficacy of corticosteroids in the treatment of SLE. 1894 was a common year starting on Monday (see link for calendar). ... Molecular structure of quinine Quinine, C20H24N2O2, is a natural white crystalline alkaloid having antipyretic, anti-malarial and analgesic properties and a bitter taste. ... Salicylic acid is a colorless, crystalline organic carboxylic acid. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ...

See also

• abzyme
• antinuclear antibody

An abzyme (from antibody and enzyme), also called catmab (from catalytic monoclonal antibody), is a monoclonal antibody with catalytic activity. ... Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are detected in a large group of autoimmune disorders. ...

External links

• Alliance for Lupus Research (http://www.lupusresearch.org/)
• Lupus Foundation (http://www.lupus.org)
• Lupus Erythematosus of the skin, New Zealand Dermatological Society Incorporated (http://www.dermnetnz.org/immune/cutaneous-lupus.html)
• History of Lupus (http://www.lupus.org.nz/history.html) (NZ Lupus Trust)
• LUpus Patients Understanding & Support (LUPUS) (http://www.lupus-support.org.uk/)
• The LuPUS Message Board (http://www.lupus-forums.org.uk/)