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*[http://www.mymommytime.com/2009/01/lupus-during-pregnancy/ Lupus During Pregnancy]


{{Diseases of the skin and appendages by morphology}}
{{Diseases of the skin and appendages by morphology}}

Revision as of 16:52, 7 February 2009

This article is about the disease commonly referred to as "lupus". For other uses, see Lupus. For forms of lupus that do not affect the body as a whole, see Lupus erythematosus.
Lupus
SpecialtyImmunology, rheumatology, dermatology Edit this on Wikidata

Systemic lupus erythematosus (SLE or lupus, pronounced sɪˈstɛmɪk ˈluːpəs ˌɛrəˌθiməˈtoʊsəsⓘ) is a chronic autoimmune connective tissue disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage.[1]

SLE 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 remissions. The disease occurs nine times more often in women than in men, especially between the ages of 15 and 50, and is more common in those of non-European descent.[2][3][4]

SLE is treatable through addressing its symptoms, mainly with corticosteroids and immunosuppressants; there is currently no cure. SLE can be fatal, although with recent medical advances, fatalities are becoming increasingly rare. Survival for people with SLE in the United States, Canada, and Europe is approximately 95% at five years, 90% at 10 years, and 78% at 20 years.[4]

Classification

Discoid lupus erythematosus lesion of the face.

There are several types of lupus; in general, when the word lupus alone is used, reference is to systemic lupus erythematosus, as discussed in this article. Other types include:[1]

Signs and symptoms

SLE is one of several diseases known as "the great imitators" because it often mimics or is mistaken for other illnesses.[7] SLE is a classical item in differential diagnosis,[2] because SLE symptoms vary widely and come and go unpredictably. Diagnosis can thus be elusive, with some people suffering unexplained symptoms of untreated SLE for years.

Common initial and chronic complaints are fever, malaise, joint pains, myalgias, fatigue, and temporary loss of cognitive abilities. 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 (see below), however, they are considered suggestive.[8]

Dermatological manifestations
Artistic rendition of a butterfly rash.

As many as 30% of sufferers have 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. Some may exhibit thick, red scaly patches on the skin (referred to as discoid lupus). Alopecia; mouth, nasal, and vaginal ulcers; and lesions on the skin are also possible manifestations.

Musculoskeletal manifestations

The most commonly saught medical attention is for joint pain, with the small joints of the hand and wrist usually affected, although all joints are at risk. The Lupus Foundation of America estimates that more than 90 percent will experience joint and/or muscle pain at some time during the course of their illness.[9] Unlike rheumatoid arthritis, lupus arthritis is less disabling and usually does not cause severe destruction of the joints. Fewer than ten percent of people with lupus arthritis will develop deformities of the hands and feet.[9]

Hematological manifestations

Anemia and iron deficiency may develop in up to 50% of case. Low platelet and white blood cell counts may be due to the disease or a side-effect of pharmacological treatment. People with SLE may have an association with antiphospholipid antibody syndrome (a thrombotic disorder), wherein autoantibodies to phospholipids are present in their 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 SLE is the anticardiolipin antibody, which can cause a false positive test for syphilis.

Cardiac manifestations

A person with SLE may have inflammation of various parts of the heart, such as pericarditis, myocarditis, and endocarditis. The endocarditis of SLE is characteristically noninfective (Libman-Sacks endocarditis) and involves either the mitral valve or the tricuspid valve. Atherosclerosis also tends to occur more often and advances more rapidly than in the general population.[10][11][12]

Pulmonary manifestations

Lung and pleura inflammation can cause pleuritis, pleural effusion, lupus pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, pulmonary hemorrhage, and shrinking lung syndrome.

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 cases.

A histological hallmark of SLE is membranous glomerulonephritis with "wire loop" abnormalities.[13] This finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical granular appearance in immunofluorescence testing.

Neuropsychiatric manifestations

Neuropsychiatric syndromes can result when SLE affects the central or peripheral nervous system. The American College of Rheumatology defines 19 neuropsychiatric syndromes in systemic lupus erythematosus.[14] The most common neuropsychiatric disorder people with SLE have is headache, although the existence of a specific lupus headache and the optimal approach to headache in SLE cases remains controversial.[15] Other common neuropsychiatric manifestation of SLE include cognitive dysfunction, mood disorder, cerebrovascular disease, seizures, polyneuropathy, anxiety disorder and psychosis.

More rare manifestations are acute confusional state, Guillain-Barré syndrome, aseptic meningitis, autonomic disorder, demyelinating syndrome, mononeuropathy (which might manifest as mononeuritis multiplex), movement disorder (more specifically, chorea), myasthenia gravis, myelopathy, cranial neuropathy and plexopathy.

Systemic manifestations

Fatigue in SLE is probably multifactorial and has been related not only to disease activity or complications such as anemia or hypothyroidism but also to pain, depression, poor sleep quality, poor physical fitness and perceived lack of social support.[16][17]

Other rarer manifestations

Lupus gastroenteritis, lupus pancreatitis, lupus cystitis, autoimmune inner ear disease, parasympathetic dysfunction, retinal vasculitis, and systemic vasculitis.[citation needed]

Causes

There is no one specific cause of SLE. There are however a number of environmental triggers and a number of genetic susceptibilities.[18]

Genetics

The first mechanism may arise genetically. Research indicates that SLE may have a genetic link. SLE does run in families, but no single, causal, gene has been identified. Instead, multiple genes appear to influence a person's chance of developing lupus when triggered by environmental factors. The most important genes are located in the HLA region on chromosome 6, where mutations may occur randomly (de novo) or may be inherited. Other genes which contain risk variants for SLE are IRF5, PTPN22, STAT4, ITGAM, BLK, TNFSF4 and BANK1.[19]

Environmental triggers

The second mechanism may be due to environmental factors. These factors may not only exacerbate existing SLE 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 that these antibodies cause connective tissue diseases such as SLE. There is also a small but growing body of evidence linking SLE to lipstick usage [20] [21]

Drug reactions

Drug-induced lupus erythematosus is a reversible condition that usually occurs in people being treated for a long-term illness. Drug-induced lupus mimics SLE. However, symptoms of drug-induced lupus generally disappear once the medication that triggered the episode is stopped. There are about 400 medications currently in use that can cause this condition, the most common of which are procainamide, hydralazine, quinidine, and Phenytoin.[2]

Non-SLE forms of lupus

Other forms of lupus can predispose to SLE. About 1–5% of cases of discoid lupus eventually develop into SLE.[citation needed] Discoid (cutaneous) lupus is limited to skin symptoms and is diagnosed by biopsy of skin rash on the face, neck, or scalp. Often an antinuclear antibody (ANA) test for discoid lupus is negative or a low-titer positive.[citation needed]

Pathophysiology

One manifestation of SLE 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.

Transmission

In SLE, the body's immune system produces antibodies against itself, particularly against proteins in the cell nucleus. SLE is triggered by environmental factors that are unknown.

"All the key components of the immune system are involved in the underlying mechanisms" of SLE, according to Rahman, and SLE is the prototypical autoimmune disease. The immune system must have a balance (homeostasis) between being sensitive enough to protect against infection, and being too sensitive and attacking the body's own proteins (autoimmunity). From an evolutionary perspective, according to Crow, the population must have enough genetic diversity to protect itself against a wide range of possible infection; some genetic combinations result in autoimmunity. The likely environmental triggers include ultraviolet light, drugs, and viruses. These stimuli cause the destruction of cells and expose their DNA, histones, and other proteins, particularly parts of the cell nucleus. Because of genetic variations in different components of the immune system, in some people the immune system attacks these nuclear-related proteins and produces antibodies against them. In the end, these antibody complexes damage blood vessels in critical areas of the body, such as the glomeruli of the kidney; these antibody attacks are the cause of SLE. Researchers are now identifying the individual genes, the proteins they produce, and their role in the immune system. Each protein is a link on the autoimmune chain, and researchers are trying to find drugs to break each of those links. [2][22][23]

SLE is a chronic inflammatory disease believed to be a type III hypersensitivity response with potential type II involvement.[24]

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 that have undergone apoptosis after somatic hypermutation. In some people 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 that may have randomly acquired self-specificity through somatic hypermutation.[25]

Clearance deficiency

Clearance deficiency.

The exact mechanisms for the development of 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 and scant serum components in addition to increased apoptosis.

Monocytes isolated from whole blood of SLE sufferers 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; 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. With SLE, these components are often missing, diminished, or inefficient.

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 simulates 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 people with cutaneous lupus erythematosus (CLE).[26]

Germinal centres.

Accumulation in germinal centres (GC)

In healthy conditions, apoptotic lymphocytes are removed in germinal centres by specialised phagocytes, the tingible body macrophages (TBM), which is why no free apoptotic and potential autoantigenic material can be seen. In some people 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 coincidentally, normally do not 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.

Diagnosis

Microphotograph of a histological section of human skin prepared for direct immunofluorescence using an anti-IgG antibody. The skin is from a person with systemic lupus erythematosus and shows IgG deposits at two different places: The first is a bandlike deposit along the epidermal basement membrane ("lupus band test" is positive); the second is within the nuclei of the epidermal cells (antinuclear antibodies are present).

Laboratory tests

Antinuclear antibody (ANA) testing and anti-extractable nuclear antigen (anti-ENA) form the mainstay of serologic testing for SLE. Antiphospholipid antibodies occur more often in SLE and can predispose for thrombosis.

ANA screening yields positive results in many connective tissue disorders and other autoimmune diseases, and may occur in normal individuals. Subtypes of antinuclear antibodies include anti-Smith and anti-double stranded DNA (dsDNA) antibodies (which are linked to SLE) and anti-histone antibodies (which are linked to drug-induced lupus). Anti-dsDNA antibodies are highly specific for SLE; they are present in 70% of cases, whereas they appear in only 0.5% of people without SLE.[2] The anti-dsDNA antibody titers also tend to reflect disease activity, although not in all cases.[2] Other ANA that may occur in SLE sufferers are anti-U1 RNP (which also appears in systemic sclerosis), SS-A (or anti-Ro) and SS-B (or anti-La; both of which are more common in Sjögren's syndrome). SS-A and SS-B confer a specific risk for heart conduction block in neonatal lupus.[27]

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.

Previously, the lupus erythematosus (LE) cell test was not commonly used for diagnosis because those LE cells are only found in 50–75% of SLE cases, and are also found in some people 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.[28]

Diagnostic criteria

Some physicians make a diagnosis on the basis of the American College of Rheumatology (ACR) classification criteria. The criteria, however, were established mainly for use in scientific research including use in randomized controlled trials which require higher confidence levels, so some people with SLE may not pass the full criteria.

The American College of Rheumatology established eleven criteria in 1982,[29] which were revised in 1997[30] as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. For inclusion in clinical trials, the following three criteria must be met to be classified as SLE: (i) person must present with four of the following eleven symptoms, (ii) either simultaneously or serially, (iii) during a given period of observation.

  1. 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).[31]
  2. Oral ulcers (includes oral or nasopharyngeal ulcers).
  3. Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion; sensitivity = 86%; specificity = 37%.[31]
  4. Photosensitivity (exposure to ultraviolet light causes skin rash, or other symptoms of SLE flareups); sensitivity = 43%; specificity = 96%.[31]
  5. Blood—hematologic disorder—hemolytic anemia (low red blood cell count) or leukopenia (white blood cell count<4000/µl), lymphopenia (<1500/µl) or thrombocytopenia (<100000/µl) in the absence of offending drug; sensitivity = 59%; specificity = 89%.[31] 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.
  6. Renal disorder: More than 0.5g per day protein in urine or cellular casts seen in urine under a microscope; sensitivity = 51%; specificity = 94%.[31]
  7. Antinuclear antibody test positive; sensitivity = 99%; specificity = 49%.[31]
  8. Immunologic disorder: Positive anti-Smith, anti-ds DNA, antiphospholipid antibody, and/or false positive serological test for syphilis; sensitivity = 85%; specificity = 93%.[31] Presence of anti-ss DNA in 70% of cases (though also positive with rheumatic disease and healthy persons)[32]).
  9. Neurologic disorder: Seizures or psychosis; sensitivity = 20%; specificity = 98%.[31]
  10. Malar rash (rash on cheeks); sensitivity = 57%; specificity = 96%.[31]
  11. Discoid rash (red, scaly patches on skin that cause scarring); sensitivity = 18%; specificity = 99%.[31]

Some people, especially those with antiphospholipid syndrome, may have SLE without four criteria, and SLE is associated with manifestations other than those listed in the criteria.[33][34][35]

Recursive partitioning has been used to identify more parsimonious criteria.[31] This analysis presented two diagnostic classification trees:

1. Simplest classification tree: SLE is diagnosed if a person has an immunologic disorder (anti-DNA antibody, anti-Smith antibody, false positive syphilis test, or LE cells) or malar rash.

2. Full classification tree: Uses 6 criteria.

Other alternative criteria have been suggested.[36]

Treatment

Being a chronic disease with no known cure, the treatment of SLE is symptomatic. In essence, this involves preventing flares and reducing their severity and duration when they occur. Currently, medication is the main form of treatment.

Medications

Due to the variety of symptoms and organ system involvement with SLE, it's severity in an individual must be assessed in order to successfully treat SLE. Mild or remittent disease can sometimes be safely left untreated. If required, nonsteroidal anti-inflammatory drugs and antimalarials may be used.

Disease-modifying antirheumatic drugs

Disease-modifying antirheumatic drugs (DMARDs) are used preventively to reduce the 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 antimalarials and immunosuppressants (e.g. methotrexate and azathioprine). Hydroxychloroquine is an FDA-approved antimalarial used for constitutional, cutaneous, and articular manifestations, whereas cyclophosphamide is used for severe glomerulonephritis or other organ-damaging complications. In 2005, mycophenolic acid became accepted for treatment of lupus nephritis.

Immunosuppressive drugs

In more severe cases, medications that modulate the immune system (primarily corticosteroids and immunosuppressants) are used to control the disease and prevent recurrence of symptoms (known as flares). Depending on the dosage, people that require steroids may develop side-effects such as central obesity, puffy round face, diabetes mellitus, large appetite, difficulty sleeping and osteoporosis. 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.

Analgesia

Since a large percentage of people with SLE suffer from varying amounts of chronic pain, stronger prescription analgesics (pain killers) may be used if over-the-counter drugs (mainly nonsteroidal anti-inflammatory drugs) do not provide effective relief. Moderate pain is typically treated with mild prescription opiates such as dextropropoxyphene and co-codamol. Moderate to severe chronic pain is treated with stronger opioids, such as hydrocodone or longer-acting continuous-release opioids, such as oxycodone, MS Contin, or Methadone. The Fentanyl duragesic transdermal patch is also a widely-used treatment option for the chronic pain caused by complications because of its long-acting timed release and ease of use. When opioids are used for prolonged periods, drug tolerance, chemical dependency, and addiction may occur. Opiate addiction is not typically a concern, since the condition is not likely to ever completely disappear. Thus, lifelong treatment with opioids is fairly common for chronic pain symptoms, accompanied by periodic titration that is typical of any long-term opioid regimen.

Lifestyle changes

Avoiding sunlight is the primary change to the lifestyle of SLE sufferers, as sunlight is known to exacerbate the disease. Drugs unrelated to SLE should be prescribed only when known not to exacerbate the disease. Occupational exposure to silica, pesticides and mercury can also make the disease worsen.[18]

Prevention

SLE 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 remain active, experience less pain, and reduce medical visits.[6]

Complications during pregnancy

While most infants born to mothers, who have SLE, 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 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.[6]

Prognosis

In the 1950s, most people diagnosed with SLE lived fewer than five years. Advances in diagnosis and treatment have improved survival to the point where over 90% 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; however, if symptoms are present after age 60, the disease tends to run a more benign course. The ANA is the most sensitive screening test, whereas 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.[37]

Epidemiology

The rate of SLE varies considerable between countries, by ethnicity, and by gender. It has also changed over time.[38] In the United States the prevalence of SLE is estimated to be about 53 per 100,000[38] well in Northern Europe the rate is about 40 per 100,000 people.[39] This would mean that about 0.3 and 1 million people in the US are effected.[40] SLE occurs with more frequently and with greater severity among those of non-European descent.[40] The rate has been found to be as high as 159 per 100,000 among those of Afro-Caribbean decent.[38]

SLE like many autoimmunie disease affects females more frequently. Between 8 to 9 out of 10 people affected are female.[38]

The incidence of SLE in the United States increased from 1.0 in 1955 to 7.6 in 1974. Whether this is due to better diagnosis or increasing frequency of the disease is unknown.[38]

History and culture

Etymology

There are several explanations ventured for the term lupus erythematosus. Lupus is Latin for wolf, and "erythro" is derived from ερυθρός[] Error: {{Lang}}: no text (help), Greek for "red." All explanations originate with the reddish, butterfly-shaped malar rash that the disease classically exhibits across the nose and cheeks.

  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 directly, but from the term for a French style of mask that 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.

History

The history of SLE can be divided into three periods: 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 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.[41]

Medical historians have theorized that people with porphyria (a disease that shares many symptoms with SLE) 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 disorder, known as dual, homozygous, or compound heterozygous porphyrias).[41]

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 until the middle of the twentieth century, when Hench discovered the efficacy of corticosteroids in the treatment of SLE.[41]

Notable cases

See also

References

  1. ^ a b James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0721629210. Cite error: The named reference "Andrews" was defined multiple times with different content (see the help page).
  2. ^ a b c d e f Anisur Rahman and David A. Isenberg (February 28, 2008). "Review Article: Systemic Lupus Erythematosus". N Engl J Med. 358 (9): 929–939. doi:10.1056/NEJMra071297. PMID 18305268.{{cite journal}}: CS1 maint: date and year (link)
  3. ^ "LUPUS FOUNDATION OF AMERICA". Retrieved 2007-07-04.
  4. ^ a b Harrison's Internal Medicine, 17th ed. Chapter 313. Systemic Lupus Erythematosus.
  5. ^ Discoid Lupus Erythematosus
  6. ^ a b c d "Handout on Health: Systemic Lupus Erythematosus". The National Institute of Arthritis and Musculoskeletal and Skin Diseases. National Institutes of Health. 2003. Retrieved 2007-11-23. {{cite web}}: Unknown parameter |month= ignored (help)
  7. ^ "Lupus, "The Great Imitator"". University Health Care. Retrieved 2009-02-03.
  8. ^ "Lupus: Symptoms - MayoClinic.com". Retrieved 2008-07-14.
  9. ^ a b Joint and Muscle Pain Lupus Foundation of America
  10. ^ 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. doi:10.1056/NEJMoa035611. PMID [http://content.nejm.org/cgi/content/abstract/349/25/2407 Abstract (full text requires registration) 14681506 [http://content.nejm.org/cgi/content/abstract/349/25/2407 Abstract] (full text requires registration)]. {{cite journal}}: Check |pmid= value (help); External link in |pmid= (help)CS1 maint: multiple names: authors list (link)
  11. ^ Bevra Hannahs Hahn, M.D. (2003). "Systemic lupus erythematosus and accelerated atherosclerosis". New England Journal of Medicine. 349 (Dec. 18): 2379–2380. doi:10.1056/NEJMp038168. PMID [http://content.nejm.org/cgi/content/extract/349/25/2379 Extract (full text requires registration) 14681501 [http://content.nejm.org/cgi/content/extract/349/25/2379 Extract] (full text requires registration)]. {{cite journal}}: Check |pmid= value (help); External link in |pmid= (help)
  12. ^ 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. doi:10.1056/NEJMoa035471. PMID [http://content.nejm.org/cgi/content/abstract/349/25/2399 Abstract (full text requires registration) 14681505 [http://content.nejm.org/cgi/content/abstract/349/25/2399 Abstract] (full text requires registration)]. {{cite journal}}: Check |pmid= value (help); External link in |pmid= (help)CS1 maint: multiple names: authors list (link)
  13. ^ "General Pathology Images for Immunopathology". Retrieved 2007-07-24.
  14. ^ "The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes". Arthritis Rheum. 42 (4): 599–608. 1999. doi:10.1002/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F. PMID 10211873. {{cite journal}}: Unknown parameter |month= ignored (help)
  15. ^ Omdal R (2002). "Some controversies of neuropsychiatric systemic lupus erythematosus". Scand. J. Rheumatol. 31 (4): 192–7. PMID 12369649.
  16. ^ D'Cruz DP (2006). "Systemic lupus erythematosus". BMJ. 332 (7546): 890–4. doi:10.1136/bmj.332.7546.890. PMC 1440614. PMID 16613963. {{cite journal}}: Unknown parameter |month= ignored (help)
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  22. ^ Mary K. Crow (February 28, 2008). "Collaboration, Genetic Associations, and Lupus Erythematosus". N Engl J Med. 358 (9): 956–961. doi:10.1056/NEJMe0800096. PMID 18204099.{{cite journal}}: CS1 maint: date and year (link)
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  24. ^ University of South Carolina School of Medicine lecture notes, Immunology, Hypersensitivity reactions. General discussion of hypersensitivity, not specific to SLE.
  25. ^ 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 [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16394661&dopt=Abstract Abstract (full text requires registration) : 1639466 [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16394661&dopt=Abstract Abstract] (full text requires registration)]. {{cite journal}}: Check |pmid= value (help); External link in |pmid= (help)CS1 maint: multiple names: authors list (link)
  26. ^ Gaipl US, Munoz LE, Grossmayer G; et al. (2007). "Clearance deficiency and systemic lupus erythematosus (SLE)". J. Autoimmun. 28 (2–3): 114–21. doi:10.1016/j.jaut.2007.02.005. PMID 17368845. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
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  28. ^ NIM encyclopedic article on the LE cell test
  29. ^ Rheumatology.org article on the classification of rheumatic diseases
  30. ^ Revision of Rheumatology.org's diagnostic criteria
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  32. ^ UpToDate Patient information article on DNA antibodies
  33. ^ 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. doi:10.1191/0961203303lu394oa. PMID 12892393. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  34. ^ 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  35. ^ Hughes GR, Khamashta MA (2003). "Seronegative antiphospholipid syndrome". Ann. Rheum. Dis. 62 (12): 1127. doi:10.1136/ard.2003.006163. PMID 14644846.
  36. ^ Hughes GR (1998). "Is it lupus? The St. Thomas' Hospital "alternative" criteria". Clin. Exp. Rheumatol. 16 (3): 250–2. PMID 9631744.
  37. ^ [EARLY STEROIDS MAY PREVENT RELAPSES IN LUPUS, P Jarman (Published in Journal Watch (General) July 18, 1995)
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  39. ^ Rahman A, Isenberg DA (2008). "Systemic lupus erythematosus". N. Engl. J. Med. 358 (9): 929–39. doi:10.1056/NEJMra071297. PMID 18305268. {{cite journal}}: Unknown parameter |month= ignored (help)
  40. ^ a b "OMHD|AMH|Factsheets|Lupus".
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  42. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Michael Jackson. Updated: August 11, 2008.
  43. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Seal. Updated: August 11, 2008.
  44. ^ A battle with the wolf. Anthony Gardner, Mail on Sunday, October 10, 2008.
  45. ^ Former chess coach named to Hall of Fame. Gary Cramer. Penn State Intercom, September 26, 2002.
  46. ^ J Dilla/Jay Dee. Retrieved February 2, 2009.
  47. ^ 1963: Labour leader Hugh Gaitskell dies. BBC News, On This Day, January 18, 1963.
  48. ^ In the Beginning, 1949~1954 - Teddi King. Allaboutjazz.com. Retrieved February 2, 2009.
  49. ^ Celebrities with Lupus: Charles Kuralt. Updated: August 11, 2008.
  50. ^ Famous Lupus Patient: Ferdinand Marcos. Updated: August 11, 2008.
  51. ^ Flannery O'Connor (1925-1964). New Georgia Encyclopedia. Sarah Gordon, Georgia College and State University. Updated 3/21/2008
  52. ^ Dennis Mclellan. Michael Wayne, 68; Producer, Guardian of His Father’s Legacy. Los Angeles Times, April 4, 2003.
  53. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Ray Walston. Updated: August 11, 2008.
  54. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Tim Raines. Updated: August 28, 2008
  55. ^ Celebrities with Lupus: Mary Elizabeth McDonough. Updated: October 28, 2008.
  56. ^ Biography. Retrieved February 2, 2009.
  57. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Mercedes Scelba-Shorte. Updated: August 28, 2008.
  58. ^ Hirschhorn N, Greaves IA. Louisa May Alcott, her mysterious illness. Perspectives in Biology and Medicine 2007;50(2):243-259.
  59. ^ Angie Davidson interviews top glamour model Sophie Howard Lupus.org.uk, accessed 21 November 2008
  60. ^ Jeri Jewett-Tennant. Celebrities with Lupus: Lauren Schuler Donner. Updated: August 28, 2008
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