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Systemic lupus erythematosus

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Lupus erythematosus
Classification and 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, pronounced /sɪˈstɛmɪk ˈluːpəs ˌɛrə

ˌθiməˈtoʊsəs/) is a chronic autoimmune disease that can be fatal; however, 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 remissions.
Lupus can occur at any age, and is most common in women, particularly of non-
European descent.[1] Lupus is treatable through addressing its symptoms, mainly with
corticosteroids and immunosuppressants; however there is currently no cure. Survival in
patients with SLE in the United States, Canada, and Europe is approximately 95% at 5
years, 90% at 10 years, and 78% at 20 years.[2]

Contents
[hide]
 • 1 Classification
• 2 Signs and symptoms
o 2.1 Common symptoms explained
• 3 Causes
• 4 Pathophysiology
o 4.1 Abnormalities in apoptosis
• 5 Diagnosis
o 5.1 Diagnostic criteria
 5.1.1 Alternative criteria
• 6 Treatment
o 6.1 Drug therapy
o 6.2 Lifestyle changes
• 7 Prevention
o 7.1 Complications during pregnancy
o 7.2 Prognosis
• 8 Epidemiology
• 9 History
o 9.1 Origins of "lupus erythematosus"
• 10 Notable patients
• 11 See also
• 12 References

• 13 External links

[edit] Classification
Lupus is a chronic autoimmune disease. It can affect multiple organ systems, including
the heart, skin, joints, kidneys, and nervous system. There are several types of lupus; in
general, when the word lupus alone is used, reference is to systemic lupus
erythematosus or SLE, as discussed in this article. Other types include:

• Drug-induced lupus erythematosus, a drug-induced form of SLE; this type of

lupus can occur equally in either sex.
• Lupus nephritis, an inflammation of the kidneys caused by SLE.
• Discoid lupus erythematosus, a skin disorder that causes a red, raised rash on the
face and scalp. Discoid lupus occasionally (1–5%) develops into SLE.[3]
• Subacute cutaneous lupus erythematosus, which causes nonscarring skin
lesions on patches of skin exposed to sunlight.[4]
• 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.[4]

[edit] Signs and symptoms

SLE is one of several diseases known as "the great imitators"[5] because its symptoms
vary so widely, it often mimics or is mistaken for other illnesses 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, 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.[6]

[edit] 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 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.[7] 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.[7]
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), wherein
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 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 in SLE patients than in the general
population.[8][9][10]
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.
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.
A histological hallmark of SLE is membranous glomerulonephritis with "wire
loop" abnormalities.[11] 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. One-third may test
positive for abnormalities in the cerebrospinal fluid.
T-cell abnormalities
Abnormalities in T cell-signaling are associated with SLE, including a 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:

• 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

[edit] Causes
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
(but probably include viruses) in people with certain combinations of genes in their
immune system.

"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. [12][13][14]

SLE is a chronic inflammatory disease believed to be a type III hypersensitivity response

with potential type II involvement.[15]

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
developing lupus when triggered by environmental factors. The most important
genes are located on chromosome 6, where mutations may occur randomly (de
novo) or may be inherited. In addition, 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 that 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, in general, disappear
 once a patient is taken off the medication that triggered the episode. There are
about 400 medications currently in use that can cause this condition, the most
common of which are procainamide, hydralazine, quinidine, and Phenytoin.
Non-SLE forms of lupus
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 patients is negative or a low-titer positive. About 1–5% of discoid
lupus patients eventually develop SLE.
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 and scant
serum components in addition to increased apoptosis.

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.

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
patients with cutaneous lupus erythematosus (CLE).[16]

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 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 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.

[edit] Pathophysiology
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.
[edit] Abnormalities in apoptosis

• Apoptosis is increased in monocytes and keratinocytes

• Expression of Fas by B cells and T cells is increased
• There are correlations between the apoptotic rates of lymphocytes and disease
activity.

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 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 that
may have randomly acquired self-specificity through somatic hypermutation.[17]

[edit] Diagnosis

Microphotograph of a histological section of human skin prepared for direct

immunofluorescence using an anti-IgG antibody. The skin is from a patient 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).

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.

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 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.
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 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.[18]

[edit] Diagnostic criteria

The American College of Rheumatology (ACR) established eleven criteria in 1982,[19]

which were revised in 1997[20] 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 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).[21]
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%.[21]
4. Photosensitivity (exposure to ultraviolet light causes skin rash, or other symptoms
of Lupus flareups); sensitivity = 43%; specificity = 96%.[21]
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%.[21] 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%.[21]
7. Antinuclear antibody test positive; sensitivity = 99%; specificity = 49%.[21]
8. Immunologic disorder: Positive anti-Smith, anti-ds DNA, antiphospholipid
antibody, and/or false positive serological test for syphilis; sensitivity = 85%;
specificity = 93%.[21] Presence of anti-ss DNA in 70% of patients (though also
positive in patients with rheumatic disease and healthy persons[22]).
9. Neurologic disorder: Seizures or psychosis; sensitivity = 20%; specificity =
98%.[21]
10. Malar rash (rash on cheeks); sensitivity = 57%; specificity = 96%.[21]
11. Discoid rash (red, scaly patches on skin that cause scarring); sensitivity = 18%;
specificity = 99%.[21]

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).

Another popular mnemonic is DOPAMIN RASH: Discoid rash (2), Oral ulcers (4),
Photosensitivity (3), Arthritis (5), Malar Rash (1), Immunological changes (11),
Neurological signs (seizures, frank psychosis) (7), Renal involvement (proteinuria or
casts) (6), ANA (10), Serositis (8), Hematological Changes (5).

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.[23][24][25]

[edit] Alternative criteria

Recursive partitioning has been used to identify more parsimonious criteria.[21] This
analysis presented two diagnostic classification trees:

1. Simplest classification tree: SLE 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.

• sensitivity = 92%
• specificity = 92%

2. Full classification tree: Uses 6 criteria.

• sensitivity = 97%
• specificity = 95%

Other alternative criteria have been suggested.[26]

[edit] Treatment
As lupus erythematosus is a chronic disease with no known cure, treatment is restricted to
dealing with the symptoms. In essence, 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.

[edit] 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,
nonsteroidal anti-inflammatory drugs and antimalarials may be used.
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 (trade name Plaquenil) is an FDA-approved antimalarial used for
constitutional, cutaneous, and articular manifestations, whereas cyclophosphamide (trade
names Cytoxan and Neosar) is used for severe glomerulonephritis or other organ-
damaging complications. In 2005, mycophenolic acid (trade name CellCept) became
accepted for treatment of lupus nephritis.

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, patients 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.

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
nonsteroidal anti-inflammatory drugs) do not provide effective relief. Moderate pain in
lupus patients is 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 name OxyContin), MS Contin, or Methadone.
The Fentanyl Duragesic Transdermal patch is also a widely-used treatment option for the
chronic pain of lupus complications because of its long-acting timed release and ease of
use. 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.

[edit] Lifestyle changes

Other measures, such as avoiding direct sunlight, covering up with sun-protective

clothing, and using strong UVA/UVB sunblock lotion can also be effective in preventing
photosensitivity problems. 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.

[edit] 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.[4]

[edit] 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 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.[4]

[edit] 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; 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, wheres
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.[27]

[edit] 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 with some certainty. 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.[28] Worldwide, a
conservative estimate states that over 5 million people have lupus.

SLE affects 1 in 4000 people in the United States, and, although SLE can occur in
anyone, at any age, it is most common in women of childbearing age. The disease
appears to be more prevalent in women of African, Asian, Hispanic, and Native American
origin, which may be due to socioeconomic factors. People with relatives that suffer from
SLE, rheumatoid arthritis, or thrombotic thrombocytopenic purpura are at a slightly
higher risk than the general population.

[edit] History
Medical historians have theorized that people with porphyria (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 disorder, known as dual,
homozygous, or compound heterozygous porphyrias).

The history of lupus erythematosus 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.

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.

[edit] Origins of "lupus erythematosus"

This section does not cite any references or sources.

Please help improve this section by adding citations to reliable sources. Unverifiable material may be
challenged and removed. (May 2008)

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.

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.

[edit] Notable patients

• Stephanie Smith, artist who died of SLE complications in 1969 at the age of 22.
The anti-Smith (or anti-Sm) antigen was discovered in her and is the basis of a
SLE diagnostic test.
• Caroline Dorough-Cochran, sister of Howie D. of the Backstreet Boys, who
founded the Dorough Lupus Foundation in her memory.
• Louisa May Alcott, American author.
• Inday Ba (also known as N'Deaye Ba), a Swedish-born actress who died from
complications of lupus at age 32.
• Donald Byrne, American chess player who died from complications of lupus in
1976.
• J Dilla (also known as Jay Dee), a hip-hop producer and beatmaker who died of
the disease in 2006.
• Hugh Gaitskell, British politician.
• Sophie Howard, British glamour model.
• Michael Jackson, pop superstar, diagnosed with the disease in 1984.
• Charles Kuralt, former anchor of CBS Sunday Morning, died of the disease in
1997.
• Ferdinand Marcos, former Philippine president, who died from complications of
lupus in 1989.
• Mary Elizabeth McDonough, American actress.
• Flannery O'Connor, American fiction writer who died of the disease in 1964.
• Elaine Paige, British actress and singer.
• Tim Raines, former major league baseball player, primarily with the Montreal
Expos and Chicago White Sox.
• Mercedes Scelba-Shorte, America's Next Top Model Season Two runner-up and
model.
• Seal, singer (discoid lupus of the face, leading to scarring)
• Fred Williamson
• Ray Walston, character actor.

[edit] See also

• Abzyme
• Alliance for Lupus Research
• Antinuclear antibody
• Canine discoid lupus erythematosus in dogs
 • Lupus Canada
• Lupus Foundation of America

[edit] References
1. ^ "LUPUS FOUNDATION OF AMERICA". Retrieved on 2007-07-04.
2. ^ Harrison's Internal Medicine, 17th ed. Chapter 313. Systemic Lupus
Erythematosus.
3. ^ Discoid Lupus Erythematosus
4. ^ 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.
5. ^ Lupus: The Great Imitator
6. ^ "Lupus: Symptoms - MayoClinic.com". Retrieved on 2008-07-14.
7. ^ a b Joint and Muscle Pain Lupus Foundation of America
8. ^ 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 14681506 Abstract (full text requires
registration).
9. ^ 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 14681501 Extract (full text
requires registration).
10. ^ 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 14681505 Abstract (full text requires registration).
11. ^ "General Pathology Images for Immunopathology". Retrieved on 2007-07-24.
12. ^ 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.
13. ^ 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.
14. ^ Geoffrey Hom, Robert R. Graham, Barmak Modrek, et al. (February 28, 2008).
"Association of Systemic Lupus Erythematosus with C8orf13–BLK and ITGAM–
ITGAX". N Engl J Med 358 (9): 900–909. doi:10.1056/NEJMoa0707865. PMID
18204098.
 15. ^ University of South Carolina School of Medicine lecture notes, Immunology,
Hypersensitivity reactions. General discussion of hypersensitivity, not specific to
SLE.
16. ^ 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.
17. ^ 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).
18. ^ NIM encyclopedic article on the LE cell test
19. ^ Rheumatology.org article on the classification of rheumatic diseases
20. ^ Revision of Rheumatology.org's diagnostic criteria
21. ^ 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.
22. ^ UpToDate Patient information article on DNA antibodies
23. ^ 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.
24. ^ 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.
25. ^ Hughes GR, Khamashta MA (2003). "Seronegative antiphospholipid
syndrome". Ann. Rheum. Dis. 62 (12): 1127. doi:10.1136/ard.2003.006163. PMID
14644846.
26. ^ Hughes GR (1998). "Is it lupus? The St. Thomas' Hospital "alternative"
criteria". Clin. Exp. Rheumatol. 16 (3): 250–2. PMID 9631744.
27. ^ [EARLY STEROIDS MAY PREVENT RELAPSES IN LUPUS, P Jarman
(Published in Journal Watch (General) July 18, 1995)
28. ^ Lupus and African-American women

[edit] External links

• Systemic lupus erythematosus at the Open Directory Project
• Alliance for Lupus Research
• The Lupus UK Site
• Resources for Lupus
 [show]
v•d•e
Diseases of the skin and appendages by morphology

verruca, clavus, seborrheic keratosis, acrochordon, molluscum

Epidermal contagiosum, actinic keratosis, squamous cell carcinoma, basal cell
carcinoma, merkel cell carcinoma, nevus sebaceous, trichoepithelioma

Pigmented ephelis, lentigo, melasma, nevus, malignant melanoma

epidermal inclusion cyst, hemangioma, dermatofibroma, keloid, lipoma,

neurofibroma, xanthoma, Kaposi's sarcoma, infantile digital
Dermal and subcutaneous
fibromatosis, granular cell tumor, leiomyoma, lymphangioma
circumscriptum, myxoid cyst

essential dermatitis, contact dermatitis, atopic

dermatitis, seborrheic dermatitis, stasis
dermatitis, lichen simplex chronicus, Darier's
Eczematous disease, glucagonoma syndrome, langerhans cell
histiocytosis, lichen sclerosus, pemphigus
foliaceus, Wiskott-Aldrich syndrome, Zinc
deficiency

psoriasis, tinea corporis, tinea cruris, tinea pedis,

tinea manuum, tinea faciale, pityriasis rosea,
Scaling secondary syphillis, mycosis fungoides,
systemic lupus erythematosus, pityriasis rubra
pilaris, parapsoriasis, icthyosis

herpes simplex, herpes zoster, varicella, bullous

impetigo, acute contact dermatitis, pemphigus
Blistering vulgaris, bullous pemphigoid, dermatitis
With epidermal involvement
herpetiformis, porphyria cutanea tarda,
epidermolysis bullosa simplex

scabies, insect bite reactions, lichen planus,

miliaria, keratosis pilaris, lichen spinulosus,
Papular
transient acantholytic dermatosis, lichen nitidus,
pityriasis lichenoides et varioliformis acuta

acne vulgaris, acne rosacea, folliculitis,

impetigo, candidiasis, gonococcemia,
Pustular
dermatophyte, coccidioidomycosis, subcorneal
pustular dermatosis

tinea versicolor, vitiligo, pityriasis alba,

 [show]
v•d•e
Diseases of the skin and subcutaneous tissue (integumentary system) (L,
680-709)

Atopic dermatitis - Seborrhoeic dermatitis (Dandruff,

Cradle cap) - Contact dermatitis (Diaper rash,
Urushiol-induced contact dermatitis) - Erythroderma -
Dermatitis and eczema
Lichen simplex chronicus/Prurigo nodularis - Itch
(Pruritus ani) - Nummular dermatitis - Dyshidrosis -
Pityriasis alba

Psoriasis (Psoriatic arthritis) - Parapsoriasis (Pityriasis

lichenoides et varioliformis acuta, Pityriasis
lichenoides chronica, Lymphomatoid papulosis) -
Papulosquamous disorders
other pityriasis (Pityriasis rosea, Pityriasis rubra
pilaris) - other lichenoid (Lichen planus, Lichen
nitidus)

Dermatographic urticaria - Cold urticaria - Cholinergic

Urticaria
urticaria

Erythema multiforme/drug eruptions: Stevens-Johnson

syndrome - Toxic epidermal necrolysis - Erythema
nodosum
Erythema
Other erythema: Erythema annulare centrifugum -
Erythema marginatum - Necrolytic migratory erythema
- Erythema toxicum

Ingrown nail - Onychogryposis - Beau's lines - Yellow nail syndrome

Nail
- Leukonychia

Alopecia areata (Alopecia totalis, Alopecia universalis,

Ophiasis)
Hair lossAndrogenic alopecia - Hypotrichosis - Telogen effluvium -
Traction alopecia - Lichen planopilaris - Trichorrhexis
nodosa

Hypertrichosis (Hirsutism)

Acneiform eruption (Acne vulgaris, Chloracne, Blackhead)

 [show]
v•d•e
Systemic CT disorders (M30-M36, 710, 440-448)

Large vesselTakayasu's arteritis · Temporal arteritis

Medium vesselPolyarteritis nodosa · Wegener's granulomatosis · Kawasaki disease

Churg-Strauss syndrome · Microscopic polyangiitis ·

Small vessel
Hypersensitivity vasculitis

[show]
v•d•e
Immune disorders: Hypersensitivity and autoimmune diseases

Retrieved from "http://en.wikipedia.org/wiki/Systemic_lupus_erythematosus"

Categories: Skin diseases | Arthritis | Diseases involving the fasciae | Rheumatology |
Nephrology | Autoimmune diseases | Diseases
Hidden category: Articles needing additional references from May 2008

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