Systemic lupus erythematosus (pronounced /sɨˈstɛmɨk ˈluːpəs ˌɛrɨˌθiːməˈtoʊsəs/ ( listen)), often abbreviated
to SLE or lupus, is a systemic autoimmune disease (or 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.[2] It is a Type III hypersensitivity reaction caused by
antibody-immune complex formation.
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 in women in child-bearing years ages 15
to 35, and is more common in those also of non-European descent.[3][4][5]
SLE is treatable through addressing its symptoms, mainly with cyclophosphamide, 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.[5]
Signs and symptoms
Common symptoms of SLE.[6]
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,[3] 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 include 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
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
The most commonly sought 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 of those affected 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]
SLE patients are at particular risk of developing osteoarticular tuberculosis.[10]
It is suggested that there might be an association between rheumatoid arthritis and SLE,[11] and that SLE is
associated with an increased risk of bone fractures in relatively young women.[12]
Hematological
Anemia may develop in up to 50% of cases. 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[13] (a thrombotic disorder), wherein autoantibodies to phospholipids are present in their
serum. Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged
PTT Partial thromboplastin time (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
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.[14][15][16]
Pulmonary
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
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.[17] This
finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical
granular appearance in immunofluorescence testing.
Neuropsychiatric
to SLE or lupus, is a systemic autoimmune disease (or 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.[2] It is a Type III hypersensitivity reaction caused by
antibody-immune complex formation.
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 in women in child-bearing years ages 15
to 35, and is more common in those also of non-European descent.[3][4][5]
SLE is treatable through addressing its symptoms, mainly with cyclophosphamide, 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.[5]
Signs and symptoms
Common symptoms of SLE.[6]
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,[3] 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 include 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
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
The most commonly sought 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 of those affected 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]
SLE patients are at particular risk of developing osteoarticular tuberculosis.[10]
It is suggested that there might be an association between rheumatoid arthritis and SLE,[11] and that SLE is
associated with an increased risk of bone fractures in relatively young women.[12]
Hematological
Anemia may develop in up to 50% of cases. 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[13] (a thrombotic disorder), wherein autoantibodies to phospholipids are present in their
serum. Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged
PTT Partial thromboplastin time (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
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.[14][15][16]
Pulmonary
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
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.[17] This
finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical
granular appearance in immunofluorescence testing.
Neuropsychiatric
