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Systemic lupus erythematosus: Difference between revisions
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'''Systemic lupus erythematosus''' (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening. | <section begin="dermatology" />'''Systemic lupus erythematosus''' (<abbr>SLE</abbr>) is the prototype of multisystemic autoimmune diseases, affecting all organ systems. Its severity can range from mild to severe and life-threatening. | ||
SLE affects 20 – 100/100 000 in Europe. It more frequently affects women in the ages 16 – 55, being 12x more common in women. Affected men have a worse prognosis. SLE more frequently affects Black and Hispanic ethnicities than White. The 10-year survival rate is 90%. | SLE affects 20 – 100/100 000 in Europe. It more frequently affects women in the ages 16 – 55, being 12x more common in women. Affected men have a worse prognosis. SLE more frequently affects Black and Hispanic ethnicities than White. The 10-year survival rate is 90%. | ||
The most important poor prognostic factors are affection of the kidneys and CNS, and so these are among the most common causes of mortality in SLE patients. SLE doubles the cardiovascular risk as well, which is another common cause of mortality. In the first years, the most common cause of death is infection due to immunosuppression. | The most important poor prognostic factors are affection of the kidneys and CNS, and so these are among the most common causes of mortality in SLE patients. SLE doubles the cardiovascular risk as well, which is another common cause of mortality. In the first years, the most common cause of death is infection due to immunosuppression.<section end="dermatology" /> | ||
== Etiology == | == Etiology == | ||
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* Formation of autoreactive B cells | * Formation of autoreactive B cells | ||
* Formation and deposition of immune complexes | * Formation and deposition of immune complexes | ||
<section begin="dermatology" /> | |||
== Clinical features == | == Clinical features == | ||
Clinical features in SLE occur in phases of remission and relapse. SLE can affect virtually any organ system, but it most frequently affects: | Clinical features in SLE occur in phases of remission and relapse. SLE can affect virtually any organ system, but it most frequently affects: | ||
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The most classical skin symptom in SLE is the malar rash (butterfly rash), which is an erythematous rash on both malar eminences which spares the nasolabial folds. Other skin symptoms include Raynaud phenomenon, photosensitivity, discoid rash, oral ulcers, and alopecia. | The most classical skin symptom in SLE is the malar rash (butterfly rash), which is an erythematous rash on both malar eminences which spares the nasolabial folds. Other skin symptoms include Raynaud phenomenon, photosensitivity, discoid rash, oral ulcers, and alopecia. | ||
The most common musculoskeletal symptom is non-erosive symmetrical [[polyarthritis]] affecting distal joints. | The most common musculoskeletal symptom is non-erosive symmetrical [[polyarthritis]] affecting distal joints.<section end="dermatology" /> | ||
The renal manifestation of lupus is called [[lupus nephritis]], which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome. | The renal manifestation of lupus is called [[lupus nephritis]], which is covered in detail in nephrology. It’s a severe complication and a major cause of death in SLE. 50% of SLE patients develop lupus nephritis at some point, but only 20% have it at the time of diagnosis. The presence of lupus nephritis in a patient with SLE means a worse prognosis and usually alterations in treatment. There are six different classes (types) of lupus nephritis with very variable prognosis. Class I (minimal mesangial type) has the best prognosis, while class IV (diffuse proliferative) has the worst. Clinically, lupus nephritis may present as isolated proteinuria or haematuria, or any nephrological syndrome. | ||
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Any haematological cell line may be affected, causing [[anaemia]], [[leukopaenia]], [[lymphocytopaenia]], or [[thrombocytopaenia]]. <abbr>GI</abbr> manifestations include abdominal pain, [[peritonitis]], [[ascites]], [[Acute pancreatitis|pancreatitis]], and [[hepatitis]]. | Any haematological cell line may be affected, causing [[anaemia]], [[leukopaenia]], [[lymphocytopaenia]], or [[thrombocytopaenia]]. <abbr>GI</abbr> manifestations include abdominal pain, [[peritonitis]], [[ascites]], [[Acute pancreatitis|pancreatitis]], and [[hepatitis]]. | ||
<section begin="dermatology" /> | |||
== Diagnosis and evaluation == | == Diagnosis and evaluation == | ||
SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an [[ANA]] titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive). | SLE is a clinical diagnosis based the presence of certain diagnostic criteria. Previously, the ACR or SLICC criteria were used. However, in 2019, a comprehensive and more sensitive and specific set of criteria was released, the ACR/EULAR criteria. These criteria give points based on typical clinical features, laboratory findings, serology, and renal biopsy findings. The “entry criterion” is an [[ANA]] titre of > 1:80, meaning that this is an obligatory finding for the diagnosis (98% sensitive). | ||
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For induction of remission, corticosteroids are used. In cases of renal or CNS affection, other immunosuppressants like [[cyclophosphamide]] or [[mycophenolate mofetil]] may also be necessary. | For induction of remission, corticosteroids are used. In cases of renal or CNS affection, other immunosuppressants like [[cyclophosphamide]] or [[mycophenolate mofetil]] may also be necessary. | ||
For maintenance of remission, [[hydroxychloroquine]] is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like [[azathioprine]], [[methotrexate]], and sometimes even long-term low-dose glucocorticoids, may be required. [[Belimumab]] and [[rituximab]] are the only biological therapies indicated for SLE. | For maintenance of remission, [[hydroxychloroquine]] is indicated in all patients as it effectively reduces morbidity and mortality. Other immunosuppressants, like [[azathioprine]], [[methotrexate]], and sometimes even long-term low-dose glucocorticoids, may be required. [[Belimumab]] and [[rituximab]] are the only biological therapies indicated for SLE.<section end="dermatology" /> | ||
Supportive treatment includes medications like [[ACE inhibitor|ACE inhibitors]], [[ARB|ARBs]], [[Statin|statins]], [[Antiepileptic drug|antiepileptics]], [[Bisphosphonate|bisphosphonates]], etc. | Supportive treatment includes medications like [[ACE inhibitor|ACE inhibitors]], [[ARB|ARBs]], [[Statin|statins]], [[Antiepileptic drug|antiepileptics]], [[Bisphosphonate|bisphosphonates]], etc. | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||