Systemic lupus erythematosus
Updates to Article Attributes
Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. It is also sometimes classified as a vasculitis.
Epidemiology
There is an overall increased female predilection. In adults, women are affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often. While it can affect any age group, the peak age at onset around the 2nd to 4th decades.
The disease is sometimes classified according to early and late onset groups 8-9.
Pathology
SLE can affect multiple components of the immune system, including the complement system, T-suppressor cells, and cytokine production. It can result in a generation of autoantibodies, which can circulate for many years prior to the development of clinical SLE. The disease tends to have a relapsing and remitting course.
SLE has a myriad of clinical features and radiographic presentations that are probably best discussed under individual subtopics:
- CNS manifestations of systemic lupus erythematosus (CNS lupus): neuropsychiatric events can occur in ~45% (range 14-75%) of cases 6,10
- renal manifestations of systemic lupus erythematosus
- gastrointestinal manifestations of systemic lupus erythematosus: there may be GI involvement in ~20% of cases 5
- thoracic manifestations of systemic lupus erythematosus
- cardiovascular manifestations of systemic lupus erythematosus
- musculoskeletal manifestations of systemic lupus erythematosus
Markers
Approximately 85% (range 70-98%) of patients test positive for anti-dsDNA antibodies 11, and ~20% of Caucasians and ~35% of Asians/blacks test positive for anti-Smith antibodies 12.
Diagnosis
The diagnosis of SLE made be if four of eleven ACR (American College of Rheumatology) criteria are present, either serially or simultaneously 2. These criteria were initially published in 1982 but have been revised in 1997.
ACR criteria:
- malar rash: fixed, flat or raised, occurs over malar eminences and tends to spare the nasolabial folds.
- discoid rash: erythematous raised patches with adherent keratotic scaling and follicular plugging
- photosensitivity
- oral ulcers
- non-erosive arthritis: where there is involvement of two or more peripheral joints with tenderness, swelling or joint effusions; can occur in ~80% of cases 1
- pleuritis and/or pericarditis: present in ~35% (range 17-50%) of cases 1
- proteinuria: >0.5 grams/day
- haematological abnormality
- haemolytic anaemia or
- leukopenia: <4000/mm3 on more than two occasions
- lymphopenia: <1500/mm3 on more than two occasions
- thrombocytopenia: <100,000/mm3 without any precipitant medications
-
neuropsychiatric manifestations
- seizures or psychotic events in the absence of any underlying precipitating drugs or metabolic abnormalities
- immune abnormality on serology
- anti-DNA antibodies or
- anti-Sm antibodies or
- antiphospholipid antibodies (requires certain criteria)
- 27-42% of SLE patient can have the anti-phospholipid antibody syndrome 1
- positive antinuclear antibodies
-<a title="CNS manifestations of systemic lupus erythematosus" href="/articles/cns-manifestations-of-systemic-lupus-erythematosus">CNS manifestations of systemic lupus erythematosus (CNS lupus)</a>: neuropsychiatric events can occur in ~45% (range 14-75%) of cases <sup>6,10</sup>- +<a title="Systemic lupus erythematosus (CNS manifestations)" href="/articles/systemic-lupus-erythematosus-cns-manifestations">CNS manifestations of systemic lupus erythematosus (CNS lupus)</a>: neuropsychiatric events can occur in ~45% (range 14-75%) of cases <sup>6,10</sup>
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