Systemic Lupus Erythematosus (SLE)

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. Its multisystem manifestations and attendant complications from use of immunosuppressive agents make the diagnosis and management of this entity challenging.

Pathophysiology: Autoantibodies, circulating immune complexes, and T lymphocytes all contribute to the expression of disease. Organ systems affected include dermatologic, renal, central nervous system (CNS), hematologic, musculoskeletal, cardiovascular, pulmonary, the vascular endothelium, and gastrointestinal. The revised criteria for SLE must include 4 of the following at any time during a patient's history (specificity 95% and sensitivity 75%):

* Malar rash

* Discoid rash

* Photosensitivity

* Oral ulcers

* Arthritis

* Serositis

* Renal disorder

* Neurologic disorder

* Hematologic disorder

* Immunologic disorder

* Antinuclear antibody

Frequency:

* In the US: Because of variable reporting, the overall prevalence ranges from 14.6-50.8 cases per 100,000. Incidence varies from 1.8-7.6 cases per 100,000 per year.

* Internationally: Incidence varies worldwide. In Northern Europe, it has been reported to be 40 cases per 100,000.

Mortality/Morbidity: Recent studies in Europe and Canada have shown a reduction in mortality in patients with lupus with 20-year survival rates close to 70% and 10-year survival rates ranging from 75-85%, with more than 90% of patients surviving more than 5 years.

* Early deaths usually are caused by active disease. Atherosclerosis is a leading cause in late deaths. Infection and nephritis are major causes of mortality in all stages of SLE.

* After dialysis or transplantation, a reduction in disease activity and flares has been reported.

* Thrombosis, often secondary to antiphospholipid syndrome, carditis, pneumonitis, pulmonary hypertension, stroke, myocardial infarction, and cerebritis cause severe morbidity and mortality.

Race: SLE is more common in blacks (1:250) than in whites (1:1000). However, all ethnic groups are susceptible.

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