4A40.0

Systemic lupus erythematosus

Lúpus eritematoso sistêmico

Category

Definition

Systemic lupus erythematosus is a multisystemic autoimmune disease resulting from immune system–mediated tissue damage. Manifestations can involve the skin, joints, kidney, central nervous system, cardiovascular system, serosal membranes, and the hematologic and immune systems. The disease is highly heterogeneous, with individual patients manifesting variable combinations of clinical features. The molecular triggers of the disease are unknown, but the pathogenesis is understood to involve the production of autoantibodies exhibiting multiple specificities, with reactivity to nucleic acid–binding proteins being a common feature. Immune complexes, together with immune system cells and soluble mediators, generate inflammation and tissue damage.

Diagnostic Criteria

4/11 of the following: malar rash, naso-oral ulcers, photosensitive rash, discoid rash, arthritis, pleuritis or pericarditis, proteinuria (>500 mg/d) or evidence of nephritis in urinalysis, hemolytic anemia, thrombocytopenia, leukopenia, or lymphopenia, seizure or psychosis, positive ANA finding, positive anti–double-stranded DNA, anti-Smith, or antiphospholipid antibody/lupus anticoagulant

Subcategories (1)