Immune-Mediated Rheumatic Diseases

Abstract

Systemic lupus erythematosus (SLE) is the prototypical human, autoimmune disease and is characterized by immune dysregulation, production of autoantibodies, generation of circulating immune complexes, and activation of the complement system. SLE is notable for unpredictable exacerbations and remissions with a predilection for clinical involvement of joint, skin, kidney, brain, serosa, lung, heart, hematological system, and gastrointestinal tract (Wallace, The clinical presentation of systemic lupus erythematosus. In: Wallace D and Hahn B, Eds. Dubois’ Lupus Erythematosus, Lippincott Williams & Wilkins, Hagerstown, MD, 2006, pp. 621–628) (Table 19.1). The pathological hallmark of SLE is recurrent, widespread, and diverse vascular lesions including both inflammatory and thrombotic vasculopathy, the latter most often occurring in the setting of antiphospholipid antibodies. Cases related to the presentation and management of SLE are discussed (Belmont and Abramson, Arthritis and Rheumatism 39:9–23, 1996). Table 19.1 Major clinical manifestations of systemic lupus erythematosus by organ system Constitutional Fever, weight loss, malaise, and fatigue Musculoskeletal Myalgia, arthralgia, deforming non-erosive arthritis (Jaccoud’s arthropathy), erosive arthritis [RA-like (rare)] myositis Mucocutaneous Acute cutaneous lupus (malar rash), subacute cutaneous lupus (annular rash), chronic cutaneous lupus (discoid lupus), painless oral and nasal ulcers, urticaria, tumid lupus, bullous lupus Cardiopulmonary Pericarditis, myocarditis, valvulitis, pleuritis, pneumonitis, pulmonary hypertension Hematologic Anemia of chronic disease, autoimmune hemolytic anemia, autoimmune thrombocytopenia purpura, Evan’s syndrome, leukopenia, lymphopenia, TTP, hypercoagulable state in setting of antiphospholipid antibodies Neuropsychiatric Acute confusional state, organic mental syndrome, organic affective disorder, seizures, cerebrovascular accident, cranial neuropathy, mononeuritis multiplex, transverse myelitis Vasculitic (rare) Cutaneous vasculitis, coronary vasculitis, dysenteric vasculitis, intracerebral vasculitis (very rare) Renal Nephrotic syndrome, glomerulonephritis, azotemia, hypertension, end stage renal disease Gastrointestinal Medical peritonitis, pancreatitis (rare), non-specific hepatitis, esophageal dysfunction (rare)