Vasculitis Syndromes
Autoimmune diseases attributed to fibrinoid degeneration of blood vessels; manifest by arthritis, rash, visceral disease.
Polyarteritis Nodosa
Widespread panarteritis, mainly affecting medium-sized arteries. Onset ages 20 to 50 in >50%. CNS involved in 25%. Systemic manifestations: fever, malaise, tachycardia, sweating, fleeting edema, weakness, pains in joints, muscles, or abdomen.
Neurologic manifestations: mononeuritis multiplex; diffuse sensorimotor peripheral neuropathy. Poor prognosis; 5-year survival 50%.
Treatment: corticosteroids, immunosuppressive drugs.
Temporal Arteritis (Giant-Cell Arteritis)
Inflammation of temporal artery with multinucleated giant cells in media. Onset after age 50, primarily in white people.
Manifestations: headache, unilateral visual loss (14% to 33% of patients), jaw claudication, scalp tenderness in temporal region, malaise, fever, anorexia, weight loss, myalgia. ESR almost always >50 mm/h.
Treatment urgent to prevent visual loss. Early steroid therapy effective, unless visual loss already severe.
Polymyalgia Rheumatica
Churg-Strauss Syndrome and Wegener Granulomatosis

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