Recurrent Churg-Strauss vasculitis. With exophthalmos, hearing loss, nasal obstruction, amyloid deposits, hyperimmunoglobulinemia E, and circulating immune complexes.
Churg-Strauss vasculitis in remission for 4 1/2 years recurred, with new, previously undescribed features, after a lapse in corticosteroid therapy. Bilateral exophthalmos, unilateral hearing loss, and nasal obstruction accompanied fever, severe asthma, and palpable purpura. Leukocytosis with eosinophilia, an elevated ESR, hyperimmunoglobulinemia E, an pulmonary infiltrates were seen again. Circulating immune complexes were detected, and microamyloid deposits were found in the conjunctiva and skin. All clinical and laboratory manifestations responded to corticosteroid therapy. Monitoring the levels of IgE may add a diagnostic and prognostic feature to the classification of necrotizing vasculitides.
D000328:Adult; D000682:Amyloid / Q000032:analysis; D000936:Antigen-Antibody Complex; D001249:Asthma / Q000150:complications; D005094:Exophthalmos / Q000150:complications; D005260:Female; D034381:Hearing Loss / Q000150:complications; D006801:Humans; D006854:Hydrocortisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D007073:Immunoglobulin E / Q000032:analysis; D009668:Nose Diseases / Q000150:complications; D010488:Polyarteritis Nodosa / Q000188:drug therapy / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D013577:Syndrome
D000682:Amyloid; D000936:Antigen-Antibody Complex; D007073:Immunoglobulin E; C007133:hydrocortisone hemisuccinate; D011241:Prednisone; D006854:Hydrocortisone