Disease Information for Wegeners granulomatosis: Definition

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  • Wegener"s granulomatosis; Klinger"s syndrome; Klinger-Wegener syndrome; Wegener"s disease; Wegener"s syndrome;

    Wegener-Churg-Klinger syndrome; Wegener-Klinger syndrome;

    Arteritis-pulmonary-nephropathy syndrome; classical granulomatosis; glomerulonephritis syndrome; granuloma-arteritis; granulomas-arteritis-glomerulonephritis syndrome; granulomata-arteritis-glomerulonephritis syndrome; granuloma gangraenescens; granulomatosis pathergic syndrome; malignant granulomatous angiitis; necrotizing granulomatosis; necrotizing respiratory granulomatosis; rhinogenic polyarteritis;

    An uncommon disorder characterized by necrotizing vasculitis, granulomatous lesions of the entire respiratory tract, and glomerulonephritis; The lesions closely resemble those in polyarteritis nodosa; The symptoms are weakness, malaise, progressive weight loss, purulent rhinitis, sinusitis, polyarthralgia, ulcerations of the nasal septum, signs of severe progressive renal disease, and fever; The disease commonly occurs in mid-adult life; Either sex may be affected; If it is not treated early, death occurs in a matter of months, most often from kidney damage;

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    Renal disease often rapidly progressive without treatment;

    Without treatment, generalized disease is invariably fatal, with most patients surviving less than 1 year after diagnosis; It occurs most commonly in the fourth and fifth decades of life and affects men and women with equal frequency; The disorder usually develops over 4-12 months, with 90% of patients presenting with upper or lower respiratory tract symptoms or both; Upper respiratory tract symptoms can include nasal congestion, sinusitis, otitis media, mastoiditis, inflammation of the gums, or stridor due to subglottic stenosis; Since many of these symptoms are common, the underlying disease is not often suspected until the patient develops systemic symptoms or the original problem is refractory to treatment; The lungs are affected initially in 40% and eventually in 80%, with symptoms including cough, dyspnea, and hemoptysis; Other early symptoms can include unilateral proptosis (from pseudotumor), red eye from scleritis, arthritis, purpura, and dysesthesia due to neuropathy; Renal involvement, which develops in three-fourths of the cases, may be subclinical until renal insufficiency is advanced. Fever, malaise, and weight loss are common;

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