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Clinical Problem Solving: Pathology
May 2013

Pathology Quiz Case 3: Diagnosis

JAMA Otolaryngol Head Neck Surg. 2013;139(5):534-535. doi:10.1001/jamaoto.2013.2871b

Diagnosis: Wegener granulomatosis

Wegener granulomatosis, more recently known as granulomatosis with polyangiitis, is an idiopathic, necrotizing vasculitis that characteristically affects the upper and lower airways and kidneys. This rare disease has an incidence that varies from 1 to 3 cases per million people and typically affects patients ages 20 to 40 years with no sex predominance.1 While the exact etiology of Wegener granulomatosis is unknown, it is thought to be immunologically mediated. Other suspected etiologies include nasal Staphylococcus aureus colonization, drugs, environmental toxins, and genetic factors.2 Wegener granulomatosis is characterized as generalized or limited based on the presence or absence of renal involvement, respectively. Left untreated, generalized cases of Wegener granulomatosis have a reported mean survival of 5 months; therefore, early diagnosis of this condition is essential to patient survival.3

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