Wegener's granulomatosis: report of a patient surviving four and a half years

Abstract

A patient with Wegener's granulomatosis, treated with steroids on a long-term basis, survived at least four and a half years from onset until death occurred in an uncontrollable exacerbation of the disease process. This is believed to be the longest recorded survival.Histological examination of the necropsy specimens suggests that the basic pathological lesion in this disease is the subendothelial deposition of so far unidentified material in the capillaries, arterioles, venules, and smaller arteries. The associated angiitis may be a consequence of this deposition. These vascular lesions are occulusive and adequately account for the observed infarctive changes. The granulomatous features of the disease probably represent a secondary reactive phenomenon.