Granulomatosis

Abstract

The diagnosis of Wegener’s granulomatosis (WG) began with a clinicopathological triad of necrotizing granulomatous inflammation of the upper and lower respiratory tracts, angiitis, and necrotizing glomerulonephritis based on tissue examined at autopsy. However, only one-half of the patients in the initial series of Godman and Churg had the complete triad. Limited and ulcerative or tumefactive forms can occur in many organs. Initial biopsy diagnosis of established disease was followed by the early histological diagnosis of limited disease. This was followed by diagnosis on the basis of anti-neutrophilic cytoplasmic antibody (ANCA) without tissue confirmation. Today, therapy prevents most cases from progressing to multi-organ involvement. The most common cause of death is renal failure resulting from glomerulonephritis, and this can occur with days or even hours, so the pathological evaluation of a lung biopsy for WG is a medical emergency. Pulmonary and cardiovascular complications of the disease are much less common causes of morbidity or mortality. The evolution of the ANCA test has provided substantiating evidence for cases with equivocal histology and for separation of WG from microscopic polyangiitis and from other forms of disseminated vasculitis.