Wegener Granulomatosis

Abstract

Wegener granulomatosis (WG) is the most common pulmonary idiopathic vasculitis. Pulmonary idiopathic vasculitides are a group of rare diseases. Most of them are linked to the presence of anti-neutrophil cytoplasmic autoantibodies (ANCAs) (Table 24.1), less frequently to the presence of anti-glomerular and/or pulmonary basal membrane (anti-GMB) antibodies. It is a great challenge to diagnose these diseases and differentiate them from other diseases, infections, adverse drug reactions, and several other entities. It is of utmost importance to make timely confirmation of the diagnosis because it is necessary to institute the therapy as soon as possible in order to prevent the lethal outcome due to fatal pulmonary hemorrhage or acute renal failure. The connoisseurship of the clinical features and differential diagnosis as well as rapid screening tests for ANCAs and anti-GMB antibodies are of extreme importance.