Granulomatosis with Polyangiitis – Formerly Known as Wegener’s Granulomatosis with Limited Manifestation Affecting Only Respiratory System

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is the most frequently occurring ANCA-associated vasculitis. As clinically incomplete, limited forms, we encounter relatively frequently with respiratory tract involvement, and not so rarely without the involvement of the lower respiratory tract or with minimal nose or ear symptoms. Positivity of anti-neutrophil cytoplasmic antibodies (ANCA) can be missing in isolated lung involvement. Patients with limited pulmonary involvement have a better prognosis than those with manifested renal involvement. DAH is a serious condition and only rarely does it occur without simultaneous renal involvement. Patients with limited involvement of the upper and lower respiratory tracts and lungs without DAH have a significantly better prognosis.