Table of Contents

ABSTRACT

This paper considers respiratory aspects of granulomatosis with polyangiitis (GPA), an anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (AAV) formerly known as Wegener’s granulomatosis. Approximately 9 out of 10 persons with GPA have some type of pulmonary or respiratory disease that may include upper or lower respiratory tract features. Upper respiratory tract issues include otitis, hearing impairment, oral/nasal ulcers, rhinitis, sinusitis, saddle-nose deformity and/or subglottic stenosis. Lower respiratory tract issues include hemoptysis, pulmonary hemorrhage, pleurisy, pulmonary nodules and/or pulmonary infiltrates. Topics considered include concepts of epidemiology, clinical features, diagnostic evaluation and management of pulmonary aspects of GPA. A challenging aspect of this condition is the overlapping aspects with other small vessel vasculitides.

Keywords: Granulomatosis with polyangiitis (GPA), Wegener’s granulomatosis, management, United States