Granulomatosis with polyangiitis complicated with bronchopleural fistula.

Granulomatosis with polyangiitis (GPA) usually involves the upper and/or lower respiratory tracts. Pulmonary manifestations include nodules, pleuritis, fixed infiltrates and alveolar haemorrhage.1 Bronchopleural fistulas have been reported in GPA but these are very rare.2 ,3 We present a 58-year-old woman who had a 1-year history of constitutional symptoms, chronic sinusitis, decreased hearing, cough and dyspnoea. On examination, she had a saddle nose deformity, and a bilateral periorbital swelling, chemosis, dacryocystitis and scleritis (figure 1). Bilateral middle ear effusion was observed. Prolonged expiration and wheezing were noticed. Laboratory workup was unremarkable except for elevated erythrocyte sedimentation rate (ESR=94 mm/h) and positive antiproteinase-3 antibodies (by ELISA). The Mantoux test was negative. A maxillofacial CT performed before treatment showed an extensive …