Granulomatosis with polyangiitis complicated with bronchopleural fistula.

To cite: Díaz-Correa LM, de León-Tellado N, Ríos G, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014205145 DESCRIPTION Granulomatosis with polyangiitis (GPA) usually involves the upper and/or lower respiratory tracts. Pulmonary manifestations include nodules, pleuritis, fixed infiltrates and alveolar haemorrhage. Bronchopleural fistulas have been reported in GPA but these are very rare. 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 thickening and sclerosis of the walls of the maxillary sinuses, sphenoid sinuses and ethmoidal air cells; nodular mucosal enhancement of the soft tissue lining the maxillary sinuses and the nasal cavity; perforation of the nasal septum; and destruction of the uncinate process and destruction of the medial walls of the maxillary sinuses bilaterally. Nasal mucosa biopsy showed necrosis with a mixed inflammatory infiltrate and multinucleated giant cells consistent with GPA (figure 2). A chest CT scan showed bilateral cavitary nodules, left distal mainstem bronchus stenosis and a left apical bronchopleural fistula (figure 3). Bronchoscopy revealed a collapse of the left main bronchus. Bronchoalveolar lavage (BAL) fluid demonstrated a mixed inflammatory infiltrate and multinucleated giant cells. BAL fluid cultures were negative for bacteria, mycobacteria and fungi. Cytology was negative for malignant cells. She was treated with intravenous methylprednisolone 2 mg/kg for 7 days followed by prednisone 1 mg/kg/day. The prednisone dose was decreased by 5 mg every 2–4 weeks down to 25 mg. Also, she Figure 1 Saddle nose deformity and bilateral periorbital swelling, chemosis, dacryocystitis and scleritis. Figure 2 Area showing necrosis, abundant neutrophils, eosinophils and plasma cells. Multinucleated giant cells are also present (arrow; H&E stain, ×400).