A Breach in the Lung: Broncho-Pleural Fistula in the Setting of Granulomatosis With Polyangiitis

A 16-year-old female presented with complaints of a persistent cough for 2 weeks, productive of yellow sputum. She reported subjective low-grade fever for 4 days prior to presenting, with a prior 6-week history of nasal congestion, frontal headaches, and facial pain. ENT review revealed hypertrophy of the right nasal turbinates with features of chronic rhinitis with grossly normal nasal flexiscopy. Imaging revealed focal areas of consolidation in the left lung, with a cavitating lesion. Investigations revealed a leucocytosis, elevated erythrocyte sedimentation rate (ESR), and an elevated proteinase 3-specific antineutrophil cytoplasmic antibodies (cytoplasmic antineutrophil cytoplasmic antibodies) (PR3-ANCA (c-ANCA)). Granulomatosis with polyangiitis (GPA) was suspected, prompting commencement of methylprednisolone 1 g intravenously for three days, then oral prednisone at 1 mg/kg. Five days later, the patient developed a left pneumothorax with partial collapse of the left lower lobe and a likely bronchopleural fistula. Further clinical deterioration led to the patient's transfer to the thoracic surgical unit at another institution. We recommend that clinicians be cognizant of the potential for spontaneous pneumothorax among patients with GPA, especially in the presence of large cavitary nodules. New-onset or worsening chest pain or dyspnoea should prompt further evaluation, and patients must be closely monitored whilst on immunosuppressive therapy.