Granulomatosis with polyangiitis is a small vessel vasculitis that manifests as multisystemic inflammation predominantly affecting the lungs, upper respiratory tract, and the kidneys. Granulomatosis with polyangiitis commonly presents with elevated inflammatory markers and has a strong association with cytoplasmic antinuclear antibodies. Pulmonary manifestations of the disease include nodules, alveolar hemorrhage, and respiratory failure. The prevalence of pleural involvement is low, but can present as pleural effusion, wall thickening, and rarely pneumothorax. We describe the first report of recurrent pneumothorax secondary to presumed granulomatosis with polyangiitis.
