Granulomatosis with polyangiitis (GPA) (Wegener's) is a multiorgan system disease of unknown aetiology characterised by granulomatous inflammation, tissue necrosis and vasculitis. The characteristic lung parenchymal lesions of GPA are firm spherical nodules that may cavitate. Pneumothorax (PX) can develop as a quiet rare complication of cavitary nodules. Our case admitted to our clinic with the diagnosis of GPA showing cavitary pulmonary mass. While taking immunosuppressive treatment, spontaneous PX on left lung was developed. A closed chest tube was inserted to the left lung for expansion of PX. Even after 30 days, the left lung did not re-expand and wedge resection with thoracotomy was conducted and the closed chest tube was still in the left lung. On the seventh day, empyema emerged as a complication and, with appropriate treatment, the patient became well. In GPA patients taking immunosuppressive medication, PX is a serious complication and requires aggressive therapy with broad-spectrum antibiotics.
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