Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: a clinical analysis of 25 cases

Objectives: To investigate the risk factors and treatment strategies for pneumothorax secondary to granulomatosis with polyangiitis (GPA). Method: Retrospective analysis of cases with pneumothorax secondary to GPA from our own practice and published on literature. Results: A total of 25 patients, 18 males and 7 females, mean age 44 +/- 15.7 years, were analyzed. Diagnosis included pneumothorax (11 cases), hydropneumothorax (n = 5), empyema (n = 8) and hemopneumothorax (n = 1). 88% (22/25) patients showed single/multiple pulmonary/subpleural nodules with/without cavitation on chest imaging. Erythrocyte sedimentation rate and C-reactive protein were both elevated. Corticosteroids and immunosuppressive agents were used in 16 cases. Five cases received steroid pulse therapy, of which 4 patients survived. Pleural drainage was effective in some patients. Seven patients underwent surgical operations. In the 10 fatal cases, infection and respiratory failure were the most common cause. Lung biopsy/autopsy showed lung/pleural necrotizing granulomatous vasculitis, breaking into the chest cavity, pleural fibrosis, bronchial pleural fistula, etc. The mean age in the death group was greater than the survival group (53 +/- 12.9 years vs 40.1 +/- 14.7 years, p = 0.05), the ineffective pleural drainage was also higher in the death group (5/5 vs 0/7, p = 0.01). Conclusions: Pneumothorax was seen in the active GPA, due to a variety of reasons, and gave rise to high fatality rate. Aggressive treatment of GPA can improve the prognosis. Older and lack of response for pleural drainage indicates poor prognosis.