Spontaneous Pneumothorax in Wegener's Granulomatosis: Case Report and Literature Review

Abstract

Background: Pulmonary manifestations of Wegener's granulomatosis (WG) are present in 45% of cases at the onset of the disease, and they reach 85% of the patients during its evolution. Pulmonary affection usually starts with unspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Pulmonary nodules are 1 of the most common manifestations. The prevalence of pleural affection is 10 to 20%. However, spontaneous pneumothorax is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases.

Objective: To present a new case of spontaneous pneumothorax in a patient suffering WG and a brief review on this subject.

Methods: We report our experience in a case of spontaneous pneumothorax due to WG. We also review the literature through a PubMed search between 1960 and 2010, using a broad range of keywords related to WG and spontaneous pneumothorax. Publications were evaluated for the demographic features of patients, manifestations of the disease, and outcome.

Results: Despite the large prevalence of the respiratory involvement, spontaneous pneumothorax is extremely rare, with only 21 cases reported in the literature.

Conclusion: Pneumothorax is a rare complication in WG cases, without a clear pathogenic mechanism involved.