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.
To present a new case of spontaneous pneumothorax in a patient suffering WG and a brief review on this subject.
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.
Despite the large prevalence of the respiratory involvement, spontaneous pneumothorax is extremely rare, with only 21 cases reported in the literature.
Pneumothorax is a rare complication in WG cases, without a clear pathogenic mechanism involved.