Spontaneous pneumothorax in scleroderma

J Clin Rheumatol. 2004 Aug;10(4):207-9. doi: 10.1097/01.rhu.0000135557.49432.56.


We present an illustrative case of a patient with advanced scleroderma who presented with a spontaneous pneumothorax, a condition that has only rarely been reported previously in association with scleroderma. Our patient and those previously reported had advanced pulmonary fibrosis with honeycombing and subpleural cysts, with spontaneous pneumothorax occurring secondary to cyst rupture. Our patient was treated with chest tube thoracostomy, but her spontaneous pneumothorax later recurred. She was then treated with talc pleurodesis and has not had a recurrence in 18 months of follow-up.Spontaneous pneumothorax remains a rare pulmonary manifestation of scleroderma, occurring only in patients with advanced pulmonary fibrosis associated with cyst formation and rupture. Initial management consists of chest tube insertion, but recurrence is high and may require pleurodesis or partial lobectomy. Physicians managing patients with scleroderma should be aware of spontaneous pneumothorax, especially in those with advanced pulmonary fibrosis and subpleural cysts.