2013 Volume 27 Issue 4 Pages 534-537
A 33-year-old male was pointed out as showing a right giant pulmonary bulla on a chest radiograph in a medical examination 4 years ago. He did not undergo treatment until dyspnea on exertion, edema, and cyanosis of the hands presented. He was diagnosed with systemic scleroderma (SSc), and administered NSAIDs and a PGI2 analogue. After the therapy for SSc started, resection of the pulmonary giant bulla was performed by video-assisted thoracic surgery (VATS). Postoperatively, the respiratory symptoms and pulmonary function were improved, and the administration of prednisolone was started for SSc. Sjogren's syndrome is well known as a collagen disease often accompanied with pulmonary bullae; however, SSc with a giant pulmonary bulla is extremely rare. Although the cause of a giant pulmonary bulla with SSc is not clear, damage to the alveolar wall by SSc was thought to be one of the reasons for pulmonary bulla formation.