Abstract
A 43-year-old Japanese woman with systemic sclerosis visited our clinic with 10 year history of Raynaud’s phenomenon and telangiectasia on the face. At the age of 49, pulmonary hypertension developed, and treatment with ACE inhibitors did not reduce the right ventricular pressure. She suddenly died of respiratory failure at the age of 58. Histopathological examination did not show pulmonary fibrosis, angiitis, embolism, thromboembolism. Autopsy specimens from other organs did not show significant lesions related to sudden death, either. Therefore, we assumed that she might have died of pulmonary arterial spasms.
