Hughes-Stovin syndrome. Report of a female autopsy case and review of the literature.

Abstract

A 37-year-old female with multiple pulmonary aneurysms, thrombotic obstruction of the inferior vena cava and mural thrombosis in the right heart was reported. Arterial blood culture repeatedly examined had been negative. The patient died of massive hemoptysis. This case was equivalent to Hughes-Stovin syndrome and was the first female case of typical Hughes-Stovin synrome. Review of the literature revealed 9 typical cases of this syndrome and 5 atypical cases who had solitary intrapulmonary aneurysm. It was obscure whether the typical cases were essentially different from the atypical ones or not, but the cases with solitary pulmonary aneurysm might proceed to the cases with multiple ones, as shown in this case. Pathogenesis of the syndrome has been controversial. In the present case, development of pulmonary aneurysms seemed to be closely related to thromboembolization derived from venous thrombosis due to artificial abortion.