Hughes-Stovin syndrome: A case report

Abstract

Hughes-Stovin syndrome (HSS) is a rare autoimmune disorder, characterized by deep venous thrombosis (DVT) and pulmonary and/or bronchial aneurysms. The pathogenesis is not clear, but it is assumed to be a consequence of angiodysplasia and vasculitis similar to those in Behcet disease. Case Report: A 70-year old male was admitted because of haemoptysis. He had sympathectomy at the age of 42 due to arteritis. Since that he had had several episodes of DVT. He had no oral or genital ulcers. CT aortography showed the aneurism of the right bronchial artery. CT pulmonary angiography demonstrated an aneurismatic dilatation of the pulmonary artery. Bronchoscopy showed compression of the intermediate bronchus and distal part of the left main bronchus with dilated submucosal blood vessels that were bleeding lightly to the touch of the bronchoscope. Immunological tests revealed antiphospholipid syndrome. Clinical and radiological work-up confirmed the diagnosis of HSS. We started treatment with corticosteroids, cyclophosphamide, and anticoagulants. At the beginning of treatment hemoptysis stopped and skin vasculitis disappeared. However, the patient died 4 months later due to pulmonary hemorrhage. Conclusion: HSS is a rare type of vasculitis. Less than 50 cases has been published in the literature, with most of the patients being young males aged from 20 to 40 years. Our patient is an example that HSS can be diagnosed in the elderly, although the symptoms had started much earlierThe management of HSS includes both medical (immunosuppressives) and surgical (resection, embolization) options. However, massive pulmonary bleeding remains the most common cause of the death in those patients.