Hughes-Stovin Syndrome: A Case Report on a Rare, Life-Threatening Vasculitis.

Abstract

Hughes-Stovin Syndrome (HSS) is a rare vasculitic disorder characterised by widespread pulmonary artery aneurysms. It shares some features with Behçet disease. Currently, the diagnosis is based on clinical suspicion. Our case describes a young male who presented with haemoptysis and previous history of pulmonary embolism. Workup was essentially unremarkable, but imaging revealed multiple pulmonary artery aneurysms. Timely initiation of glucocorticoids and immunosuppression with cyclophosphamide led to improvement. High-dose glucocorticoids and immunosuppressants are the mainstays of treatment. Untreated cases can result in fatal outcomes.