CASE REPORT Hughes–Stovin syndrome: is it incomplete Behçet’s? Report of two cases and review of the literature Yasser Emad & Yasser Ragab & Abd El-Hakim Shawki & Tamer Gheita & Ahmed El-Marakbi & Mohamed Hanfi Salama Received: 14 February 2007 / Revised: 13 March 2007 / Accepted: 14 March 2007 / Published online: 25 April 2007 # Clinical Rheumatology 2007 Abstract In this report, we described two male patients with Hughes–Stovin syndrome. The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis. Pulmonary CT angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis. While the second case was a 16-year-old male patient who initially presented with generalized fits due to sagittal sinus thrombosis and later followed by DVT in the left lower limb. Pulmonary CT angiography showed small pulmonary artery aneu- rysms with thrombosis of peripheral pulmonary artery branches. Both patients were treated successfully with steroids and immunosuppressive therapy and showed clinical improvement. Keywords Behcet’s disease . Hughes–Stovin syndrome . Incomplete Behcet’s disease . Pulmonary vasculitis Introduction In 1959, Hughes and Stovin [1] described four patients, all young men, with a syndrome of multiple pulmonary artery aneurysms and venous thrombosis. In particular, three of the original four cases reviewed by Hughes and Stovin were noted to have cardiac thrombi. Hughes and Stovin found histologic evidence of a degenerative defect of the bronchial arteries and postulated that the aneurysms may be mycotic in origin, presumably resulting from infected emboli with organisms of low-grade virulence. However, Clin Rheumatol (2007) 26:1993–1996 DOI 10.1007/s10067-007-0609-y Y. Emad (*) : T. Gheita Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University, Cairo, Egypt e-mail: yasseremad68@yahoo.com Y. Emad Rheumatology and Rehabilitation Department, Dr. Erfan and Bagedo Hospital, King Fahad Street, P.O. Box 6519, Jeddah 21452, Saudi Arabia Y. Ragab Radio Diagnosis Department, Faculty of Medicine, Cairo University, Cairo, Egypt Y. Ragab Radio Diagnosis Department, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia A. E.-H. Shawki Neurology Department, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia A. El-Marakbi General Surgery Department, Faculty of Medicine, Cairo University, Cairo, Egypt A. El-Marakbi Vascular Unit, Dr. Erfan and Bagedo Hospital, Jeddah, Saudi Arabia M. H. Salama Rheumatology Department, Faculty of Medicine, El Azhar University, Cairo, Egypt