Hughes–Stovin Syndrome Without Peripheral Venous Thrombosis: Early Recognition and Successful Immunosuppressive Management—A Case Report and Brief Literature Review

Hughes–Stovin syndrome (HSS) is an exceptionally rare and potentially life-threatening systemic vasculitis characterized by the coexistence of pulmonary artery aneurysms and venous thrombosis. Its striking clinical overlap with Behçet disease has led to ongoing debate regarding whether HSS constitutes a distinct disease entity or instead represents a vascular variant within the Behçet spectrum. In the absence of standardized diagnostic criteria, diagnosis relies primarily on clinical suspicion supported by radiologic evidence. Early identification is crucial, as rupture of pulmonary aneurysms is frequently fatal. We report a young male patient diagnosed with HSS who demonstrated favorable clinical and radiologic improvement following treatment with corticosteroids and mycophenolate mofetil. This case highlights the importance of considering HSS in young individuals presenting with unexplained pulmonary artery aneurysms, even in the absence of venous thrombosis. Prompt diagnosis, early initiation of immunosuppressive therapy, and coordinated multidisciplinary management are essential to mitigate the risk of catastrophic complications. This case is unusual in that HSS occurred in the absence of peripheral venous thrombosis and responded favorably to combined corticosteroid and mycophenolate mofetil therapy. • Hughes–Stovin syndrome is a rare vasculitis with pulmonary artery aneurysms. • Venous thrombosis may be absent, adding to the diagnostic challenge. • Early diagnosis and aggressive immunosuppression can prevent fatal rupture. • This case showed marked radiologic improvement with steroids and mycophenolate. • HSS should be considered in young patients with unexplained pulmonary artery aneurysms.