Hughes–Stovin Syndrome: A Systematic Review and Integrative Analysis of Clinical Spectrum, Pathophysiology, and Therapeutic Strategies

Background: Hughes–Stovin syndrome (HSS) is an ultra-rare, life-threatening vascular inflammatory disorder defined by the concurrence of venous thrombosis and pulmonary artery aneurysms. Despite its clinical severity, the condition remains poorly characterized, and its nosological relationship with Behçet’s disease is unresolved . Methods: We conducted a systematic review in accordance with PRISMA 2020 guidelines. PubMed/MEDLINE, Embase, and Scopus were searched from inception to December 2025. Case reports and case series describing HSS were included. Results: Thirty-five studies encompassing 57 patients met inclusion criteria. HSS predominantly affected young men (80–90%), with a mean age of 30–40 years. Venous thrombosis and pulmonary artery aneurysms were universally present. Hemoptysis emerged as the principal life-threatening manifestation . Conclusions: HSS should be recognized as a distinct, high-risk vascular inflammatory disorder within the vasculitis spectrum, where early diagnosis and prompt immunosuppressive therapy are critical to prevent fatal complications.