"Hughes-Stovin Syndrome: A Comprehensive Narrative Review"

Hughes-Stovin Syndrome (HSS) is an extremely rare vasculitic disorder characterized by a combination of pulmonary artery aneurysms and deep venous thrombosis (DVT). Often considered a variant or incomplete form of Behçet's disease (BD), it presents diagnostic and therapeutic challenges due to its overlap with other conditions and the lack of standardized criteria. This narrative review aims to describe the epidemiology, pathophysiology, clinical presentation, diagnostic process, treatment strategies, and clinical outcomes associated with HSS.

A narrative review of the literature was conducted using PubMed and Google Scholar. The search included the terms "HSS" OR "pulmonary artery aneurysm" AND "Behçet's disease." Relevant literature was selected for inclusion based on thematic relevance and clinical importance. Only English-language articles focusing on human subjects were included, with an emphasis on case reports, series, reviews, and expert opinions. Literature on Behçet's disease was also reviewed for comparative purposes. The reference lists of key articles were manually searched for additional sources. No publication date restrictions were applied. The final search was completed on April 6, 2025.

Fewer than 100 cases of HSS have been published in the English-language literature to date. It primarily affects young adult males and typically presents with hemoptysis, chest pain, and venous thrombosis. Diagnosis is clinical and radiological, with CTPA being the preferred imaging modality. Immunosuppressive therapy remains the cornerstone of management, though there is no consensus on treatment protocols. The use of anticoagulation is controversial due to the risk of aneurysmal rupture.

HSS presents significant diagnostic and therapeutic challenges due to its rarity and clinical overlap with other vasculitides. Bridging the current gaps in clinical knowledge will require global collaboration to establish consistent diagnostic standards, deepen our understanding of its underlying mechanisms, and develop evidence-based treatment protocols.