ArticleInternational journal of rheumatic diseases2025
Hughes-Stovin Syndrome: A Rare Cause of Pulmonary Artery Aneurysm and Thrombosis-A Case Report.
Article in International journal of rheumatic diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.
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Who cites it
2 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Hughes-Stovin syndrome (HSS) preliminary diagnostic criteria: a report by the HSS International Study Group (HSSISG).Clinical rheumatology · 2026Pooled it
- Organizing Hematoma Involving a Native Tricuspid Valve: Fatal Case of Cardiac Behçet Disease-Hughes-Stovin Syndrome Overlap.JACC. Case reports · 2026Article
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Authors and funding
4 authors.
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Abstract
Hughes-Stovin Syndrome (HSS) is a rare, life-threatening condition characterized by thrombophlebitis and pulmonary artery aneurysms, often viewed as a variant of Behçet's disease. Its diagnosis and management are challenging due to its rarity and overlapping symptoms with other vascular disorders. A 33-year-old male presented with massive hemoptysis and a history of recurrent oral and scrotal ulcers. Imaging revealed bilateral pulmonary emboli, a pulmonary artery aneurysm, and a large right atrial thrombus. These findings, coupled with his clinical history, led to a diagnosis of HSS. A multidisciplinary team initiated anticoagulation for the thrombus and immunosuppressive therapy (steroids, cyclophosphamide). Follow-up imaging showed improvement in both the aneurysm and thrombus. This case highlights the complexity of diagnosing and managing HSS. Early recognition, supported by a collaborative approach, is critical to improving outcomes in this rare syndrome.
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