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Hughes-Stovin syndrome (HSS) is a life-threatening disorder, believed to be a cardiovascular clinical variant manifestation of Behcet's disease (BD). It is characterized by the association of multiple pulmonary artery aneurysms (PAAs) and peripheral venous thrombosis.
Features include very common findings: High blood pressure in lung arteries (pulmonary arterial hypertension), Dyspnea, Hemoptysis, and Pulmonary embolism and others; and common findings: Fever, Nausea and vomiting, Headache, and Pulmonary artery aneurysm and others. 18 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Lungs and breathing | 4 | High blood pressure in lung arteries (pulmonary arterial hypertension), Dyspnea, Pulmonary embolism |
Biomarker and diagnostic research for Hughes-Stovin syndrome has been reported in the published literature.
Phenotype severity distribution: 11 very common features, 5 common features.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for Hughes-Stovin syndrome.
21 publications have been identified in PubMed for Hughes-Stovin syndrome. Research spans Case Report / Case Series (57%), Review / Meta-Analysis (19%), and Other (14%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 12 | 57% |
Data assembled from 4 of 12 sources · Last updated Oct 3, 2026, 8:12 PM UTC
Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Hughes-Stovin syndrome
Heart and blood vessels | 2 | High blood pressure in lung arteries (pulmonary arterial hypertension), Chest pain |
Brain and nerves | 2 | Headache, Fatigue |
Metabolism | 1 | Fever |
Digestive system | 1 | Nausea and vomiting |
Blood and immune system | 1 | Vasculitis |
4 |
19% |
Other research | 3 | 14% |
Testing and diagnosis research | 1 | 5% |
Disease patterns and progression | 1 | 5% |
Naseer Z (2026). [PMID: 42165773](https://pubmed.ncbi.nlm.nih.gov/42165773/). *JACC Case Rep*. [Case Report / Case Series]
Emad Y (2026). [PMID: 41984348](https://pubmed.ncbi.nlm.nih.gov/41984348/). *Clin Rheumatol*. [Review / Meta-Analysis]
Tahani I (2026). [PMID: 42007204](https://pubmed.ncbi.nlm.nih.gov/42007204/). *Radiol Case Rep*. [Case Report / Case Series]
Galego PH (2026). [PMID: 41474441](https://pubmed.ncbi.nlm.nih.gov/41474441/). *Lung India*. [Case Report / Case Series]
Al-Bitar A (2026). [PMID: 41947193](https://pubmed.ncbi.nlm.nih.gov/41947193/). *J Med Case Rep*. [Case Report / Case Series]
Ebraheem K (2026). [PMID: 41808877](https://pubmed.ncbi.nlm.nih.gov/41808877/). *Respir Med Case Rep*. [Case Report / Case Series]
Ediboğlu ED (2026). [PMID: 41906389](https://pubmed.ncbi.nlm.nih.gov/41906389/). *Int J Rheum Dis*. [Other]
Maruo K (2025). [PMID: 40222941](https://pubmed.ncbi.nlm.nih.gov/40222941/). *Intern Med*. [Case Report / Case Series]
Imran MM (2025). [PMID: 41234379](https://pubmed.ncbi.nlm.nih.gov/41234379/). *Health Sci Rep*. [Review / Meta-Analysis]
Qadir A (2025). [PMID: 40223472](https://pubmed.ncbi.nlm.nih.gov/40223472/). *Int J Rheum Dis*. [Case Report / Case Series]
AI-curated news mentioning Hughes-Stovin syndrome
Updated May 20, 2026
A recent case study highlights a fatal instance of cardiac Behçet disease in a patient with Hughes-Stovin syndrome, emphasizing the complexities of this overlap. The findings contribute to the understanding of hematoma formation in these rare conditions.
hughes stovin syndrome as an uncommon cause of cardiac thrombus and pulmonary embolism a case report