ArticleWiener klinische Wochenschrift2024
Hughes-Stovin syndrome-An important differential diagnosis in patients with suspected chronic thromboembolic pulmonary hypertension : A case report.
Article in Wiener klinische Wochenschrift, 2024. 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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2 citing papers in PubMed, 1 synthesis or guideline pooled it, 1 citations in OpenAlex.
- Hughes-Stovin syndrome (HSS) preliminary diagnostic criteria: a report by the HSS International Study Group (HSSISG).Clinical rheumatology · 2026Pooled it
- A Rare Case of Hughes-Stovin Syndrome Presenting with Ischemic Colitis Caused by an Arteriovenous Malformation.Internal medicine (Tokyo, Japan) · 2025Article
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7 authors at 2 institutions in 1 country.
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Abstract
Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.
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