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Hughes-Stovin syndrome—An important differential diagnosis in patients with suspected chronic thromboembolic pulmonary hypertension: A case report.

Authors :
Pochepnia, Svitlana
Lang, Irene M.
Milos, Ruxandra Iulia
Röhrich, Sebastian
Ba-Ssalamah, Ahmed
Beer, Lucian
Prosch, Helmut
Source :
Wiener Klinische Wochenschrift; Feb2024, Vol. 136 Issue 3/4, p118-121, 4p
Publication Year :
2024

Abstract

Summary: 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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00435325
Volume :
136
Issue :
3/4
Database :
Complementary Index
Journal :
Wiener Klinische Wochenschrift
Publication Type :
Academic Journal
Accession number :
175234890
Full Text :
https://doi.org/10.1007/s00508-023-02299-w