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Inflammatory, Hypersensitivity and Immune Lung Diseases, including Parasitic Diseases.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
The Hughes-Stovin syndrome, in which young males develop major vessel venous thromboses, as well as pulmonary artery aneurysms, is very similar to Behget's syndrome, and may be a variant of it. It is also a vasculitis and clinical features include pyrexia, erythematous skin lesions, arthralgia, optic neuritis, cerebral thrombophlebitis and scrotal ulcers.
Disseminated Mycobacterium chelonae infection with Hughes-Stovin syndrome
Published in Baylor University Medical Center Proceedings, 2021
Vrinda Vyas, Stephanie M. Bryant, Amish Shah, Dana Savici
A 27-year-old man had Hughes-Stovin syndrome (HSS) with aneurysms of pulmonary arteries, the left renal artery, and the superior mesenteric artery, recurrent deep venous thromboses, and pulmonary emboli. He had a recent hospital admission for massive hemoptysis due to left pulmonary artery aneurysm rupture. A left lobectomy was performed. His chest computed tomography revealed a postsurgical left-sided hydropneumothorax and diffuse bilateral ground-glass opacities thought to be secondary to alveolar hemorrhages. He was noted to have high titers of lupus anticoagulant with concerns for catastrophic antiphospholipid syndrome, for which he underwent plasmapheresis and was started on monthly infliximab intravenous infusions and azathioprine. He ultimately required tracheostomy and gastrostomy due to inability to wean off ventilation.
Hughes–Stovin syndrome with pulmonary aneurysms and renal dysfunction mimicking polyarteritis nodosa
Published in Modern Rheumatology Case Reports, 2018
Eri Watanabe, Kumiko Nishina, Hiroki Yabe, Takahisa Gono, Chihiro Terai
Hughes–Stovin syndrome (HSS) is an extremely rare condition characterised by pulmonary artery aneurysms and deep vein thrombosis (DVT) [1]. The exact etiology of this syndrome is not clear, and it is often recognised as an incomplete version or variant of Behçet’s disease (BD) because of the significant overlap in clinical, radiological, and histopathological findings between HSS and BD [2–5]. It usually affects young men, and the typical symptoms are recurrent fever, hemoptysis, cough, dyspnea, and chest pain [2], but renal dysfunction is not a typical symptom of HSS. We report a case of HSS with right pulmonary artery aneurysms and renal dysfunction, which required a differential diagnosis of polyarteritis nodosa (PAN).
Concurrent pulmonary artery aneurysms and intracardiac thrombus in a Caucasian man with Behçet’s disease: case report and literature review
Published in Modern Rheumatology Case Reports, 2018
Jean Liew, Daniela Ghetie, Danielle Hosmer, Cailin Sibley
A PubMed search was conducted for articles describing concurrent PAA and extrapulmonary thromboses published between 1980–2016 in English using combinations of the search terms “Behçet’s”, “thrombus”, “thrombosis”, “thromboembolism” and “pulmonary artery aneurysm”. Articles describing cases of Hughes-Stovin Syndrome were excluded. Further relevant articles were extracted from the references cited in the retrieved articles. 30 relevant articles were found, describing 38 individual cases [12–39]. Data were extracted from these cases for basic demographic information, clinical manifestations of BD, treatment regimen and outcomes.