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Rev Med Interne. 2019 Feb;40(2):120-125. doi: 10.1016/j.revmed.2018.07.009. Epub 2018 Jul 27.

[Hughes-Stovin syndrome: About one case in a young man with recurrent thrombosis and pulmonary artery aneurysm and literature review].

[Article in French]

Author information

1
Service d'immunologie clinique et médecine interne, centre de référence des maladies auto-immunes systémiques rares (RESO), hôpitaux universitaires de Strasbourg, nouvel hôpital civil, 67091 Strasbourg, France; UFR médecine Strasbourg, université de Strasbourg, 67000 Strasbourg, France. Electronic address: thomas.el-jammal@chru-strasbourg.fr.
2
Service d'immunologie clinique et médecine interne, centre de référence des maladies auto-immunes systémiques rares (RESO), hôpitaux universitaires de Strasbourg, nouvel hôpital civil, 67091 Strasbourg, France; UFR médecine Strasbourg, université de Strasbourg, 67000 Strasbourg, France. Electronic address: pierre-edouard.gavand@chru-strasbourg.fr.
3
Service de médecine interne, maladies infectieuses et tropicales, hôpital de la Milétrie, 86000 Poitiers, France; Inserm U 1082 (IRTOMIT), Poitiers, France. Electronic address: mickael-martin@outlook.com.
4
Service d'immunologie clinique et médecine interne, centre de référence des maladies auto-immunes systémiques rares (RESO), hôpitaux universitaires de Strasbourg, nouvel hôpital civil, 67091 Strasbourg, France; UFR médecine Strasbourg, université de Strasbourg, 67000 Strasbourg, France. Electronic address: anne-sophie-korganow@chru-strasbourg.fr.
5
Service d'immunologie clinique et médecine interne, centre de référence des maladies auto-immunes systémiques rares (RESO), hôpitaux universitaires de Strasbourg, nouvel hôpital civil, 67091 Strasbourg, France; UFR médecine Strasbourg, université de Strasbourg, 67000 Strasbourg, France. Electronic address: aurelien.guffroy@chru-strasbourg.fr.

Abstract

INTRODUCTION:

First described in 1959, Hughes-Stovin syndrome is a very rare disorder combining vascular aneurysms, especially from pulmonary arteries, and thrombosis. The disease affects mostly the young male and is sometime associated with Behçet' disease.

CASE REPORT:

Here, we report the case of a 19-year-old man with hemoptysis and dyspnea revealing recurrent pulmonary embolisms despite efficient anticoagulant therapy. The patient subsequently developed fever and an inflammatory syndrome. Physical examination showed ulcers of the tongue. Angio-CT revealed recent pulmonary embolism, femoral vein thrombosis, and a unique threatening aneurysm of a left pulmonary artery segment. The aneurysm was embolized and simultaneously a vena cava filter was inserted.

CONCLUSION:

Hughes-Stovin syndrome requires immediate therapeutic decision, with an important risk of the anticoagulation. High dose steroids and in most cases, intensive immunosuppressive therapies are required such as cyclophosphamide.

KEYWORDS:

Aneuvrysm; Anévrysme; Aphtose; Aphtosis; Behçet; Hughes-Stovin syndrome; Maladie de Behçet; Syndrome de Hughes-Stovin; Thromboses; Thrombosis

PMID:
30061011
DOI:
10.1016/j.revmed.2018.07.009
[Indexed for MEDLINE]

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