[Hughes-Stovin syndrome: a life-threatening manifestation of Behçet's syndrome].
Hughes-Stovin-Syndrom: eine lebensbedrohliche Manifestation des Behçet-Syndroms.
Deep vein thrombosis
Hemoptysis
Pulmonary artery aneurysm
Pulmonary artery thrombosis
Vasculitis
Journal
Zeitschrift fur Rheumatologie
ISSN: 1435-1250
Titre abrégé: Z Rheumatol
Pays: Germany
ID NLM: 0414162
Informations de publication
Date de publication:
06 Jun 2023
06 Jun 2023
Historique:
accepted:
19
04
2023
medline:
7
6
2023
pubmed:
7
6
2023
entrez:
6
6
2023
Statut:
aheadofprint
Résumé
Hughes-Stovin syndrome (HSS) is a systemic inflammatory condition of unknown origin that is considered to be part of the Behçet's syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis. The management of HSS is based on the European Alliance of Associations for Rheumatology (EULAR) recommendations for BS and mainly comprises immunosuppressive therapy with glucocorticoids and cyclophosphamide. In addition to drug therapy, PAA should be evaluated for interventional treatment. Spontaneous PAA rupture due to fragile vessel architecture can occur even in cases of remission and/or PAA regression. Das Hughes-Stovin-Syndrom (HSS) ist eine entzündliche Systemerkrankung unklarer Genese, die inzwischen dem Spektrum des Behçet-Syndroms (BS) zugeordnet wird. Wegweisende Befunde sind rezidivierende Thrombosen des venösen Systems und oberflächliche Thrombophlebitiden in Kombination mit beidseitigen Pulmonalarterienaneurysmen (PAA). Die Pulmonalisangiographie mittels Computertomographie ist von entscheidender diagnostischer Bedeutung, um die (entzündliche) Beteiligung der Pulmonalarterien darzustellen. Die Therapie des HSS orientiert sich an den Empfehlungen der European Alliance of Associations for Rheumatology (EULAR) für das BS und sieht primär eine Immunsuppression mit Cyclophosphamid und Glukokortikoiden vor. Neben einer medikamentösen Therapie sollte eine interventionelle Versorgung der PAA evaluiert werden. Eine spontane PAA-Ruptur muss auch bei Remission der Erkrankung und/oder deutlicher Regredienz des PAA-Durchmessers aufgrund einer fragilen Gefäßarchitektur bedacht werden.
Autres résumés
Type: Publisher
(ger)
Das Hughes-Stovin-Syndrom (HSS) ist eine entzündliche Systemerkrankung unklarer Genese, die inzwischen dem Spektrum des Behçet-Syndroms (BS) zugeordnet wird. Wegweisende Befunde sind rezidivierende Thrombosen des venösen Systems und oberflächliche Thrombophlebitiden in Kombination mit beidseitigen Pulmonalarterienaneurysmen (PAA). Die Pulmonalisangiographie mittels Computertomographie ist von entscheidender diagnostischer Bedeutung, um die (entzündliche) Beteiligung der Pulmonalarterien darzustellen. Die Therapie des HSS orientiert sich an den Empfehlungen der European Alliance of Associations for Rheumatology (EULAR) für das BS und sieht primär eine Immunsuppression mit Cyclophosphamid und Glukokortikoiden vor. Neben einer medikamentösen Therapie sollte eine interventionelle Versorgung der PAA evaluiert werden. Eine spontane PAA-Ruptur muss auch bei Remission der Erkrankung und/oder deutlicher Regredienz des PAA-Durchmessers aufgrund einer fragilen Gefäßarchitektur bedacht werden.
Identifiants
pubmed: 37280333
doi: 10.1007/s00393-023-01371-0
pii: 10.1007/s00393-023-01371-0
doi:
Types de publication
English Abstract
Journal Article
Review
Langues
ger
Sous-ensembles de citation
IM
Informations de copyright
© 2023. The Author(s).
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