[休斯-斯托文综合征：白塞氏综合征的一种危及生命的表现]。

IF 0.9 4区医学 Q4 RHEUMATOLOGY

Zeitschrift fur Rheumatologie Pub Date : 2024-05-01 Epub Date: 2023-06-06 DOI:10.1007/s00393-023-01371-0

Nikolas Ruffer, Martin Krusche, Konstanze Holl-Ulrich, Fabian Lötscher, Ina Kötter

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引用次数: 0

摘要

休斯-斯托文综合征（HSS）是一种原因不明的全身性炎症，被认为是白塞氏综合征（BS）的一部分。复发性静脉血栓和浅表血栓性静脉炎合并双侧肺动脉瘤（PAA）是 HSS 的特征。诊断评估包括计算机断层扫描肺血管造影术，以检测肺血管炎的迹象。HSS 的治疗以欧洲风湿病学协会联盟（EULAR）对 BS 的建议为基础，主要包括使用糖皮质激素和环磷酰胺进行免疫抑制治疗。除药物治疗外，还应对 PAA 进行介入治疗评估。由于血管结构脆弱，即使在病情缓解和/或 PAA 消退的情况下，也可能发生 PAA 自发性破裂。

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[Hughes-Stovin syndrome: a life-threatening manifestation of Behçet's syndrome].

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.

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来源期刊

Zeitschrift fur Rheumatologie 医学-风湿病学

CiteScore

2.20

自引率

20.00%

发文量

150

审稿时长

6-12 weeks

期刊介绍： Die Zeitschrift für Rheumatologie ist ein international angesehenes Publikationsorgan und dient der Fortbildung von niedergelassenen und in der Klinik tätigen Rheumatologen. Die Zeitschrift widmet sich allen Aspekten der klinischen Rheumatologie, der Therapie rheumatischer Erkrankungen sowie der rheumatologischen Grundlagenforschung. Umfassende Übersichtsarbeiten zu einem aktuellen Schwerpunktthema sind das Kernstück jeder Ausgabe. Im Mittelpunkt steht dabei gesichertes Wissen zu Diagnostik und Therapie mit hoher Relevanz für die tägliche Arbeit – der Leser erhält konkrete Handlungsempfehlungen. Frei eingereichte Originalien ermöglichen die Präsentation wichtiger klinischer Studien und dienen dem wissenschaftlichen Austausch.