Ruffer Nikolas, Krusche Martin, Holl-Ulrich Konstanze, Lötscher Fabian, Kötter Ina
III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland.
Konsultations- und Referenzzentrum für Vaskulitis-Diagnostik, Labor Lademannbogen MVZ GmbH, Hamburg, Deutschland.
Z Rheumatol. 2024 May;83(4):327-333. doi: 10.1007/s00393-023-01371-0. Epub 2023 Jun 6.
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.
休斯-斯托文综合征（HSS）是一种病因不明的全身性炎症性疾病，被认为是白塞病（BS）谱系的一部分。复发性静脉血栓形成和浅表血栓性静脉炎，合并双侧肺动脉瘤（PAA）是HSS的标志。诊断评估包括计算机断层扫描肺动脉造影，以检测肺血管炎的迹象。HSS的治疗基于欧洲风湿病协会联盟（EULAR）对白塞病的建议，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。即使在缓解期和/或PAA消退的情况下，由于血管结构脆弱，PAA也可能发生自发性破裂。
