[胶原病中的肺动脉高压：德国系统性硬化症网络的经验]

[Pulmonary arterial hypertension in collagen disease: experiences of the German Network for Systemic Scleroderma].

作者信息

Hunzelmann N, Riemekasten G

机构信息

Klinik für Dermatologie und Venerologie, Klinikum der Universität zu Köln, Kerpener Strasse 62, 50937 Köln.

出版信息

Dtsch Med Wochenschr. 2006 Dec 8;131(49 Suppl 9):S325-7. doi: 10.1055/s-2006-957203.

DOI:10.1055/s-2006-957203

PMID:17139598

Abstract

The German Network for Systemic Scleroderma (Deutsches Netzwerk Für Systemische Sklerodermie [DNSS]) has as its aim to improve and standardize the diagnosis and treatment of this rare disease. 14.6 % of patients in the DNSS register have been shown to have pulmonary hypertension (PHT). Comparable international data range from 12 % to 26.7 %. The DNSS recommends annual echocardiography for patients with scleroderma and right-heart catheterization if the systolic pulmonary arterial pressure is higher than 35 mmHg. Aggressive immunosuppressive treatment--in the first instance with cyclophosphamide--should be given only if there is also progressive fibrosis. Drugs that lower the level of PHT also have a favourable effect on other signs of scleroderma.

摘要

德国系统性硬皮病网络（Deutsches Netzwerk Für Systemische Sklerodermie [DNSS]）的目标是改善和规范这种罕见疾病的诊断与治疗。DNSS登记册中的患者有14.6%被证实患有肺动脉高压（PHT）。国际上的可比数据范围为12%至26.7%。DNSS建议对硬皮病患者每年进行超声心动图检查，若收缩期肺动脉压高于35 mmHg，则进行右心导管检查。仅当存在进行性纤维化时，才应给予积极的免疫抑制治疗——首先使用环磷酰胺。降低PHT水平的药物对硬皮病的其他症状也有有利影响。