Moinzadeh Pia, Riemekasten Gabriela, Siegert Elise, Fierlbeck Gerhard, Henes Joerg, Blank Norbert, Melchers Inga, Mueller-Ladner Ulf, Frerix Marc, Kreuter Alexander, Tigges Christian, Lahner Nina, Susok Laura, Guenther Claudia, Zeidler Gabriele, Pfeiffer Christiane, Worm Margitta, Karrer Sigrid, Aberer Elisabeth, Bretterklieber Agnes, Genth Ekkehard, Simon Jan C, Distler Joerg H W, Hein Ruediger, Schneider Matthias, Seitz Cornelia S, Herink Claudia, Steinbrink Kerstin, Sárdy Miklos, Varga Rita, Mensing Hartwig, Mensing Christian, Lehmann Percy, Neeck Gunther, Fiehn Christoph, Weber Manfred, Goebeler Matthias, Burkhardt Harald, Buslau Michael, Ahmadi-Simab Keihan, Himsel Andrea, Juche Aaron, Koetter Ina, Kuhn Annegret, Sticherling Michael, Hellmich Martin, Kuhr Kathrin, Krieg Thomas, Ehrchen Jan, Sunderkoetter Cord, Hunzelmann Nicolas
J Rheumatol. 2016 Jan;43(1):66-74. doi: 10.3899/jrheum.150382. Epub 2015 Nov 15.
Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry.
The data of 3248 patients with SSc were analyzed.
Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005.
These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed.
血管病变是系统性硬化症（SSc）病理生理学的关键因素，也是雷诺现象（RP）、指端溃疡（DU）和/或肺动脉高压（PAH）的主要病因。目前尚不清楚在日常实践中SSc患者如何接受血管活性药物治疗。为了确定SSc患者接受不同血管活性药物治疗的程度，我们使用了德国系统性硬皮病登记网络的数据。
分析了3248例SSc患者的数据。
61.1%的病例（1984/3248）患者接受了血管活性药物治疗。其中，47.6%接受钙通道抑制剂治疗，其次是34.2%接受血管紧张素转换酶（ACE）抑制剂治疗，21.1%接受静脉注射（IV）前列腺素治疗，10.1%接受己酮可可碱治疗，8.8%接受血管紧张素1受体拮抗剂（AT1RA）治疗，8.7%接受内皮素1受体拮抗剂（ET1RA）治疗，4.1%接受5型磷酸二酯酶（PDE5）抑制剂治疗，5.3%接受其他治疗。RP患者63.3%的病例接受了血管活性治疗，DU患者70.1%，PAH患者78.2%。逻辑回归分析显示，PAH患者接受PDE5抑制剂和ET1RA治疗的频率显著更高，DU患者接受ET1RA和IV前列腺素治疗的频率更高。此外，41.8%的患者接受了ACE抑制剂和/或AT1RA治疗。2009年后登记的患者与2005年前登记的患者相比，接受ET1RA、AT1RA和IV前列腺素治疗的频率显著更高。
这些数据清楚地表明，许多SSc患者尚未接受足够的血管活性治疗。此外，近年来，可以观察到治疗方案有明显变化。
