Juche A, Siegert E, Mueller-Ladner U, Riemekasten G, Günther C, Kötter I, Henes J, Blank N, Voll R E, Ehrchen J, Schmalzing M, Susok L, Schmeiser T, Sunderkoetter C, Distler J, Worm M, Kreuter A, Horváth O N, Schön M P, Korsten P, Zeidler G, Pfeiffer C, Krieg T, Hunzelmann N, Moinzadeh P
Klinik für Rheumatologie, Immanuel Krankenhaus Berlin-Buch, Berlin, Deutschland.
Klinik für Rheumatologie u. klinischer Immunologie, Charité Berlin, Berlin, Deutschland.
Z Rheumatol. 2020 Dec;79(10):1057-1066. doi: 10.1007/s00393-019-00743-9.
Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers.
In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives.
Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower.
Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations.
雷诺现象以及随之频繁出现的手指溃疡是系统性硬化症（硬皮病，SSc）患者早期出现的极为痛苦的症状，会严重限制患者的工作能力和生活质量。建议使用血管活性药物（尤其是静脉注射前列环素衍生物）来降低组织缺氧损伤直至手指丧失的风险。
为了解德国日常临床中使用前列环素衍生物治疗患者的现状，对德国系统性硬化症网络（DNSS）下属各中心进行了一项调查。此外，硬皮病自助组织（Sklerodermie Selbsthilfe e. V.）单独开展了一项患者调查，该调查仅涵盖雷诺综合征症状、手指溃疡以及静脉注射前列环素衍生物的使用情况。
在接受调查的433名患者中，56%表示他们因病情和症状已接受过前列环素衍生物（伊洛前列素/前列地尔）治疗。共有61%的患者因严重雷诺现象接受治疗，39%因手指溃疡接受治疗。大多数受访者不仅雷诺现象和手指溃疡有所改善，日常生活中的受限情况也有显著改善。他们还明显减少了对外部帮助的需求，旷工情况也大幅减少。
患者一致报告前列环素衍生物治疗对雷诺现象、肢端溃疡、疼痛和日常活动受限有积极作用，并且在住院治疗期间感觉得到了良好且安全的护理。患者认知中的这些积极作用为支持和确认当前欧洲及国际治疗建议提供了关键信息。
