Fartura Braga Temido Maria Helena, Gomes Manuel, Parente Francisco, Santos Lèlita
Internal Medicine Department A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Internal Medicine Department, Trofa Saúde Hospital, Trofa, Portugal.
J Scleroderma Relat Disord. 2019 Feb;4(1):NP1-NP4. doi: 10.1177/2397198318765596. Epub 2018 Apr 10.
Systemic sclerosis is a systemic autoimmune disease characterized by microangiopathy and fibroblast dysfunction resulting in fibrosis of the skin and internal organs. Raynaud's phenomenon and digital ulcers are the main clinical features of vascular involvement. Treatment with iloprost is recommended to reduce the frequency and severity of Raynaud's phenomenon attacks and to heal active digital ulcer. Classical forms of treatment require admission to a ward or day-hospital unit to ensure safety during infusion, causing disruption of the patient's normal daily life and resulting in significant costs with hospitalization. Nowadays, new portable devices, of which the elastomeric pump is an example, are becoming available in order to avoid hospitalization.
We describe five cases of patients with systemic sclerosis or mixed connective tissue disease and severe Raynaud's phenomenon/critical ischaemia or active digital ulcers that were successfully treated with iloprost infusion through elastomeric pump without experiencing any side effects. We present our unit's protocol for ambulatory infusion.
DISCUSSION/CONCLUSION: Our case reports and a brief review of literature prove that iloprost infusion through elastomeric pump is safe, easy and well tolerated and might even improve patient compliance with treatment. Meanwhile, it may also decrease the economic burden of hospitalization with these patients.
系统性硬化症是一种系统性自身免疫性疾病,其特征为微血管病变和成纤维细胞功能障碍,导致皮肤和内脏器官纤维化。雷诺现象和指端溃疡是血管受累的主要临床特征。推荐使用伊洛前列素治疗以减少雷诺现象发作的频率和严重程度,并治愈活动性指端溃疡。传统的治疗方式需要住院或日间病房治疗,以确保输液安全,这会打乱患者的正常日常生活,并导致住院费用高昂。如今,新型便携式设备(如弹性泵)已问世,可避免住院治疗。
我们描述了5例系统性硬化症或混合性结缔组织病患者,伴有严重雷诺现象/严重缺血或活动性指端溃疡,通过弹性泵输注伊洛前列素成功治疗,且未出现任何副作用。我们展示了本单位的门诊输液方案。
讨论/结论:我们的病例报告及简要文献回顾证明,通过弹性泵输注伊洛前列素安全、简便且耐受性良好,甚至可能提高患者的治疗依从性。同时,这也可能减轻这些患者的住院经济负担。