Zulian F, Corona F, Gerloni V, Falcini F, Buoncompagni A, Scarazatti M, Martini G, Zacchello F
Pediatric Rheumatology Unit, University of Padua, Italy.
Rheumatology (Oxford). 2004 Feb;43(2):229-33. doi: 10.1093/rheumatology/keg480. Epub 2003 Aug 15.
We analysed our experience with the use of iloprost for the treatment of critical ischaemic digits (ID) in children with connective tissue diseases (CTD) in order to assess its safety and efficacy.
This was a retrospective analysis of paediatric patients with CTD who were treated with iloprost for critical ID resistant to conventional therapy. Information on demographics, clinical and laboratory features, the regimen of iloprost treatment and outcome were collected.
Fifteen patients (10 female, five male) treated one or more times with iloprost were included (total of 19 treatments). Six had juvenile systemic sclerosis, five had systemic lupus erythematosus, three had mixed connective tissue disease and one had cutaneous polyarteritis nodosa. Thirteen patients were already taking calcium channel blockers with no improvement; in two patients ID were the presenting signs of the disease without prior treatment. Eleven patients had more than two fingers involved; one child had involvement of all 10 fingers. Normal digital blood flow was achieved in 74% of treatments and significant improvement was noted in 26%. Fingertip necrosis was present in 11 patients (14 treatments). It healed completely in seven, improved in one and remained unchanged in six. Raynaud's phenomenon (RP) was present in 14 patients (17 treatments): in two no RP attack occurred during the follow-up period, eight improved both in the number of attacks per week and in the duration of each attack. Complete pain relief was observed in 10/17 treatments (59%) and there was a significant decrease in pain in the remaining seven. No major side-effects or withdrawal symptoms were reported. Minor side-effects reported include reversible headache (seven patients), hypotension or irritability (three), nausea/vomiting (two) and injection site reaction (one).
Iloprost appears to be a safe and effective treatment for ischaemic digits and digital ulcers in children with CTD. In conjunction with immunosuppressive drugs, it has a potential role in preventing irreversible complications, such as digital gangrene and amputation.
我们分析了使用伊洛前列素治疗结缔组织病（CTD）患儿严重缺血性手指（ID）的经验，以评估其安全性和有效性。
这是一项对接受伊洛前列素治疗的常规治疗无效的CTD患儿的回顾性分析。收集了人口统计学、临床和实验室特征、伊洛前列素治疗方案及结果等信息。
纳入15例接受过一次或多次伊洛前列素治疗的患者（10例女性，5例男性）（共19次治疗）。6例患有青少年系统性硬化症，5例患有系统性红斑狼疮，3例患有混合性结缔组织病，1例患有皮肤型结节性多动脉炎。13例患者已在服用钙通道阻滞剂但无改善；2例患者ID是疾病的首发症状，之前未接受过治疗。11例患者累及超过两根手指；1例儿童10根手指均受累。74%的治疗实现了正常的手指血流，26%有显著改善。11例患者（14次治疗）存在指尖坏死。7例完全愈合，1例改善，6例无变化。14例患者（17次治疗）存在雷诺现象（RP）：2例在随访期间未发生RP发作，8例每周发作次数和每次发作持续时间均有所改善。10/17次治疗（59%）观察到疼痛完全缓解，其余7次疼痛显著减轻。未报告重大副作用或戒断症状。报告的轻微副作用包括可逆性头痛（7例患者）、低血压或易怒（3例）、恶心/呕吐（2例）和注射部位反应（1例）。
伊洛前列素似乎是治疗CTD患儿缺血性手指和手指溃疡的安全有效的方法。与免疫抑制药物联合使用，它在预防不可逆并发症如手指坏疽和截肢方面具有潜在作用。
