Department of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey.
Clin Rheumatol. 2012 Aug;31(8):1267-71. doi: 10.1007/s10067-012-2009-1. Epub 2012 Jun 7.
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever and polyserositis. Reactive amyloid A amyloidosis is the most devastating complication of FMF, and amyloidosis continues to occur in the colchicine era in untreated and noncompliant patients. Unfortunately, there is no proven effective treatment for established amyloidosis. In this report, we present four FMF-related amyloidosis patients that were treated with long term infliximab therapy with the longest duration of follow-up, together with the literature review. Infliximab was very effective in controlling gastrointestinal system findings and protracted arthritis, and it also had a favorable impact on the clinical findings of nephrotic syndrome in these patients. In conclusion, by controlling debilitating complaints of amyloidosis with infliximab, quality of life increases in these patients, and they get rid of recurrent hospitalizations and return to school or work.
家族性地中海热(FMF)是一种常染色体隐性疾病,其特征为反复发作的自限性发热和多浆膜炎。反应性淀粉样蛋白 A 淀粉样变性是 FMF 最具破坏性的并发症,在未接受治疗和不依从的患者中,即使在秋水仙碱时代,淀粉样变性仍会继续发生。不幸的是,对于已确立的淀粉样变性,尚无经过证实的有效治疗方法。在本报告中,我们介绍了 4 例 FMF 相关淀粉样变性患者,他们接受了最长时间的英夫利昔单抗治疗,随访时间最长,并结合文献复习。英夫利昔单抗在控制胃肠道表现和迁延性关节炎方面非常有效,并且对这些患者肾病综合征的临床表现也有良好的影响。总之,通过用英夫利昔单抗控制淀粉样变性的使人衰弱的症状,这些患者的生活质量得到提高,他们不再需要频繁住院,并可以重返学校或工作。