Kallinich T, Blank N, Braun T, Feist E, Kiltz U, Neudorf U, Oommen P T, Weseloh C, Wittkowski H, Braun J
Pädiatrie m.S. Pulmonologie und Immunologie, Universitätsmedizin Charité Berlin, Berlin, Deutschland.
Rheumatologie, Universitätklinikum Heidelberg, Heidelberg, Deutschland.
Z Rheumatol. 2019 Feb;78(1):91-101. doi: 10.1007/s00393-018-0588-1.
Familial Mediterranean fever (FMF) in Germany is a rare, genetically linked disease of childhood and adolescence, which is characterized by recurrent febrile episodes and clinical signs of peritonitis, pleuritis and arthritis. Treatment with colchicine is effective and well-tolerated in the majority of patients; however, some patients do not sufficiently respond to this treatment or are intolerant to colchicine. For these patients first-line treatment with biologics which block interleukin-1 can be used.
The aim was to formulate evidence-based treatment recommendations for patients with an insufficient response and intolerance to colchicine treatment.
Based on a literature search and the European League Against Rheumatism (EULAR) recommendations on FMF from 2016 the appointed members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Rheumatology (DGRh) convened to work out and form a consensus in a joint statement on evidence-based treatment recommendations on FMF.
After intensive discussions all decisions were in concordance. A total of 5 superordinate principles and 10 recommendations were agreed upon.
The joint activities of the GKJR and the DGRh were successfully concluded in a timely manner. The recommendations form a good basis for optimal treatment of all age groups of patients with FMF.
在德国，家族性地中海热（FMF）是一种罕见的、与基因相关的儿童和青少年疾病，其特征为反复发热发作以及腹膜炎、胸膜炎和关节炎的临床症状。大多数患者使用秋水仙碱治疗有效且耐受性良好；然而，一些患者对这种治疗反应不佳或对秋水仙碱不耐受。对于这些患者，可以使用阻断白细胞介素 -1 的生物制剂进行一线治疗。
旨在为对秋水仙碱治疗反应不足和不耐受的患者制定基于证据的治疗建议。
基于文献检索以及欧洲抗风湿病联盟（EULAR）2016 年关于 FMF 的建议，儿科和青少年风湿病学会（GKJR）及德国风湿病学会（DGRh）指定的成员召开会议，以制定并在一份关于 FMF 基于证据的治疗建议的联合声明中达成共识。
经过深入讨论，所有决策达成一致。共商定了 5 条上级原则和 10 条建议。
GKJR 和 DGRh 的联合活动及时且成功地结束。这些建议为 FMF 各年龄组患者的最佳治疗奠定了良好基础。
