[Results of the systematic literature search as basis for the "Evidence-based treatment recommendations for familial Mediterranean fever patients with insufficient response or intolerability to colchicine" of the Society for Pediatric and Adolescent Rheumatology and the German Society for Rheumatology].

BACKGROUND

Familial Mediterranean fever (FMF) is a genetic disease of childhood and adulthood which is relatively rare in Germany. It is characterized by recurrent febrile attacks, peritonitis, pleuritis and arthritis. The established treatment with colchicine is effective and well-tolerated by most patients; however, some patients do not adequately respond or do not tolerate this treatment. Biologics can be considered for some of these patients. The Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Rheumatology (DGRh) have agreed to develop joint recommendations for this specific clinical situation.

AIM

Implementation of a systematic literature search (SLR) on the basis of the EULAR recommendations published in 2016 as the foundation for the development of evidence-based treatment recommendations for FMF patients with insufficient response or intolerance to colchicine.

METHODS

The SLR was performed using references from various databases as an update of the SLR carried out by EULAR up to 2014, whereby all articles must have been published between 1 January 2015 and 31 December 2017. The Rayyan abstract tool for the preselection and the classification of the Oxford Centre for Evidence Based Medicine 2009 were used for the preparation of the evidence tables.

RESULTS

The search yielded 360 hits and after duplicate matching 263. A total of 88 publications were included (34%) and 102 excluded (39%), a review of the full publication was necessary for a further 73 (28%) and 43 were discussed more intensively. Finally, 64 publications (24%) remained. A total of 4 case-control studies, 31 cohort studies, 8 case series, 7 controlled studies (including 5 abstracts), 10 reviews, 4 meta-analyses and systematic reviews were accepted.

DISCUSSION

The SLR was carried out in a scientifically accurate and transparent manner according to international standards. The SLR proved to be a good basis for a consensus on the 5 overarching principles and the 10 recommendations, so that the joint activity of the GKJR and DGRh was successfully and even promptly concluded. The recommendations are a solid basis for treating patients of all ages with FMF. The explanations on the problem of colchicine resistance play an important role here.