Objectives: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease despite being a rare disease for many rheumatologists. These evidence-based recommendations update the ones issued in 2016 to account for the recent developments in the field and aim to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF.
Methods: A multidisciplinary panel was assembled, including rheumatologists, internists, paediatricians, a nephrologist, an occupational therapist, a physiotherapist, 2 methodologists, and 2 patient representatives, all from the Eastern Mediterranean area and Europe. Several systematic reviews were performed on the pharmacological treatment of FMF and its complications. The previous recommendations were revised considering the updated evidence, and the new levels of evidence were incorporated. The agreement with the recommendations was obtained through a Delphi survey.
Results: The final set comprises 4 overarching principles and 12 recommendations, each presented with its degree of agreement (0-10), level of evidence, and rationale. The degree of agreement was greater than 9/10 in all instances, and the level of evidence improved in most updated statements. Improving adherence is emphasised as an important aspect in several statements. These new recommendations include a priority set, quality indicators, and other suggested implementation strategies.
Conclusions: This article presents a set of widely accepted recommendations for treating and monitoring FMF, supported by the best available evidence and expert opinion. These recommendations are valuable for guiding physicians in caring for patients with FMF.
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