Overarching principles

• FMF has complex clinical presentation and genetics, which requires expertise in diagnosis and management
• The treatment goal in FMF is to achieve minimal or no clinical activity and complete control of subclinical inflammation to prevent associated damage
• FMF requires lifetime management, including long-term prophylaxis with colchicine; therefore, treatment adherence is the cornerstone of FMF management
• Patient-centred management is required to promote a good quality of life and support health and well-being

Recommendations

1. Treatment with colchicine should start as soon as a clinical diagnosis is made 
2. Colchicine dosing can be in single or divided doses depending on adherence and tolerance
3. In adherent patients, the persistence of attacks or subclinical inflammation represents an indication to increase the colchicine dose within the recommended range. The maximum dose should not exceed 2 mg/d in children and 3 mg/d in adults
4. Adherent patients not responding adequately to the maximum tolerated dose of colchicine should be considered for additional treatments; the highest level of evidence is for IL-1-targeting treatments
5. Chronic inflammatory musculoskeletal involvement of FMF may need additional treatments
6. Response, toxicity, and adherence should be monitored regularly, more often at diagnosis and when the disease is uncontrolled
7. As overdose with colchicine can be fatal; intentional or accidental overdosing should be carefully assessed
8. In AA amyloidosis, treatment aims to obtain complete and sustained control of the biochemical inflammation, measured by SAA or equivalent (CRP)
9. In FMF patients on biologics, doses should be optimised; if remission is achieved, tapering and discontinuation may be considered
10. Colchicine should be continued during conception, pregnancy, and lactation; amniocentesis is not currently recommended
11. During a characteristic attack, the usual dose of colchicine should be continued, and symptomatic treatment, such as NSAIDs, is recommended
12. To standardise patient care and research, a minimum outcome set should include measures of clinical disease activity (eg, attack frequency per 3 mo, physician assessment), patient experience (eg, PROMs and PREMs), and biochemical markers (eg, CRP, SAA)