EULAR recommendations for the management of FMF with the level of agreement, of evidence and grade of recommendation (GR)
| Recommendation | A | LoE | GR |
|---|---|---|---|
| 01. Ideally, FMF should be diagnosed and initially treated by a physician with experience in FMF | 7.6 | 5 | D |
| 02. The ultimate goal of treatment in FMF is to reach complete control of unprovoked attacks and minimising subclinical inflammation in between attacks | 9.3 | 4 | C |
| 03. Treatment with colchicine should start as soon as a clinical diagnosis is made | 8.9 | 1b | A |
| 04. Dosing can be in single or divided doses, depending on tolerance and compliance | 9.4 | 5 | D |
| 05. The persistence of attacks or of subclinical inflammation represents an indication to increase the colchicine dose | 9.7 | 3 | C |
| 06. Compliant patients not responding to the maximum tolerated dose of colchicine can be considered non-respondent or resistant; alternative biological treatments are indicated in these patients | 9.8 | 2b | B |
| 07. FMF treatment needs to be intensified in AA amyloidosis using the maximal tolerated dose of colchicine and supplemented with biologics as required | 9.5 | 2b | C |
| 08. Periods of physical or emotional stress can trigger FMF attacks, and it may be appropriate to increase the dose of colchicine temporarily | 7.6 | 5 | D |
| 09. Response, toxicity and compliance should be monitored every 6 months | 8.6 | 5 | D |
| 10. Liver enzymes should be monitored regularly in patients with FMF treated with colchicine; if liver enzymes are elevated greater than twofold the upper limit of normal, colchicine should be reduced and the cause further investigated | 8.4 | 5 | D |
| 11. In patients with decreased renal function, the risk of toxicity is very high, and therefore signs of colchicine toxicity, as well as CPK, should be carefully monitored and colchicine dose reduced accordingly | 9.3 | 4 | C |
| 12. Colchicine toxicity is a serious complication and should be adequately suspected and prevented | 9.4 | 4 | C |
| 13. When suspecting an attack, always consider other possible causes. During the attacks, continue the usual dose of colchicine and use NSAID | 9.5 | 2b | C |
| 14. Colchicine should not be discontinued during conception, pregnancy or lactation; current evidence does not justify amniocentesis | 9.3 | 3 | C |
| 15. In general, men do not need to stop colchicine prior to conception; in the rare case of azoospermia or oligospermia proven to be related to colchicine, temporary dose reduction or discontinuation may be needed | 8.2 | 3 | C |
| 16. Chronic arthritis in a patient with FMF might need additional medications, such as DMARDs, intra-articular steroid injections or biologics | 9.5 | 2b | C |
| 17. In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and IL-1-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain | 9.3 | 2b | C |
| 18. If a patient is stable with no attacks for more than 5 years and no elevated APR, dose reduction could be considered after expert consultation and with continued monitoring | 8.0 | 5 | D |
A, agreement (/10); APR, acute phase reactants; CPK, creatinine phosphokinase; DMARDs, disease modifying antirheumatic drugs; EULAR, European League Against Rheumatism; FMF, familial Mediterranean fever; IL-1, interleukin 1; LoE, level of evidence; NSAID, non steroidal anti inflammatory drugs.