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Orphanet J Rare Dis. 2017 Mar 16;12(1):54. doi: 10.1186/s13023-017-0609-1.

A survey of resistance to colchicine treatment for French patients with familial Mediterranean fever.

Author information

1
Department of Paediatrics Rheumatology, CEREMAI, Bicêtre Hospital, AP-HP, University of Paris SUD, Le Kremlin-Bicêtre, France.
2
Internal medicine, CERAIF, Tenon hospital, AP-HP, University of Paris EsT, Paris, France.
3
Paediatrics, CEREMAI, Mignot Hospital, Versailles-Le Chainay, France.
4
Internal medicine, Huriez hospital, University of Lille, Lille, France.
5
General Paediatrics, Robert Debré Hospital, AP-HP, University of Paris, Paris, France.
6
Paediatric rheumatology and immunology, CERHUMIP, Necker Hospital, University of Paris, Paris, France.
7
Department of Paediatrics Rheumatology, CEREMAI, Bicêtre Hospital, AP-HP, University of Paris SUD, Le Kremlin-Bicêtre, France. Isabelle.kone-paut@aphp.fr.

Abstract

BACKGROUND:

Colchicine is the standard treatment for familial Mediterranean fever (FMF), preventing attacks and inflammatory complications. True resistance is rare and yet not clearly defined. We evaluated physicians' definition of colchicine resistance and report how they manage it.

PATIENTS AND METHODS:

We recruited patients with a clinical diagnosis of FMF, one exon-10 Mediterranean fever (MEFV) gene mutation and considered resistant to colchicine, via networks of expert physicians. Clinical, biological characteristics and information about colchicine treatment (dose adjustment, compliance) were collected. The severity of FMF was assessed by the Tel Hashomer criteria.

RESULTS:

We included 51 patients, most females (55%), mean age 34 ± 23.1 years years (range 4.7-86.3). Overall, 58% (27/47) patients had homozygous M694 MEFV gene mutations. Seventeen of 42 patients (40%) declared full adherence to colchicine treatment, greater for children (48%) than adults (22%). Physicians considered colchicine resistance with > 6 attacks/year (n = 21/51, 42%), > 4 attacks in the last 6 months (n = 13/51, 26%), persistent inflammation (n = 23/51, 45%), renal amyloidosis in (n = 6/28, 22%) of adult patients and intolerance to an increase in colchicine dose (n = 10/51, 19%), and other reasons (n = 13/51, 23%), including chronic arthralgia (n = 6/51, 12%). Interleukin 1-targeting drugs represented the only alternative treatments in addition to daily colchicine.

CONCLUSION:

Resistance to colchicine is rare (<10% of patients) and mostly observed in severe MEFV genotypes. The main reasons for physicians assessing resistance were severe clinical symptoms, persistent subclinical inflammation, and secondary amyloidosis. Low adherence to colchicine treatment is a key component of resistance.

KEYWORDS:

Anti interleukin 1; Colchicine; Compliance; Familial Mediterranean fever; Resistance to treatment

PMID:
28302131
PMCID:
PMC5356243
DOI:
10.1186/s13023-017-0609-1
[Indexed for MEDLINE]
Free PMC Article

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