Colchicine-free remission in familial Mediterranean fever: featuring a unique subset of the disease-a case control study

Orphanet Journal of Rare Diseases

Avi Livneh¹  Shaye Kivity³  Merav Lidar¹  Olga Feld²  Tami Krichely-Vachdi²  Ilan Ben-Zvi³ 

[1] Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel;Department of Medicine F and the Rheumatology unit, Sheba Medical Center, Tel-Hashomer, Israel;The Pinchas Borenstein Talpiot Medical Leadership Program 2012, Chaim Sheba, Medical Center, Tel-Hashomer, Israel

关键词: Colchicine;    Severity;    Demographics;    Genetics;    Remission;    Familial Mediterranean fever;   

Others  :  863327
DOI  :  10.1186/1750-1172-9-3

 received in 2013-10-10, accepted in 2014-01-03,  发布年份 2014

【 摘 要 】

Background

To demonstrate and clinically, genetically and demographically characterize familial Mediterranean fever (FMF) patients, maintaining remission despite colchicine abstinence.

Methods

FMF patients were screened for an endurance of prolonged remission (≥ 3 years), despite refraining from colchicine. Clinical, demographic and genetic parameters were collected. Data were compared with those of consecutive control FMF subjects, coming to the clinic for their periodic follow up examination.

Results

Of 1000 patients screened over 5 years, 33 manifested colchicine-free remission. The mean duration of the remission period was 12.6 ± 8.1 years. Patients in the remission group had milder severity of FMF, compared to the control group (22 vs. 11 patients with mild disease, respectively, p = 0.003) and a longer diagnosis delay (21 ± 15.7 vs. 13.4 ± 13.5 years, respectively, p = 0.04). Patients experiencing remission suffered mostly of abdominal attacks, low rate of attacks in other sites and low rate of chronic and non-attack manifestations. When the disease resumed activity, it responded well to colchicine, despite using a lower dose, as compared to the control subjects (p < 0.001). None of the patients in this group was homozygous for the M694V mutation (p = 0.0008).

Conclusions

Prolonged colchicine-free remission defines a rare and milder form of FMF with unique clinical, demographic, and molecular characteristics.

【 授权许可】

   
2014 Ben-Zvi et al.; licensee BioMed Central Ltd.

【 图 表 】

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