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01.12.2014 | Research | Ausgabe 1/2014 Open Access

Orphanet Journal of Rare Diseases 1/2014

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

Zeitschrift:
Orphanet Journal of Rare Diseases > Ausgabe 1/2014
Autoren:
Ilan Ben-Zvi, Tami Krichely-Vachdi, Olga Feld, Merav Lidar, Shaye Kivity, Avi Livneh
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1750-1172-9-3) contains supplementary material, which is available to authorized users.
Ilan Ben-Zvi, Tami Krichely-Vachdi contributed equally to this work.

Competing interest

The authors declare that they have no competing interest.

Authors’ contributions

AL, IBZ and TKV designed the study and wrote the manuscript. IBZ and TKV recruited patients to the study, collected the data and analyzed the results. OF, ML and SK recruited patients to the study and critically reviewed the manuscript. All authors read and approved the final manuscript.

Abstract

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.
Zusatzmaterial
Authors’ original file for figure 1
13023_2013_694_MOESM1_ESM.tif
Literatur
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