TY - JOUR
T1 - Colchicine-free remission in familial Mediterranean fever
T2 - Featuring a unique subset of the disease-a case control study
AU - Ben-Zvi, Ilan
AU - Krichely-Vachdi, Tami
AU - Feld, Olga
AU - Lidar, Merav
AU - Kivity, Shaye
AU - Livneh, Avi
N1 - Funding Information:
This study was supported by a grant from Johanna Feller family in memory of her and other family members, who were the victims of the Nazi holocaust. Dr. Ilan Ben-Zvi is supported by The Talpiot Medical Leadership Program fellowship, Sheba Medical Center.
PY - 2014/1/9
Y1 - 2014/1/9
N2 - 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.
AB - 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.
KW - Colchicine
KW - Demographics
KW - Familial Mediterranean fever
KW - Genetics
KW - Remission
KW - Severity
UR - http://www.scopus.com/inward/record.url?scp=84892186708&partnerID=8YFLogxK
U2 - 10.1186/1750-1172-9-3
DO - 10.1186/1750-1172-9-3
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AN - SCOPUS:84892186708
VL - 9
JO - Orphanet Journal of Rare Diseases
JF - Orphanet Journal of Rare Diseases
SN - 1750-1172
IS - 1
M1 - 3
ER -