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Introduction: We aimed to investigate the effects of colchicine use on first and second trimester screening markers in pregnancies complicated with familial Mediterranean fever (FMF) and to evaluate the overall impact of these effects on perinatal outcomes., Methods: A retrospective case-control study was conducted in pregnancies complicated with FMF using colchicine and healthy pregnancies as controls without any defined risk factors and medication use. Biochemical markers for the aneuploidy screening, including free ß-hCG and PAPP-A in the first trimester, and AFP, HCG, and unconjugated estriol (uE3) in the second trimester, were recorded, and MoM levels of these markers were compared between the FMF and control groups. Obstetric history and outcomes were also compared between groups. We used propensity score matching to form a cohort in which patients had similar baseline characteristics., Results: Among 93 eligible pregnant women, 31 women in FMF group and 31 in control group had similar propensity scores and were included in the analyses. Levels of serum-free ß-hCG, PAPP-A and AFP were similar between FMF and control groups (p = 0.671, p = 0.387 and p = 0.963, respectively). For the second-trimester markers, maternal serum uE3 MoM level were significantly lower in the FMF group using colchicine than in the controls (p = 0.045). We also compared these markers according to the daily colchicine dose between FMF subgroups. We did not detect significant difference between the different colchicine treatment modalities (0.5-1 mg/day vs. 1.5-2 mg/day, p > 0.05)., Conclusion: Maternal biochemical serum markers of an aneuploidy screening test in the second trimester may be affected by FMF with colchicine use, leading to misinterpretation of the risk level of tests. For these tests with decreased uE3 levels, FMF and colchicine use should be considered as a causative etiology after ruling out common etiologies and confounding factors before recommending invasive diagnostic testing., Competing Interests: Declaration of Competing Interest The authors have no relevant financial or non-financial interests to disclose., (Copyright © 2023. Published by Elsevier Masson SAS.)

Aim: To evaluate the sleep quality and the relation of sleep quality with depression, anxiety, fatigue and disease activity in adult patients with familial Mediterranean fever (FMF)., Method: One hundred and seventy-four FMF patients and 84 age-sex matched healthy individuals were included in this study. The Pittsburgh Sleep Quality Index (PSQI), Multidimensional Assessment of Fatigue (MAF) and the Hospital Anxiety and Depression Index (HADS) were used to assess sleep quality, fatigue, depression and anxiety, respectively., Result: FMF patients had significantly higher depression, anxiety, fatigue and PSQI scores than healthy controls. As the severity of the disease increased, scores of total PSQI and its domains increased. Patients with total PSQI score higher than 5 had statistically significantly higher erythrocyte sedimentation rates (ESR), serum C-reactive protein and serum amyloid levels during attacks, more attack numbers in last 3 months and worse fatigue, depression scores. Total PSQI score was positively correlated with inflammatory markers during attacks, attack numbers in the last 3 months and fatigue score. Logistic regression models identified disease duration, ESR during attacks, fatigue, attack numbers in the last 3 months as predictors of poor sleep quality., Conclusion: Poor sleep quality is common in adult FMF patients. Anxiety, depression and fatigue are more frequent in FMF patients than healthy individuals. Poor sleep quality is associated with inflammatory marker levels during attacks, fatigue and attack numbers in the last 3 months., (© 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients., Methods: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed., Results: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced., Conclusion: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients., (© 2017, American College of Rheumatology.)

Objective: To investigate the effect of anti-interleukin-1 (anti-IL-1) treatment on the frequency and severity of attacks and other disease-related clinical parameters and to evaluate the adverse effects associated with anti-IL-1 treatment in 26 patients with refractory familial mediterranean fever (FMF)., Methods: The study included 26 FMF patients followed up in our centre using colchicine for 4 months to 30 years. The treatment was switched to anti-IL-1 treatment for various reasons; 20 cases were resistant to colchicine, 8 were intolerant to colchicine, and 3 had prolonged arthritis under colchicine. Clinical response was monitored through the number of attacks, and laboratory inflammation was monitored through erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A concentrations. Colchicine resistance was defined as at least two attacks/month together with C-reactive protein and serum amyloid A levels above the normal range between attacks. The colchicine dose was increased to 2 mg/day before they were considered colchicine-resistant., Results: 24 patients used anakinra (100 mg/day), and 2 used canakinumab (150 mg/month), for -36 months. Sixteen patients with colchicine resistance had no attacks under anti-IL-1 treatment, and 4 had decreased frequency and duration of attacks. Seven of 8 patients intolerant to colchicine used anakinra, and 6 were attack-free under treatment, while 1 using canakinumab had attacks under treatment. One patient with prolonged arthritis used canakinumab but arthritis showed progression and the treatment was changed to IL-6 inhibitor. Three patients had injection site erythema and one had fatigue with anti-IL-1 treatment. Topical steroids with systemic antihistaminics were sufficient for symptom control in two cases, but canakinumab treatment was given due to severe injection site erythema in one case., Conclusion: Anti-IL-1 agents are rational treatment modalities in patients resistant or intolerant to colchicine. Anti-IL-1 agents can control FMF attacks quite effectively and they have a promising role in the treatment of FMF.