Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey

Kayaalp, Gülşah Kavrul; Sözeri, Betül; Sönmez, Hafize Emine; Demir, Ferhat; Çakan, Mustafa; Öztürk, Kübra; Karadağ, Şerife Gül; Yener, Gülçin Otar; Özdel, Semanur; Bağlan, Esra; Çelikel, Elif; Şahin, Nihal; Yıldırım, Deniz Gezgin; Ömeroğlu, Rukiye Eker; Ayaz, Nuray Aktay

doi:10.1007/s00296-020-04776-1

Cited by 6 publications

(6 citation statements)

References 42 publications

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“…In the studies conducted with canakinumab, no side effects were observed other than mild infection, the drug was reported to be safe, and no severe side effects were observed in our study [ 36 – 38 ]. No serious adverse events were observed in the present study, and patients with SARS-CoV2 infection were treated without hospitalization.…”

Section: Discussionsupporting

confidence: 54%

“…There are limited data on the drug’s dose intervals and discontinuation in the adult patient group. If no attacks and subclinical inflammation are observed after starting canakinumab, dose intervals can be doubled initially and then tripled if no new attack occurs within one year [ 38 ]. As the present study is a real-life study, which included a limited number of cases and is conducted in routine outpatient clinic conditions, it may contribute to the literature in this respect.…”

Section: Discussionmentioning

confidence: 99%

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Real-life data on tapering or discontinuation of canakinumab therapy in patients with familial Mediterranean fever

Karabulut¹,

Gezer

Öz

et al. 2022

Rheumatol Int

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This study aimed to investigate the disease characteristics of familial Mediterranean fever (FMF) patients undergoing dose optimisation and discontinuation of canakinumab therapy. A total of 61 patients diagnosed with FMF and using canakinumab for the resistant disease were enrolled on this retrospective study. Patients’ characteristics, disease activity, treatment response, dose optimisation, dose intervals, attack-free periods, drug-free periods and side effects were noted. Dose intervals were extended in patients who achieved remission without being bound by any protocol at the discretion of the rheumatology physician who followed up with the patients in the outpatient clinic. The drug was discontinued in some patients whose dose intervals were 2 months or longer and remained in remission for 6 months or longer. A total of 57 patients (56% female, median age 32.4 years) were included. The mean attack frequency before canakinumab was 3.4/6 months, while it was 1.2 at the last post-treatment visit ( p < 0.001). The median duration of canakinumab use was 46 months. After the first 6 months, the dosing interval was extended in 22 patients, and then treatment was discontinued in 12 of them who did not have an attack in the last 6 months. Three of the 12 patients whose treatment was discontinued started monthly treatment again after their attacks recurred. In the remaining ten patients, dose intervals were extended to 8–12 weeks after 6 months of monthly treatment. Nine patients are still being followed up without attacks and receive only colchicine therapy. Canakinumab is a safe and effective treatment, dose intervals may be extended, and follow-up without medication may be possible for eligible patients. However, there is a need for a consensus on dose optimisation or tapering.

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Section: Discussionsupporting

confidence: 54%

Section: Discussionmentioning

confidence: 99%

Real-life data on tapering or discontinuation of canakinumab therapy in patients with familial Mediterranean fever

Karabulut¹,

Gezer

Öz

et al. 2022

Rheumatol Int

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“…The percentage of colchicine‐resistant patients in this study was 19.5%. Previous studies have reported that approximately 5%–10% of FMF patients were not responsive to colchicine 10,15,22 . Long‐term administration of colchicine in pediatric FMF patients has been shown to provide complete remission in 64% of patients.…”

Section: Discussionmentioning

confidence: 99%

Colchicine resistance: Associated factors and its effect on health‐related quality of life in patients with familial Mediterranean fever

et al. 2022

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Aim To determine the factors associated with colchicine resistance and the effect of colchicine resistance on health‐related quality of life (QoL) in patients with familial Mediterranean fever (FMF). Methods Demographic and clinical features, MEFV gene mutations, and Pras disease severity scores were recorded. QoL was assessed using the Short Form‐36 (SF‐36) and FMF‐QoL scales. Colchicine resistance was defined as at least 1 attack per month for 6 months at the maximum tolerated dose of colchicine in fully compliant patients. Results The mean age of 118 patients (90 female, 28 male) with FMF was 38.4 ± 12.5 years. The percentage of colchicine‐resistant patients was 19.5%. In univariable analysis, smoking (odds ratio [OR] = 2.885; 95% confidence interval [CI] = 1.104–7.539; P = 0.031), attack duration (OR = 1.955; 95% CI = 1.137–3.360; P = 0.015), presence of arthritis (OR = 5.235; 95% CI = 1.508–18.179; P = 0.009), and disease severity score (OR = 1.790; 95% CI = 1.334–2.402; P < 0.001) were associated with colchicine resistance. The FMF‐QoL and subscales of SF‐36 except for role emotional and vitality, were different between colchicine‐resistant and non‐resistant patients (P < 0.05). Conclusion Smoking, attack duration, presence of arthritis, and disease severity were associated with colchicine resistance in fully compliant FMF patients. Colchicine‐resistant patients had poorer health‐related QoL.

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“…Based on the present findings, we think that as the quantity of real-life data increases, standard CAN protocols may be developed. Recently, a modified Delphi study suggested that in patients without any attacks or laboratory evidence of subclinical inflammation within 6 months of initiation of biologics the CAN dose interval can be doubled and in the absence of any new attacks within 1 year the dose interval can be tripled (5). Based on these suggestions, the same group of researchers are presently conducting a prospective study.…”

Section: Discussionmentioning

confidence: 99%

“…There is no standard definition for "colchicine resistance" in FMF patients. Clinicians agree that patients may be considered colchicine resistant in the presence of ongoing clinical disease activity and inflammation, whereas opinions vary regarding the frequency of attacks required to define patients as colchicine resistant (4,5). With the advent of translational research, anti-IL-1 agents have emerged as a new and effective therapeutic approach in colchicine-resistant FMF (crFMF) patients (6)(7)(8)(9)(10).…”

Section: Introductionmentioning

confidence: 99%

The feasibility of withdrawing canakinumab in paediatric colchicine-resistant familial Mediterranean fever patients

Sözeri

Sönmez

Karadağ

et al. 2021

Clinical and Experimental Rheumatology

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Objective. To determine the feasibility of withdrawing canakinumab (CAN) in a large cohort of paediatric patients with colchicine-resistant familial Mediterranean fever (crFMF). Methods. This retrospective observational cohort study included paediatric crFMF patients that received CAN treatment for ≥6 months. Patient data were recorded at treatment onset (baseline), and at 1, 3, 6, 12, 18, and 24 months after initiation of treatment.Results. The study included 114 patients that were followed-up for 2736 person-months. During the 24-month follow-up period, the CAN dose interval remained unchanged in 44 patients. The dose interval was extended in 58 patients within a median 6 months (range: 3-18 months) of treatment initiation. In all, 4 of these 58 patients had a new attack of crFMF after the dose interval was extended. CAN was withdrawn in 12 patients (in 5 at month 12 month and in 7 at month 18), of which 2 had a new attack within 3 months of withdrawal. In these 2 patients CAN was re-initiated with a dose interval of 8 weeks. The remaining 10 patients in which CAN was withdrawn did not report any symptoms throughout the remainder of the 24-month follow-up period. The median attack-free period in those treated with . Conclusion. The present findings showthat it may be feasible to withdraw CAN or extend its dose interval in paediatric crFMF patients. Based on the present findings, we think that as the quantity of real-life data increases, standard CAN protocols may be developed.

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Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey

Cited by 6 publications

References 42 publications

Real-life data on tapering or discontinuation of canakinumab therapy in patients with familial Mediterranean fever

Real-life data on tapering or discontinuation of canakinumab therapy in patients with familial Mediterranean fever

Colchicine resistance: Associated factors and its effect on health‐related quality of life in patients with familial Mediterranean fever

The feasibility of withdrawing canakinumab in paediatric colchicine-resistant familial Mediterranean fever patients

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