Anakinra for colchicine refractory familial Mediterranean fever: a cohort of 44 patients

L, Marko; Shemer, Asaf; Lidar, Merav; Grossman, Chagai; Druyan, Amit; Livneh, Avi; Kivity, Shaye

doi:10.1093/rheumatology/keaa728

Cited by 17 publications

(13 citation statements)

References 26 publications

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“…13,14,17,[20][21][22]24 Anti-IL-1 therapies are highly beneficial in controlling inflammatory attacks in colchicine-resistant patients and may prevent ASBO. 9,10 There are no reports on the efficacy of anti-IL-1 therapy specifically in FMF patients whose disease is complicated with ASBO. However, Talerico et al 29 described a 65-year-old patient diagnosed with peritoneal mesothelioma due to repeated peritoneal inflammation of FMF.…”

Section: Discussionmentioning

confidence: 99%

Adhesive small‐bowel obstruction as a challenging complication of familial Mediterranean fever: A case‐based review

Küçükali,

Gezgin Yıldırım,

Esmeray Şenol

et al. 2023

Int J of Rheum Dis

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Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self‐limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis. Physicians are generally aware of other complications associated with FMF but reports on peritoneal adhesions and intestinal obstruction in English‐language literature are inadequate to increase clinicians' awareness. Therefore, it is crucial to meticulously evaluate FMF patients presenting with abdominal pain and ileus because these symptoms could be due to adhesive small‐bowel obstruction (ASBO). Furthermore, patients presenting with ASBO without a history of abdominal surgery should also be thoroughly evaluated, especially as it could be an initial presentation for an autoinflammatory disease. Herein, we present a pediatric case of FMF with the M694V homozygous mutation, complicated by ASBO while under colchicine treatment. Additionally, we provide a comprehensive review of the available literature on ASBO in FMF.

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Section: Discussionmentioning

confidence: 99%

Adhesive small‐bowel obstruction as a challenging complication of familial Mediterranean fever: A case‐based review

Küçükali,

Gezgin Yıldırım,

Esmeray Şenol

et al. 2023

Int J of Rheum Dis

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“…Marko et al showed that the Global Assessment Score assessed by the number of attacks per month, duration of attacks, and number of sites involved in the attacks decreased signi cantly from 6.6 (IQR 5.3-7.8) to 2 (IQR 0-4.2) in 44 colchicine-resistant patients with anakinra. Anakinra was reported to be bene cial for the majority of colchicine-resistant FMF patients [17]. Ben-Zvi et al found that among 25 patients with colchicine-resistant FMF, who were randomly assigned to groups that received anakinra consisting of 12 patients and placebo consisting of 13 patients, the number of monthly attacks was 1.7 ± 1.7 in the anakinra group and 3.5 ± 1.9 in the placebo group.…”

Section: Discussionmentioning

confidence: 99%

Does Switching Colchicine Preparations Have a Role in The Treatment of Familial Mediterranean Fever?

Öner

Çelikel

Tekin

et al. 2022

Preprint

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Objective: The aim of this study was to evaluate the treatment response of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. Another objective was to determine the demographic and clinical characteristics of the group that responded to compressed colchicine preparations. Methods: In this retrospective study, the files of 1574 patients with FMF in our center were reviewed. Sixty-one patients who did not respond to coated colchicine and were switched to compressed colchicine treatment were evaluated. The number of attacks and the International Severity Score for FMF (ISSF) during the 6 months before and 3, 6, 9, 12, and 24 months after switching from coated colchicine to compressed colchicine were recorded. Results: Of the 61 patients, 36 (59%) were boys, and 25 (41%) were girls. The mean age of symptom onset was 34±22 months. The mean age at diagnosis was 8.4±5.1 years. The mean age at the time of switching from coated colchicine to compressed colchicine was 11.8±3.5 years. Among 61 patients whose treatments were switched to compressed colchicine, 12 had intolerance, while 49 had uncontrolled attacks and persistent subclinical inflammation. The problems of 12 patients who were switched to compressed colchicine due to intolerance were resolved. Response to compressed colchicine was observed in 25/49 patients with uncontrolled attacks and persistent subclinical inflammation. The frequency of attacks decreased significantly at the 3rd and 6th months after switching from coated colchicine to compressed colchicine (p<0.05). The mean ISSF score of the patients in the last 3 months while receiving coated colchicine was 5.4±2.3. The mean ISSF scores of the patients at the 3rd and 6th months after switching to compressed colchicine were 3.2±2.1 and 2.6±1.7, respectively. Twenty-four of the 49 patients did not respond to compressed colchicine. Anakinra was added to the treatments of 14 of the 24 patients, and canakinumab was added to the treatments of 10. The frequency of attacks and ISSF scores of the patients who did not respond to compressed colchicine significantly decreased after the addition of IL-1-targeting drugs to their treatments. At the end of the two-year follow-up, 42 patients were tolerant to compressed colchicine, and 19 patients (4 anakinra, 15 canakinumab) were on compressed colchicine plus IL-1-targeting drugs. Conclusions: Compressed colchicine was shown to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine, especially those with side effects.

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“…Although the treatment period was much longer and the population much larger, than in the RCT, the results were comparable. Anakinra has been shown to reduce attack rate and severity, decrease the levels of inflammatory markers, and achieve improvement in composite measures, that included patient and physician global assessments such as FMF50 or global assessment score [ 139 , 140 , 141 , 142 , 143 ]. Usually, the toll of safety was minimal, mainly consisting of injection-site reactions, as discussed separately in Section 7.13 .…”

Section: Anakinra In Fmfmentioning

confidence: 99%

“…Shortly after the introduction of anakinra for the treatment of FMF, increasing evidence has begun to accumulate, suggesting that anakinra may favorably affect FMF-amyloidosis. Based on published data, it appears that anakinra may lead to stabilization [ 143 , 146 , 147 ], regression [ 139 , 142 , 147 , 148 ], and normalization of urinary protein levels, even in patients with nephrotic range proteinuria [ 139 , 149 ]. Pitifully, this beneficial effect is limited to early stages of amyloidosis, before significant renal failure develops [ 146 , 147 , 149 ].…”

Section: Anakinra In Fmfmentioning

confidence: 99%

The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease

Giat

Ben-Zvi

Lidar

et al. 2022

IJMS

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Familial Mediterranean fever (FMF), the most frequent monogenic autoinflammatory disease, is manifested with recurrent and chronic inflammation and amyloid A (AA) amyloidosis, driven by overproduction of interleukin 1 (IL-1) through an activated pyrin inflammasome. Consequently, non-responsiveness to colchicine, the cornerstone of FMF treatment, is nowadays addressed by IL-1- blockers. Each of the two IL-1 blockers currently used in FMF, anakinra and canakinumab, has its own merits for FMF care. Here we focus on anakinra, a recombinant form of the naturally occurring IL-1 receptor antagonist, and explore the literature by using PubMed regarding the utility of anakinra in certain conditions of FMF. Occasionally we enrich published data with our own experience. To facilitate insights to anakinra role, the paper briefs some clinical, genetic, pathogenetic, and management aspects of FMF. The clinical settings of FMF covered in this review include colchicine resistance, AA amyloidosis, renal transplantation, protracted febrile myalgia, on- demand use, leg pain, arthritis, temporary suspension of colchicine, pediatric patients, and pregnancy and lactation. In many of these instances, either because of safety concerns or a necessity for only transient and short-term use, anakinra, due to its short half-life, is the preferred IL-1 blocker.

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Anakinra for colchicine refractory familial Mediterranean fever: a cohort of 44 patients

Cited by 17 publications

References 26 publications

Adhesive small‐bowel obstruction as a challenging complication of familial Mediterranean fever: A case‐based review

Adhesive small‐bowel obstruction as a challenging complication of familial Mediterranean fever: A case‐based review

Does Switching Colchicine Preparations Have a Role in The Treatment of Familial Mediterranean Fever?

The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease

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