Effect of interleukin-1 antagonists on the quality of life in familial Mediterranean fever patients

Varan, Özkan; Küçük, Hamit; Babaoğlu, Hakan; Ataş, Nuh; Salman, Reyhan Bilici; Satış, Hasan; Öztürk, Mehmet Akif; Haznedaroğlu, Şeminur; Göker, Berna; Tufan, Abdurrahman

doi:10.1007/s10067-018-4384-8

Cited by 23 publications

(18 citation statements)

References 19 publications

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“…The highest agreement for colchicine resistance is the persistent elevation of acute phase reactants between the attacks ( 12 , 15 – 20 , 23 , 24 , 26 – 29 , 33 , 34 , 36 , 38 – 40 , 44 , 46 , 49 , 50 , 52 , 55 , 57 – 59 , 61 , 63 – 65 , 73 ). No consensus exists about attack frequency: some authors define colchicine resistance in patients who experience more than 1 typical inflammatory attack per 3 months ( 38 , 53 ), while others refer to colchicine resistance if there are more than 2 typical attacks per trimester ( 46 ) and still others if the patient has monthly attacks ( 35 , 40 , 48 , 51 , 52 , 55 , 59 , 62 – 65 ). None of the studies considers that attack frequency may vary with age and therefore the probable necessity to define this parameter differently in adults and children.…”

Section: Discussionmentioning

confidence: 99%

The Use of Interleukine-1 Inhibitors in Familial Mediterranean Fever Patients: A Narrative Review

et al. 2020

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Purpose: Familial Mediterranean fever (FMF) is the most common monogenic auto-inflammatory disease characterized by recurrent attacks of fever and serositis. It is associated with mutation in pyrin inflammasome leading to interleukin-1 (IL-1) over secretion. Although colchicine is the first line treatment in FMF, 5-10% of patients are reported in literature as non-responders. Colchicine is not always well-tolerated due either to its direct toxicity or to co-morbidities that preclude the administration of its proper dosage. For these patients an alternative or additional treatment to colchicine is necessary. This literature review reports the published data regarding the use of IL-1 inhibitors in Familial Mediterranean Fever. Results: There is no uniform definition of colchicine resistance, but the different studies of treatment with IL-1 inhibitors provide evidence of IL-1 pathogenic role in colchicine-resistant FMF. IL-1 inhibition is an efficacious option for controlling and preventing flares-at least at the short term-in FMF patients who are insufficiently controlled with colchicine alone. Although canakinumab is the only approved drug in Europe for colchicine resistant FMF treatment, experience with anakinra is also substantial. In the absence of comparative studies both treatments seem to be an equal option for the management of these patients. Overall the safety profile of IL-1 inhibitors seems not different in FMF patients than in the other diseases and can be considered as globally safe. The main side effects are local injection site reactions and infections. Conclusion: IL-1 inhibitors have the potential to improve patient outcome even in FMF patients with co-morbidities or severe complications in whom inflammation control is difficult to achieve with colchicine alone. Nevertheless, current data are limited and further evaluation of long-term efficacy and safety of IL-1 inhibitors are necessary, in order to provide robust evidence in this domain.

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Section: Discussionmentioning

confidence: 99%

The Use of Interleukine-1 Inhibitors in Familial Mediterranean Fever Patients: A Narrative Review

et al. 2020

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“…15 Therefore, anti-IL-1 treatment has been introduced in patients with ongoing clinical exacerbations with adequate doses of colchicine, or who continue to have subclinical inflammation without attacks. 16,17 Inadequate response to colchicine in FMF patients may occur due to a higher inflammatory activity via IL-1β pathway and a stronger inflammatory activity exceeding anti-inflammatory effects of colchicine. The higher inflammatory activity may be associated with MEFV variations, environmental factors or accompanying inflammatory conditions.…”

Section: Discussionmentioning

confidence: 99%

“…The goal of FMF treatment is to prevent periodic attacks, suppress inflammation, and prevent the development of amyloidosis 15 . Therefore, anti‐IL‐1 treatment has been introduced in patients with ongoing clinical exacerbations with adequate doses of colchicine, or who continue to have subclinical inflammation without attacks 16,17 …”

Section: Discussionmentioning

confidence: 99%

Effect of anti‐interleukin‐1 treatment on quality of life in children with colchicine‐resistant familial Mediterranean fever: A single‐center experience

et al. 2020

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Aim: The aim of this study is to evaluate the clinical parameters, acute-phase reactants, side effects, genetic mutations among colchicine-resistant Familial Mediterranean fever (FMF) patients who received anti-interleukin-1 (anti-IL-1) treatment. We also evaluate the quality of life and school attendance among colchicine-resistant FMF patients, in relation to treatment with anti-IL-1. Introductıon: Familial Mediterranean fever is the most common inherited autoinflammatory disorder. Although the main treatment of FMF is colchicine, a small group of patients are resistant to colchicine treatment. Anti-IL-1 treatment is promising in colchicine-resistant patients due to excessive IL-1β production in pathogenesis. The aim of this study is to evaluate the quality of life and school attendance rates among colchicine-resistant FMF patients after anti-IL-1 treatment. Methods: This is a single center retrospective study of 25 pediatric colchicine-resistant FMF patients treated with anti-IL-1 treatment. Autoinflammatory Disease Activity Index (AIDAI) was used for disease activity assessment. School attendance rates were evaluated before and after treatment. Results: There were 25 patients with FMF (11 M/14 F) who were treated with anakinra or canakinumab for various indications (colchicine-resistant recurrent febrile attacks in 20, colchicine-related side effects in 2, subclinical inflammation in 3 patients). Only 3 patients developed side effects with anakinra (2 headache, 1 urticarial rash). There was a significant decrease in the frequency of attacks, acute-phase reactants (erythrocyte sedimentation rate and C-reactive protein), AIDAI and physician's and patient's global assessment scores and improvement in school attendance rates. At the last follow-up, all patients were in remission, and only 3 had subclinical inflammation. Conclusıon: Anti-IL-1 treatment is quite effective in children with colchicine-resistant FMF patients, proven with improved AIDAI scores and school attendance rates. In the long term by lowering disease activation even development of amyloidosis may be prevented.

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“…Three IL-1 antagonists have been studied in the treatment of crFMF: anakinra, canakinumab, and rilonacept. All of the IL-1 antagonists significantly reduce the number of attacks and improve quality of life with good safety profiles [108,109]. Good quality studies are scarce for the use of IL-1 antagonists and all randomized or open label studies included relatively small numbers of patients.…”

Section: Treatment Of Colchicine-resistant or -Intolerant Patientsmentioning

confidence: 99%

Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review

Tufan¹,

Lachmann²

2020

Turk J Med Sci

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111

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Familial Mediterranean fever (FMF) (OMIM #249100) is the most common hereditary autoinflammatory disease in the world. FMF is caused by gain of function mutations of MEFV gene which encodes an immune regulatory protein, pyrin. Over the last few years, we have witnessed several new developments in the pathogenesis, genetic testing, diagnosis, comorbidities, disease related damage and treatment approaches to FMF. Elucidation of some of the pathogenic mechanisms has led to the discovery of pathways involved in inflammatory, metabolic, cardiovascular and degenerative diseases. The use of next generation sequencing in FMF has revealed many new gene variants whose clinical significance may be clarified by developing functional assays and biomarkers. Clinically, although FMF is considered an episodic disease characterized by brief attacks, recent systematic studies have defined several associated chronic inflammatory conditions. Colchicine is the mainstay of FMF treatment, and interleukin (IL)-1 antagonists are the treatment of choice in refractory or intolerant cases. Experience of IL-1 antagonists, anakinra and canakinumab, is now available in thousands of colchicine resistant or intolerant FMF patients. In this contemporary review, we surveyed current FMF knowledge in the light of these recent advances.

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Effect of interleukin-1 antagonists on the quality of life in familial Mediterranean fever patients

Cited by 23 publications

References 19 publications

The Use of Interleukine-1 Inhibitors in Familial Mediterranean Fever Patients: A Narrative Review

The Use of Interleukine-1 Inhibitors in Familial Mediterranean Fever Patients: A Narrative Review

Effect of anti‐interleukin‐1 treatment on quality of life in children with colchicine‐resistant familial Mediterranean fever: A single‐center experience

Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review

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