PW01-015 – Canakinumab in adults with colchicin resistant FMF

Gül, Asiye; Özdoğan, Huri; Kasapçopur, Özgür; Erer, Burak; Uğurlu, Serdal; Davis, Nicole L.; Sevgi, Serhan

doi:10.1186/1546-0096-11-s1-a68

Cited by 2 publications

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“…A Phase II proof-of-concept study of canakinumab in crFMF involved three injections of open-label canakinumab 1 month apart in nine adults with a median attack rate of 1.2/month, most of whom had a poor condition as per the Physician's Global Assessment score criteria. 41,42 Most patients had a complete and satisfactory response, with an increase in attack severity during the follow-up period when the drug was withdrawn (Figure 2). 41 A study of canakinumab in paediatric patients with severe crFMF (median 2.8 attacks/month) also demonstrated an improvement in Physician's Global Assessment score during treatment.…”

Section: Outlook: Other Il-1 Driven Diseasesmentioning

confidence: 99%

From Bench to Bedside: Targeting the IL-1β Pathway in Autoinflammatory Diseases

McEvoy¹

2016

EMJ Rheumatol

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Prof Gerd Horneff opened the symposium with a summary of the recent advances in the understanding of the role of interleukin (IL)-1β in the pathogenesis of autoinflammatory (AI) diseases. Prof Angelo Ravelli then discussed the concept of ‘treat-to-target’ in systemic juvenile idiopathic arthritis (SJIA) and the various tools that can be used for monitoring treatment response. Dr Jasmin B Kümmerle-Deschner followed with an overview of the efficacy and safety data from the β-Confident Registry of patients with cryopyrin-associated periodic syndromes (CAPS) receiving treatment with canakinumab. Prof Fabrizio De Benedetti concluded with an overview of the up-and-coming data in the field of AI diseases. The objectives of the symposium were to summarise new insights in the pathogenesis of AI diseases including the role of IL-1β; to evaluate the use of anti-IL-1β and other treatment options for AI diseases; to describe the treat-to-target concept and recognise the potential of a tight disease control strategy in patients with SJIA; to integrate new knowledge on the long-term use of canakinumab in patients with CAPS based on the latest β-Confident Registry data; and to discuss management strategies in patients with AI diseases.

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Section: Outlook: Other Il-1 Driven Diseasesmentioning

confidence: 99%

From Bench to Bedside: Targeting the IL-1β Pathway in Autoinflammatory Diseases

McEvoy¹

2016

EMJ Rheumatol

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The Emerging Treatments in Familial Mediterranean Fever

Özdoğan

Uğurlu

2015

Rare Diseases of the Immune System

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9As described in detail by Ben-Chetrit in the previous chapter, colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF) [ 1 , 2 ]. If used daily for lifelong, it controls the febrile attacks in the majority of the patients and prevents the development of amyloidosis, the most serious complication of the disease.Colchicine is very effective as shown in controlled trials [ 3 ] and within the therapeutic range. Side effects are rare and/or mild, so that it can be used even during pregnancy and lactation, plus it is very cheap; however, even colchicine has some limitations in the treatment of FMF:1. Response to therapy: A small proportion of patients with FMF continue to experience attacks despite proper dose and good compliance to colchicine. Overall, it has been accepted that colchicine prevents febrile attacks in more than 60 % of patients and signifi cantly reduces the number of attacks in another 20-30 %. Five to ten percent of patients do not respond to therapy [ 4 , 5 ].Compliance is a main issue among patients who report attacks under colchicine treatment. Approximately 40 % of the patients are not compliant and receive lower than the dose needed to control the infl ammatory attacks and thus prone to high risk of amyloidosis [ 6 ]. Therefore, it is very important to check the compliance and the daily dose before diagnosing the patient as unresponsive to colchicine therapy. Recently the data that comes from the Eurofever Registry which collects information on patients with various autoinfl ammatory diseases, including FMF, showed that among the 121 FMF patients on colchicine, 62 % reported complete, 36 % partial, and 2 % no response [ 7 ]. In another recent report, only 5 % of the compliant patients on proper dose of colchicine continued to experience frequent attacks [ 8 ].

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PW01-015 – Canakinumab in adults with colchicin resistant FMF

Cited by 2 publications

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From Bench to Bedside: Targeting the IL-1β Pathway in Autoinflammatory Diseases

From Bench to Bedside: Targeting the IL-1β Pathway in Autoinflammatory Diseases

The Emerging Treatments in Familial Mediterranean Fever

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