Canakinumab is effective in patients with familial Mediterranean fever resistant and intolerant to the colchicine and/or anakinra treatment

Karabulut, Yusuf; Gezer, Halise Hande; Duruöz, Mehmet Tuncay

doi:10.1007/s00296-021-04997-y

Cited by 8 publications

(4 citation statements)

References 22 publications

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“…Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disease characterized by self-limiting episodes of fever, serositis, or synovitis. FMF is common among Eastern Mediterranean populations, mainly Turkish, Jewish, Armenian, and Arab; however, it can also be seen sporadically in different parts of the world [ 1 , 2 ]. In 60–90% of patients with FMF, symptoms begin before the age of 20 and in 10–20% after the age of 20 [ 3 ].…”

Section: Introductionmentioning

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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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: Introductionmentioning

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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“…Colchicine demonstrated efficacy in preventing acute inflammatory episodes as well as preventing or slowing the progression toward amyloidosis [15]. Canakinumab is the only FDA-approved cytokine blocker for the treatment of colchicine-resistant FMF in the United States and was found to be effective and tolerable [16].…”

Section: Discussionmentioning

confidence: 99%

Acute Myopericarditis as the First Manifestation of Familial Mediterranean Fever: A Case Report

Khalil,

Greenhalgh,

Gurung

et al. 2024

Cureus

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Familial Mediterranean fever (FMF) is an autoinflammatory disorder, characterized by recurrent episodes of fever and polyserositis, and usually presents during the first two decades of life. Acute pericarditis is a rare manifestation of FMF and typically presents with other symptoms of the inflammatory disorder. A 27-yearold Arabian male presented to our hospital with pleuritic chest pain and shortness of breath while lying flat. His electrocardiogram showed changes suggestive of pericarditis, and his inflammatory markers and troponin were raised. His echocardiogram revealed a moderate-sized pericardial effusion with septa and a normal left ventricular function. He had a strong family history of FMF and consanguinity of the parents. He was treated for acute myopericarditis with colchicine and ibuprofen, and his symptoms improved gradually along with his inflammatory markers and troponin. Six weeks after discharge, he had a cardiac MRI, which revealed a thickened pericardium with profound enhancement (features suggestive of pericarditis) and no signs of myocarditis. He was asymptomatic, and his markers and troponin were within the normal range. His colchicine medication was continued indefinitely, and he was referred to a tertiary care hospital with a specialized periodic fever clinic for follow-up and genotype testing.

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“…We took detailed medical history and physical examination with special attention to identify potential peripheral neuropathies, and none of the patients had the symptoms such as numbness, tingling, pain or tremor. Generally, the preference of one treatment over the other can therefore be based on the price and/or the country’s payment conditions of the drugs [ 27 ]. Despite the possible toxic effects on the peripheral nervous system, this drug has the advantage of a low cost and could be considered in developing countries where the use of biologic agents is limited for economic reasons [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Efficacy and safety of thalidomide in children with monogenic autoinflammatory diseases: a single-center, real-world-evidence study

Zhang,

Yu,

Gao

et al. 2023

Pediatr Rheumatol

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Background Monogenic autoinflammatory diseases (AIDs) are rare inflammatory diseases caused by genetic variants. The pathogenesis is complex and treatment options are limited. This study aimed to describe the safety and efficacy of thalidomide in the treatment of monogenic AIDs. Methods This was a single-center, single-arm, real-world study. From September 2016 to August 2021, patients with monogenic AIDs who met the inclusion and exclusion criteria were given thalidomide for 12 months. There was a 3-month run-in period before dosing. The efficacy and adverse events were evaluated and recorded every 3 months. After 3 and 12 months of thalidomide treatment, clinical manifestations, disease activity score, inflammatory markers, and background medication adjustments were compared with baseline for efficacy analyses. Results A total of 16 patients entered this study, including 3 with Aicardi-Goutières syndrome (AGS), 4 Blau syndrome, 2 chronic infantile neurologic cutaneous articular syndrome (CINCA), 2 A20 haploinsufficiency (HA20), 1 adenosine deaminase 2 deficiency(DADA2), 1 familial Mediterranean fever (FMF),1 tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS), 1 PLCγ2-associated antibody deficiency and immune dysregulation (PLAID), and 1 stimulator of interferon genes-associated vasculopathy with onset in infancy(SAVI). The efficacy rate in the 16 patients after 3-month and 12-month thalidomide treatment in patients was 56.3%. Twelve patients completed the study, the fever improved in all of them, rash improved in 7 patients, and 5 patients stopped using glucocorticoids or other immunosuppressive agents. C-reactive protein was normal in 8 patients and erythrocyte sedimentation rate was normal in 11 patients. Anorexia and nausea occurred in 2 cases, with no other reported drug-related adverse reactions. Conclusion The largest cohort of monogenic AIDs with the treatment of thalidomide demonstrated that thalidomide can help reduce disease activity and inflammation, reduce the dosage of glucocorticoids, and improve clinical outcomes. Thalidomide is relatively safe in monogenic AIDs.

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Canakinumab is effective in patients with familial Mediterranean fever resistant and intolerant to the colchicine and/or anakinra treatment

Cited by 8 publications

References 22 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

Acute Myopericarditis as the First Manifestation of Familial Mediterranean Fever: A Case Report

Efficacy and safety of thalidomide in children with monogenic autoinflammatory diseases: a single-center, real-world-evidence study

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