scholarly journals Effectiveness of Canakinumab Treatment in Colchicine Resistant Familial Mediterranean Fever Cases

2021 ◽  
Vol 9 ◽  
Author(s):  
Burcu Bozkaya Yücel ◽  
Ozlem Aydog ◽  
Hulya Nalcacioglu ◽  
Ayşegül Yılmaz
Keyword(s):  
Complete Remission ◽  
Familial Mediterranean Fever ◽  
Interleukin 1 ◽  
Age At Onset ◽  
Dose Interval ◽  
Renal Amyloidosis ◽  
Cervical Lymphadenitis ◽  
Dosing Interval ◽  
Mediterranean Fever ◽  
The Mean

Anti-interleukin 1 agents are used successfully in colchicine-resistant or intolerant Familial Mediterranean Fever (FMF) patients. Sixty-five patients with FMF who received canakinumab treatment for at least 6 months due to colchicine resistance or intolerance between 2016 and 2020 in our department were retrospectively analyzed. Canakinumab treatment was given subcutaneously every 4 weeks. After completing monthly canakinumab therapy over 12 months, in patients with complete remission, the dosing interval was extended to every 1.5 months for 6 months, then every 2 months for 6 months, and finally every 3 months for a year. In patients without disease activation, canakinumab treatment was discontinued at the end of 3 years and followed up with colchicine treatment. Patients who had a flare switched to the previous dosing interval. In patients with renal amyloidosis, monthly canakinumab treatment was continued without extending the dose intervals. The mean duration of canakinumab use in our patients was 31.4 ± 10.57 months (6–52 months). The mean age at onset of symptoms was 4.65 ± 3.84 (range, 1–18) years, and the mean age at diagnosis was 5.59 ± 3.9 (range, 4–19) years. Complete remission was achieved in 57 (87.6%) and partial remission in seven (10.7%) patients. One patient was unresponsive to treatment. Canakinumab treatment was discontinued in three patients with complete remission and one patient with drug resistance. Erythrocyte sedimentation rate (ESR) (51.85 ± 15.7 vs. 27.80 ± 13.73 mm/h) and C-reactive protein (CRP) [26 (3-73) vs. 5 (1–48) mg/L] values were compared before and after canakinumab treatment in attack-free periods, a significant decrease was found after canakinumab treatment (p < 0.001, p < 0.001, respectively). Bodyweight Z-scores (respectively −0.80 ± 0.86 vs. −0.49 ± 0.92) were compared, similarly, a statistically significant increase after canakinumab treatment (p < 0.001), but no significant increase in height Z scores (−1.00 ± 0.88 vs. −0.96 ± 0.94) (p = 0.445) was detected. Four patients had FMF-related renal amyloidosis. The decrease in proteinuria with canakinumab treatment was not statistically significant (p = 0.068). Cervical lymphadenitis developed in one and local reactions in two patients. No severe adverse effects requiring discontinuation of canakinumab treatment were observed. Our study showed that canakinumab treatment was highly effective, well-tolerated in pediatric FMF patients, and controlled extension of the canakinumab dose interval was safe.

scholarly journals Complete remission of nephrotic syndrome in a woman with renal amyloidosis due to Familial Mediterranean Fever

2016 ◽  
Vol 36 (3) ◽  
pp. 331-332
Author(s):  
Manuel Heras ◽  
Ana Saiz ◽  
María José Fernández-Reyes ◽  
Álvaro Molina ◽  
María Astrid Rodríguez ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Complete Remission ◽  
Familial Mediterranean Fever ◽  
Renal Amyloidosis ◽  
Mediterranean Fever

Interleukin-1 Targeting Drugs in Familial Mediterranean Fever: A Case Series and a Review of the Literature

2011 ◽  
Vol 41 (2) ◽  
pp. 265-271 ◽  
Author(s):  
Ulrich Meinzer ◽  
Pierre Quartier ◽  
Jean-François Alexandra ◽  
Véronique Hentgen ◽  
Frédérique Retornaz ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Interleukin 1 ◽  
Case Series ◽  
Review Of The Literature ◽  
Mediterranean Fever

scholarly journals Abdominal syndrome in monogenic autoinflammatory disease – is it just a familial Mediterranean fever?

2020 ◽  
Vol 14 (4) ◽  
pp. 144-149
Author(s):  
S. O. Salugina ◽  
E. S. Fedorov ◽  
N. G. Volf
Keyword(s):  
Familial Mediterranean Fever ◽  
Autoinflammatory Disease ◽  
Bowel Perforation ◽  
Interleukin 1 ◽  
Diagnostic Features ◽  
Peritoneal Adhesions ◽  
Strict Adherence ◽  
Surgical Interventions ◽  
Abdominal Symptoms ◽  
Mediterranean Fever

Gastrointestinal (GI) manifestations, such as abdominal pain, nausea, vomiting, and diarrhea, are common autoinflammatory disease (AID) symptoms. The abdominal symptomatology reflecting serositis is one of the most important classification and diagnostic criteria for the classic monogenic AID (MAID) – familial Mediterranean fever (FMF). Failure to timely diagnose FMF frequently leads to unjustified surgical interventions. Other periodic fevers may also present as abdominal symptoms; however, the latter are outside their diagnostic features. These diseases include, first of all, tumor necrosis factor receptor-associated periodic syndrome (TRAPS). Interleukin 1 (IL1) inhibitors serve as the major targeted drugs for the treatment of TRAPS. Russia has registered the IL1 inhibitor canakinumab that prevents the development of organ damages, including those in the GI tract. The paper describes a clinical case of the classic manifestations of TRAPS (fever, rash, periorbital edema, arthritis, and elevated levels of acutephase inflammatory markers) concurrent with severe abdominalgia during attacks and with the development of severe peritoneal adhesions, which led to bowel perforation and emergency surgical intervention. The prolonged persistence of inflammatory attacks before the initiation of therapy, as well as violation of the IL1 inhibitor administration regimen facilitated the development of an urgent exacerbation. Thus, TRAPS should be included in the differential diagnostic circle for patients with severe gastrointestinal manifestations characterized by an attack-like course. These patients need timely prescription of targeted therapy, strict adherence to the dosing and intervals between drug administrations, and careful monitoring to prevent serious complications with the visceral organs, including the gastrointestinal tract, and their immediate correction.

scholarly journals Il-1antagonist Treatment in Recurrent Aseptic Meningitis Related to Familial Mediterranean Fever

2020 ◽  
Author(s):  
Ruth Livny ◽  
Yuval Bitterman ◽  
Riva Brik ◽  
Yonatan Butbul Aviel
Keyword(s):  
Nervous System ◽  
Familial Mediterranean Fever ◽  
Aseptic Meningitis ◽  
Inflammatory Diseases ◽  
Case Reports ◽  
Interleukin 1 ◽  
Colchicine Treatment ◽  
Cns Involvement ◽  
Mediterranean Fever ◽  
Recurrent Aseptic Meningitis

Abstract Background Familial Mediterranean fever (FMF) is an autosomal recessive, auto-inflammatory disease, presenting with recurrent bouts of fever and polyserositis. FMF has been associated with central nervous system (CNS) manifestations such as Headache and Myalgia. The occurrence of other forms of nervous system involvement is rare, including seizures, sinus vein thrombosis, pseudotumor cerebri and more. There are only few case reports of aseptic meningitis due to FMF. Case presentation We present the case of a 14 year-old girl diagnosed with FMF, who experienced recurrent episodes of severe headache and aseptic meningitis while on maximal dose of colchicine therapy. She had a dramatic response to anakinra with symptoms resolving completely within a few days without recurrence. Subsequently, we identified seven cases in the literature describing recurrent aseptic meningitis in patients with underlying FMF; all showed response to colchicine treatment, without treatment failure. Conclusion Our case suggests a role for Interleukin 1 (IL-1) antagonists for cases of CNS involvement secondary to FMF in patients who fail to respond to colchicine, and might imply that anakinra could be effective in other auto-inflammatory diseases with CNS involvement.

scholarly journals Familial Mediterranean fever (periodic disease): history or a real problem

2018 ◽  
Vol 12 (3) ◽  
pp. 61-69 ◽  
Author(s):  
E. S. Fedorov ◽  
S. O. Salugina
Keyword(s):  
Familial Mediterranean Fever ◽  
Inflammatory Diseases ◽  
Systemic Vasculitis ◽  
Interleukin 1 ◽  
Mefv Gene ◽  
Real Problem ◽  
Randomized Controlled Studies ◽  
Bowel Diseases ◽  
Mediterranean Fever ◽  
Definition Of

The review is devoted to the most common monogenic autoinflammatory disease – familial Mediterranean fever (FML) caused by MEFV gene mutation that occurs mainly in the representatives of certain ethnic groups and manifests itself as recurrent 6–72-hour attacks of pyretic fever accompanied by the phenomena of aseptic peritonitis, pleurisy, arthritis, and inflammatory rash. The disease can lead to a life-threatening complication, such as amyloidosis. FML is noted to be comorbid with a number of other inflammatory diseases: systemic vasculitis, chronic joint inflammatory diseases, and inflammatory bowel diseases. Emphasis is laid on the therapy aspects set out in the 2016 EULAR guidelines. The mainstay of treatment for FML is colchicine that prevents recurrences of the disease, minimizes the risk of amyloidosis, and should be prescribed immediately, once diagnosed. The paper deals with the definition of colchicine resistance that is observed in 5–10% of patients. Biological agents, among which interleukin-1 are most preferred, are now used to treat this category of patients. The high efficacy of these agents in patients with FML has been confirmed in randomized controlled studies.

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