scholarly journals Anti-interleukin-1 treatment among patients with familial Mediterranean fever resistant to colchicine treatment. Retrospective analysis

2019 ◽  
Vol 137 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Gokhan Sargin ◽  
Reyhan Kose ◽  
Taskin Senturk
2020 ◽  
Author(s):  
Ruth Livny ◽  
Yuval Bitterman ◽  
Riva Brik ◽  
Yonatan Butbul Aviel

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.


2020 ◽  
Vol 12 (1) ◽  
pp. e20200059
Author(s):  
Maria Grazia Massaro ◽  
Maurizio Pompili ◽  
Ludovico Luca Sicignano ◽  
Fabrizio Pizzolante ◽  
Elena Verrecchia ◽  
...  

A 44-year-old Jewish woman with familial Mediterranean fever (FMF) developed non-alcoholic steato-hepatitis during colchicine treatment (2,5 mg per day), confirmed by both elastographic study and liver biopsy. A combined therapy with the interleukin-1 (IL-1) blocking agent canakinumab (150 mg every 4 weeks) and colchicine (at a reduced dose of 1.5 mg per day) was started. Three months later transaminases became normal, and six months later there was a marked improvement of liver fibrosis on the elastographic study. Hepatic involvement in FMF occurs ranging from nonspecific increase in liver enzymes to cryptogenic cirrhosis. Liver is mostly involved in patients bearing the homozygous M694V MEFV mutation, as in our case. IL-1 blockade has the power to halt or mitigate liver involvement in FMF patients, though further experience is required to assess its therapeutic potential in the most severe patients with hepatic disease who are partially responsive to long-term colchicine.


2011 ◽  
Vol 41 (2) ◽  
pp. 265-271 ◽  
Author(s):  
Ulrich Meinzer ◽  
Pierre Quartier ◽  
Jean-François Alexandra ◽  
Véronique Hentgen ◽  
Frédérique Retornaz ◽  
...  

2021 ◽  
Vol 67 (4) ◽  
pp. 416-420
Author(s):  
Ayşe Aydemir Ekim ◽  
Fulya Bakılan ◽  
Emel Gönüllü ◽  
Hatice Hamarat

Objectives: This study aims to determine femoral cartilage thickness using ultrasonography in familial Mediterranean fever (FMF). Patients and methods: A total of 45 patients (16 males, 29 females; mean age: 38.5±9.1 years; range, 24 to 49 years) with the diagnosis of FMF and 31 healthy individuals (6 males, 25 females; mean age: 37.0±8.7 years; range, 25 to 47 years) between January 2016 and July 2016 were included in this study. Clinical data and demographic characteristics of the patients were recorded. All FMF cases in the study were in remission with colchicine treatment. The thickness of femoral cartilage in both knees were evaluated using ultrasonography. Three measurements (mid-point) were taken from both knees (at the medial/lateral femoral condyles and intercondylar area). Results: Ultrasonographical measurements revealed that cartilage measurements of FMF patients were significantly thinner at both the medial/lateral femoral condyles and intercondylar area on the right knee and at the medial/lateral femoral condyles on the left knee (p<0.001). The cartilage measurements in FMF patients were significantly thinner at the intercondylar area on left knee, compared to those in controls (p=0.023). Conclusion: Our study showed decreased femoral cartilage thickness in FMF patients. These findings indicate that even if these patients do not have an attack, they may have subacute and chronic arthritis in their joints, and their femoral cartilage thickness can be affected.


2020 ◽  
Vol 14 (4) ◽  
pp. 144-149
Author(s):  
S. O. Salugina ◽  
E. S. Fedorov ◽  
N. G. Volf

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.


2020 ◽  
Vol 50 (3) ◽  
pp. e10
Author(s):  
Ori Goldberg ◽  
Yoel Levinsky ◽  
Orit Peled ◽  
Gideon Koren ◽  
Liora Harel ◽  
...  

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