Is age associated with disease severity and compliance to treatment in children with familial Mediterranean fever?

Sönmez, Hafize Emine; Esmeray, Pelin; Batu, Ezgi Deniz; Arıcı, Zehra Serap; Sag, Erdal; Özen, Seza; Bilginer, Yelda

doi:10.1007/s00296-018-4123-0

Cited by 26 publications

(22 citation statements)

References 15 publications

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“…As an example, early onset disease has a more severe course when compared with the late-onset disease. 8 But when we compared the ages of onset of disease in two groups there was not any difference, as the ages at diagnosis were also similar.…”

Section: Discussionmentioning

confidence: 92%

Gender is not a Prognostic Factor for Familial Mediterranean Fever

Bilge¹,

Bodakçı²,

Bilge³

et al. 2019

Ankara Medical Journal

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Objectives: Familial Mediterranean fever (FMF) is the most common hereditary auto-inflammatory disease. Our aim in the current study was to evaluate the relation between gender and clinical findings, the severity of disease and treatment compliance in FMF patients. Materials and Methods: Medical records of the 150 FMF patients were retrospectively evaluated. Disease severity scores were evaluated with the international severity score system for familial Mediterranean fever (ISSF), treatment compliance of the patients was evaluated with six-item modified Morisky scale and compared between males and females. Results: Of the 150 patients, 87 (58 %) were female and 63 (42 %) were male. Erysipelas like erythema (ELE) was more common in females (51.70% vs 30.90%, p= 0.03) and myalgia were more common in males (31.03% vs 36.50%, p=0.015). Other clinical findings were at similar frequencies. The ISSF scores of the groups were similar (2.93±2.05 vs 3.12±2.21, p= 0.716). The motivation for treatment and knowledge levels were the same in both groups (p=0.228 and p=0.112, respectively). Conclusion: Clinical findings, ISSF scores and treatment compliance of the FMF patients in both sexes are similar. The gender of the patient does not affect the clinical course of disease in FMF patients.

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Section: Discussionmentioning

confidence: 92%

Gender is not a Prognostic Factor for Familial Mediterranean Fever

Bilge¹,

Bodakçı²,

Bilge³

et al. 2019

Ankara Medical Journal

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“…The higher rates among pediatric patients may be related to administering the drugs by their parents. In another study evaluating the adherence rate among pediatric patients with FMF, the adherence rate was higher in younger patients (18). Corsia et al reported the adherence rate for colchicine treatment as 40% in the study group, whereas, in terms of adult and childhood patients, the adherence rates were 22% and 48%, respectively.…”

Section: Discussionmentioning

confidence: 93%

“…This situation may be associated with the assistance of the partner to take the pills regularly or due to increased sense of responsibility in married patients. Similarly, due to the administration of the drug by parents, the adherence rate was reported as 90.5% among patients under five years old (18). Reminding (a partner, a parent, a phone alarm, or an application, vs.) may increase the adherence.…”

Section: Discussionmentioning

confidence: 99%

Non-adherence to colchicine treatment is a common misevaluation in familial Mediterranean fever

Tekgöz¹,

Çolak²,

Çınar³

et al. 2021

Turk J Med Sci

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Background: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease that requires lifelong colchicine treatment. Colchicine is the mainstay of the treatment which decreases the frequency and the severity of recurrent FMF attacks, also, prevents the development of amyloidosis. This study aimed to investigate the rates of colchicine treatment adherence in patients with FMF and the factors related to treatment non-adherence. Materials and methods: This observational study was conducted with 179 patients with FMF between November 2018 and April 2019 in a tertiary rheumatology outpatient clinic. The sociodemographic and clinical data were recorded. Compliance Questionnaire on Rheumatology (CQR) was used to assess the treatment adherence and the Beliefs About Medicines Questionnaire (BMQ-T) was used to assess a patient's beliefs about colchicine. The factors associated with adherence to the treatment were evaluated. Results:The study included 113 male (63.1%) and 66 (36.9%) female patients with a mean age of 30 (25-44) years. The rate of the patients who declared regular colchicine usage was 66.5%. The frequency of non-adherent patients was 83.8% according to CQR. Treatment adherence was better in patients with comorbid diseases than those without (41.4% vs. 22%, respectively, p = 0.028). The frequency of married patients in the adherent group (72.4%) was higher than the non-adherent group (47.3%) (p = 0.013). The colchicine dose used in the adherent group was 1.5 (1.3-1.8) mg/day, whereas it was 1.5 (1.0-1.5) mg/day in the non-adherent group (p = 0.033). The 2 adherence rate was rising with increasing scores of BMQ-T Specific Necessity. As the scores of BMQ-T General Overuse and General Harm increased, non-adherence to colchicine increased. Conclusion:Evaluating adherence to colchicine treatment with objective methods is crucial to ensure sufficient treatment and prevent amyloidosis. Determining beliefs about colchicine may increase patients' adherence to treatment.

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“…Patients older than 12 years at the onset, showed a reduced frequency of fever attacks than the younger patients. Patients younger than 5 years at the onset showed more severe attacks [5].…”

Section: Clinical Presentation Of Fmfmentioning

confidence: 92%

FMF is not always “fever”: from clinical presentation to “treat to target”

Maggio

Corsello

2020

Ital J Pediatr

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Familial Mediterranean Fever, a monogenic autoinflammatory disease secondary to MEFV gene mutations in the chromosome 16p13, is characterized by recurrent self-limiting attacks of fever, arthritis, aphthous changes in lips and/or oral mucosa, erythema, serositis. It is caused by dysregulation of the inflammasome, a complex intracellular multiprotein structure, commanding the overproduction of interleukin 1. Familial Mediterranean Fever can be associated with other multifactorial autoinflammatory diseases, as vasculitis and Behçet disease. Symptoms frequently start before 20 years of age and are characterized by a more severe phenotype in patients who begin earlier. Attacks consist of fever, serositis, arthritis and high levels of inflammatory reactants: C-reactive protein, erythrocyte sedimentation rate, serum amyloid A associated with leucocytosis and neutrophilia. The symptom-free intervals are of different length. The attacks of Familial Mediterranean Fever can have a trigger, as infections, stress, menses, exposure to cold, fatrich food, drugs. The diagnosis needs a clinical definition of the disease and a genetic confirmation. An accurate differential diagnosis is mandatory to exclude infective agents, autoimmune diseases, etc. In many patients there is no genetic confirmation of the disease; furthermore, some subjects with the relieve of MEFV mutations, show a phenotype not in line with the diagnosis of Familial Mediterranean Fever. For these reasons, diagnostic criteria were developed, as Tel Hashomer Hospital criteria, the "Turkish FMF Paediatric criteria", the "clinical classification criteria for autoinflammatory periodic fevers" formulated by PRINTO. The goals of the treatment are: prevention of attacks recurrence, normalization of inflammatory markers, control of subclinical inflammation in attacks-free intervals and prevention of medium and long-term complications, as amyloidosis. Colchicine is the first step in the treatment; biological drugs are effective in non-responder patients. The goal of this paper is to give a wide and broad review to general paediatricians on Familial Mediterranean Fever, with the relative diagnostic, clinical and therapeutic aspects.

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Is age associated with disease severity and compliance to treatment in children with familial Mediterranean fever?

Cited by 26 publications

References 15 publications

Gender is not a Prognostic Factor for Familial Mediterranean Fever

Gender is not a Prognostic Factor for Familial Mediterranean Fever

Non-adherence to colchicine treatment is a common misevaluation in familial Mediterranean fever

FMF is not always “fever”: from clinical presentation to “treat to target”

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