The factors considered as trigger for the attacks in patients with familial Mediterranean fever

Abstract

Although the inflammatory cascade of familial Mediterranean fever (FMF) is partially understood, triggering factors of those attacks has not been studied well. It is supposed that physical stresses such as cold exposure, tiredness and emotional stresses could provoke attacks. This study is aimed to survey the factors regarded as triggering the attacks in patients with FMF and their relationship with MEFV gene mutations. Clinical findings and genetic mutations (consist of M694V, M694I, M680I, V726A, E148Q) of patients were recorded. Patients were questioned about cold exposure, emotional stress, tiredness, long-lasting standing, long-duration travel, starvation, high intake of food, trauma, and infection as triggering factors for the attacks with both serositis and musculoskeletal pain. The study is comprised of 275 FMF patients (male/female: 177/98). The most common triggering factors for the attacks with serositis were cold exposure (59.3 %), emotional stress (49.8 %), tiredness (40.0 %) and menstruation (33.7 % in females). Long-lasting standing (78.8 %), long-duration travel (64.1 %) and tiredness (47.8 %) were the triggering factors for the attacks with musculoskeletal symptoms. The relationships between MEFV mutations and triggering factors were found as M694V allele with starvation, E148Q allele with high intake of food and V726A allele with long-duration travel. The attacks with serositis seem to be triggered by those factors to which whole body exposed, whereas the attacks with musculoskeletal complaints seem to be triggered by those factors to which regional or local part of body exposed. Since the number of alleles was small, a clear conclusion for a relationship between a particular gene variant and a specific trigger was not made.

EULAR recommendations for the management of familial Mediterranean fever

“…Recognised trigger factors include physical or emotional stress, menstruation, physical trauma, exposure to cold, infections, inflammation, high fat intake, starvation, sleeplessness and tiredness 47. These triggers may differ depending on the type of attack 48 49. Some authors recommend increasing the dose of colchicine to pre-empt trigger events 50 51…”

Toll‐like receptor 4 promoter polymorphisms in a familial mediterranean fever patient with asymptomatic bacteriuria

“…The relationship between the menstrual period and FMF attacks is controversial . Takano et al confirmed changes in hydrogen and potassium excretion in urine during the menstrual cycle .…”

The Diagnosis of Familial Mediterranean Fever in a Child Presenting with Trauma Following Acute Abdomen

“…Şimdiye kadar atakları tetikleyen birçok faktör tanımlanmıştır. Ülkemizde bu konu ile ilgili bir çalışmada AAA hastalarında serozit ataklarını tetikleyen en sık etkenin soğukla karşılaşma (%59,9) olduğu, psikolojik stresin (%49,8) ve fiziksel yorgunluğun (%40) bunu izlediği, en nadir sebebin ise travma (%1,5) olduğu saptanmıştır (4). Olgumuzda da travma, ve/veya bunun sebep olduğu psikolojik stresin AAA atağına sebep olabildiği düşünülmüştür.…”