Articular manifestations in Egyptian children with familial Mediterranean fever

Abstract

Background Familial Mediterranean fever (FMF) is an autosomal recessive auto-inflammatory disease. Arthritis in early-onset FMF is a common finding. The aim of this study was to assess frequency of arthritis in 200 Egyptian children with FMF and also to detect its clinical characteristics, response to colchicine treatment, its effect on disease severity, and the most common MEFV gene mutations in patients with arthritis. Results We studied 200 children with FMF. We analyzed joint involvement in FMF attacks regarding its clinical characteristics, its effect on the disease severity, and response to colchicine treatment. We found arthritis in 20.5% of the studied population. Most of the children with arthritis had mono-articularjoint involvement during the FMF attack (73.1%), followed by oligo-articularjoint (22%). The knees and ankles were the most commonly affected joints. Arthritis was the presenting symptom in only 4%. We observed redness of the affected joints in 70.7%, and persistence of swelling after the attacks only in 17.1%. The majority of patients (85.4%) had a 75–100% decrease in the frequency of arthritic attacks after colchicine treatment. We did not find any joint disability in all studied patients, and arthritis was only present during attacks. The most frequent MEFV gene mutations in arthritic patients wereV726A and E148Q, each occurring in 28.6%. We observed an earlier age of disease onset and a more disease severity in patients with arthritis (p = 0.031 and p ≤ 0.001, respectively). We also observed that chest pain, erysipelas-like-erythema, and testicular affection were more observed in patients with arthritis (p values 0.001, 0.001, and 0.006, respectively). Conclusion This study showed that around 20% of Egyptian children with FMF can develop arthritis during the attacks, which usually runs a benign course. The presence of arthritis can denote a more severe disease course.

Musculoskeletal and neurological manifestations in a cohort of Egyptian Familial Mediterranean fever patients: genotype-phenotype correlation

“…The most common joint affection was the knee and ankles, the small joints of the feet, and the hands respectively. This goes in accordance with Farag et al [11] , Jargour and Dodaki [13] and Lidar M et al [14] Monoarthritis was the most frequent pattern of arthritis, being present in 26.9% (39 patients) of the studied patients. This goes in accordance with many other studies [11,14,15,36].…”

“…This goes in accordance with two other Egyptian and Turkish studies [30,34]. While Farag Y et al [11] and other two Egyptian studies [29,35] found lower frequency. On the other hand, a Turkish study [36] found a higher frequency of 57%.…”

“…This study involved 145 FMF patients (99 adult and 46 pediatric patients). The majority of the studied patients were female (62.1%) and this was in line with several studies from Egypt and Israel [11,25]. Two other studies that were performed on Italian and Arabs showed a similar result [26,27].…”

“…The presenting manifestation in 71.9% of these patients was abdominal pain followed by musculoskeletal manifestations in 35.2% in the form of arthralgia, myalgia, and arthritis, to be followed by fever in 30.4% of patients. This is the same finding of other Egyptian studies [11,29,30], while Duşunsel et al [31] reported that fever was the most common followed by abdominal pain in a cohort of Turkish FMF patients. It seems that the ethnicity of studied patients affects the clinical picture of the attacks.…”

“…However, in about 5% of the patients, protracted arthritis develops, almost involving the hips or knees. Although complete recovery is the rule, disabling joint damage can occur and even may lead to joint replacement [11][12][13][14][15][16]. Myalgia is also a common manifestation of FMF and occurs in about 20% of patients.…”