Do Pediatric Patients with Familial Mediterranean Fever who have Phenotypically Predominant Arthritis, Arthralgia, and Myalgia Reflect a More Serious Underlying Disease than Those with Phenotypically Predominant Abdominal Pain?

Öz

Aim: The study aimed to determine the presence of different demographic, familial, clinical, laboratory, and genotypic characteristics between children with Familial Mediterranean Fever (FMF) with recurrent arthritis, arthralgia, and myalgia phenotype and children with FMF with recurrent abdominal attacks phenotype. Material and Method: The study included patients who were admitted to the Pediatric Rheumatology Outpatient Clinic of the Kafkas University Research Hospital before 2020, who were diagnosed with FMF and followed up for at least 1 year, and who were diagnosed with clinically recurrent arthritis, arthralgia, myalgia, and recurrent abdominal pain. Patients were divided into two groups: 151 patients with recurrent arthritis, arthralgia, and myalgia with FMF phenotypic features (Group 1) and 102 patients with recurrent diffuse, incomplete peritoneal involvement with predominant FMF phenotypic features (Group 2). The demographic and familial characteristics, age at diagnosis, and other clinical, laboratory, and genetic features of these 2 groups were compared, and the differences were statistically evaluated. Results: While 60.6% of the patients in Group 1 and 39.4% in Group 2 were female, 58.7% of the patients in Group 1 and 41.3% in Group 2 were male. No significant statistical difference was found between the two groups. When the groups were compared regarding genetic mutations, M694V homozygotes were observed in 17.2% of Group 1 and 8.8% of Group 2. There was a statistically significant difference between Group 1 and Group 2 in M694V homozygotes (p<0.05) Conclusion: Patients with FMF with predominant recurrent arthritis, arthralgia, and myalgia tended to be diagnosed at an older age and exhibited more frequent symptoms such as pallor and nausea compared to patients with FMF who predominantly had peritoneal involvement. Furthermore, pleural involvement was frequently observed, and the co-occurrence with the homozygous M694V mutation was genotypically high.

Anahtar Kelimeler

• familial Mediterranean fever
• arthritis
• arthralgia
• myalgia; involvement

Kaynakça

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