Gender differences in clinical features and burden in patients with familial mediterranean fever: Preliminary report

Abstract

Background: Familial Mediterranean fever (FMF) is the most common hereditary auto-inflammatory disease, which is more common in groups in the Mediterranean basin, characterized by recurrent attacks of febrile peritonitis, pleuritis and arthritis.

Objectives: The aim of this study is to investigate the gender differences in clinical and demographic features in patients with familial Mediterranean fever (FMF).

Methods: Patients with FMF diagnosed according to Tel-Hashomer criteria were included in this multi-center study. Data about age, sex, disease duration, symptom duration, age at diagnosis, comorbid diseases and medications were noted. All patients were screen with Hospital Anxiety and Depression Scale (HADS), Health Assessment Questionnaires (HAQ), Short Form 36 (SF-36). The severity of FMF was assessed by the PRASS scoring system.

Results: Of the 286 patients, 199 (69,6%) were female with the mean age 36,2(SD:12.32) years and 87 (30,4%) were male with the mean age 32,9(SD:12,6) years (p=0,017). Age of diagnosis in male patients was lower than in females (p=0,008). The most common genetic mutation in both genders was M694V gene mutation. Homozygous M694V positivity was higher in males (31,79% vs 17,4%). More than 90% of patients were receiving colchicine,10 (3,5%) patients were receiving anakinra and 5(1,75%) patients were receiving canakinumab. Among the patients regularly taking colchicine, 86.2% were females and 86% were males (p=0,981). There were no differences between male and females in terms of clinical findings, frequency and duration of attacks, presence of colchicine resistance, and the presence of amyloidosis (p>0.05). ESR was detected higher in female group (p=0,011). Headache (p=0,001), migraine (p=0,031), and concomitant diseases (p=0,017) were also more common in females than males. Anxiety and depression scores were higher in females (p<0,05). The PRASS activity scores were similar between genders (p>0,05). All parameters of SF-36 were higher in male patients, while HAQ score was higher in females (p<0,05).

Conclusion: Although clinical findings, disease severity and treatment compliance are similar in both genders, males are diagnosed with FMF earlier than females, but findings such as anxiety, depression, migraine, and headache are more common in female patients.