Abstract

Background:Fatigue is an important and common symptom in rheumatologic diseases. It causes disability and worsens patients quality of life. Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever and serositis. Mutations in the MEFV gene that encodes pyrin protein are responsible for the disease. Most frequent mutation is M694V and FMF patients with M694V/M694V genotype have more severe disease.Objectives:The aim of this study is to investigate fatigue and its impact on quality of life of FMF patients who are attack-free for more than one year.Methods:Seventy-seven FMF patients and 70 age and sex matched healthy controls were enrolled in the study. Fatigue severity scale (FSS) was used to evaluate fatigue level. Disease severity was evaluated via FMF disease severity score. Short form-36 (SF-36) was used to evaluate the quality of life of the participants and Beck depression inventory (BDI) was used to evaluate depression.Results:No statistically significant difference was found when FMF group and healthy controls were compared for demographic variables and laboratory markers (complete blood count, erythrocyte sedimentation rate, c-reactive protein levels, liver and kidney function tests). All of the subscale scores of SF-36 were lower in FMF group when compared with the control group. BDI and FSS scores were higher in the FMF group.(Table 1)Table 1.Comparison of FMF and control groups for fatigue, depression and quality of lifeFMF group (n: 77)median(min-max)Control group (n: 70)Median (min-max)pFatigue severity scale (FSS)31(27-47)28 (24-37)p<0.05Beck depression inventory (BDI)15 (0-35)12 (0-19)p<0.05SF-36 Physical functioning80.00(25.00-100.00)95.00 (50.00-100.00)p<0.01SF-36 Social functioning55.50 (40.00-88.80)77.70 (22.20-88.80)p<0.01SF-36 Role physical25.00 (0.00-100.00)100.00 (0.00-100.00)p<0.01SF-36 Role emotional33.30 (0.00-100.00)100.00 (0.00-100.00)p<0.01SF-36 Mental health56.00 (24.00-88.00)72.00 (24.00-100.00)p<0.01SF-36 Pain55.50 (0.00-100.00)77.70 (33.30-100.00)p<0.01SF-36 Vitality45.00 (15.00-95.00)65.00 (5.00-100.00)p<0.01Sf-36 General health40.00 (5.00-92.00)67.00 (20.00-100.00)p<0.01Twenty-three of FMF patients had M694V/M694V genotype and those patients had higher disease severity scores (p<0.01), higher FSS (p<0.01) and higher BDI scores (p<0.05) when compared with other FMF patients. Regarding the quality of life, patients with M694V/M694V genotype had lower scores in social functioning, role physical, role emotional and pain subscales of SF-36. In the correlation analysis depression and fatique were found to be the major determinants of quality of life in FMF. Disease severity or duration were not strongly correlated with the SF-36 scores (Table 2).Table 2.Correlation of fatigue, depression, disease severity and duration with quality of life in FMF patientsPhysical functioningSocial functioningRole PhysicalRole emotionalMental healthVitalityPainGeneral healthFMF disease severity scaler= -0,223r= -0,319**r= -0,313**r= -0,184r= -0,124r= -0,188r= -0,191r= -0,351**Beck Depression Inventoryr= -0,418**r= -0,554**r= -0,461**r= -0,470**r= -0,660**r= -0,594**r= -0,308**r= -0,576**Fatigue severity scaler= -0,391**r= -0,426**r= -0,475**r= -0,324**r= -0,650**r= -0,739**r= -0,410**r= -0,521**Disease durationr= -0,40**r= -0,218r= -0,232*r= -0,287*r= -0,026r= -0,211r= -0,189r= -0,109** p<0.01*P<0.05Conclusion:Despite being attack-free for more than one year, FMF patients had poor quality of life and fatigue when compared with the healthy controls. The quality of life in FMF patients, whose attacks are well controlled, is mainly determined by fatigue and depression rather than disease severity.

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