Familial Mediterranean fever: perspective on female fertility and disease course in pregnancy from a multicenter nationwide network.

The aim of this study was to analyze the pregnancy process, especially the Familial Mediterranean fever (FMF) disease course and attack types during pregnancy, and to examine the relationship between disease-related factors and female infertility in FMF patients. The study, which was planned in a multicenter national network, included 643 female patients. 435 female patients who had regular sexual intercourse were questioned in terms of infertility. Pregnancy and delivery history, FMF disease severity and course during pregnancy were evaluated. The relationship between demographic and clinical findings, disease severity, genetic analysis results and infertility was investigated. 401 patients had at least 1 pregnancy and 34 patients were diagnosed with infertility. 154 patients had an attack during pregnancy. 61.6% of them reported that attacks during pregnancy were similar to those when they were not pregnant. The most common attack symptoms were fever, fatigue and abdominal pain-peritonitis (96%, 87%, and 83%, respectively) in the pregnancy period. The disease-onset age, disease activity score, gene mutation analyses, and regular colchicine use (> 90%) were similar between the fertile and infertile groups, while the frequency of previous appendectomy and alcohol consumption rates were higher in individuals with infertility. Our results indicated no significant change in the frequency and severity of attacks during pregnancy. The low rate of infertility (7.8%) in our patients was noted. It has been suggested that the risk of FMF-related infertility may not be as high as thought in patients who are followed up regularly and received colchicine.

Evaluation of hepatitis serology and frequency of viral reactivation in patients with inflammatory arthritis receiving biologic agents: a multicenter observational study.

To evaluate of hepatitis serology and reactivation frequency in patients with rheumatic disease receiving biologic agents. Our study included patients with inflammatory rheumatic diseases from 23 centers, who were followed up with biological therapy. Demographic and clinical characteristics of the patients, duration of drug use and hepatitis serology and the state of viral reactivation were analyzed. A total of 4060 patients, 2095 being males, were included in our study. Of the patients, 2463 had Ankylosing Spondylitis (AS), 1154 had Rheumatoid Arthritis (RA), 325 had Psoriatic Arthritis (PsA), and 118 had other inflammatory rheumatic diseases. When the viral serology of the patients was evaluated, 79 patients (2%) who were identified as HBs Ag positive, 486 (12%) patients who were HBs Ag negative and anti-HBc IgG positive and 20 patients (0.5%) who were anti-HCV positive. When evaluated on a disease-by-disease basis, the rate of HBsAg was found to be 2.5% in RA, 2% in AS and 0.9% in PsA. Viral reactivation was detected in 13 patients while receiving biologic agents. HBs Ag was positive in nine patients with reactivation and negative in four patients. Anti-HBc IgG, however, was positive. Six of these patients had AS, four had RA, and three had PsA. The development of hepatitis reactivation in 11.4% of HBs Ag positive patients and 0.82% of anti-HBc IgG positive patients due to the use of biologic agents is an important problem for this group of patients. Antiviral prophylaxis is recommended to be started especially in patients who are HBs Ag positive and who are using biologic agents due to viral reactivation. Therefore, it is important to carry out hepatitis screenings before biologic agent treatment and to carefully evaluate the vaccination and prophylaxis requirements.

Familial Mediterranean fever: Health-related quality of life and associated variables in a national cohort.

OBJECTIVES: This study aims to evaluate the effectivity of Familial Mediterranean Fever Quality of Life (FMF-QoL) Scale for the measurement of QoL in patients with FMF and to perform correlations between related clinical variables in Turkish patients. PATIENTS AND METHODS: This multicenter prospective study performed between December 2017 and November 2018 included 974 FMF patients (334 males, 640 females; median age: 35; range, 26 to 45 years). Sociodemographic characteristics and clinical features were recorded. All participants were asked to complete the FMF-QoL Scale, Short Form-36 (SF-36), Hospital Anxiety and Depression Scale (HADS), Health Assessment Questionnaire (HAQ), and Functional Assessment of Chronic Illness Therapy (FACIT) Scale. RESULTS: The median FMF-QoL Scale score was 26. Higher FMF-QoL Scale scores were shown to be related to female sex, illiteracy or primary education, monthly low-income (US$<300), smoking, late-onset FMF (>20 years), a higher number of attacks per month (>1/month), and severe disease. FMF-QoL Scale scores were correlated negatively with subscales of SF-36, and positively with HADS-anxiety and HADS-depression scores, HAQ and FACIT. CONCLUSION: Female sex, smoking, lower educational status, more severe disease, fatigue, and functional impairment were associated with poor QoL. FMF-QoL Scale was noted as a valid and simple patient-reported outcome instrument and correlated with the SF-36 scale.

The impact of COVID-19 on familial Mediterranean fever: a nationwide study.

The study aimed to evaluate the impact of the coronavirus disease 2019 (COVID-19) in patients with familial Mediterranean fever (FMF) and to assess the relationships between FMF characteristics and severe COVID-19 outcomes such as hospitalization. The study was planned within a national network of 21 different centers. Demographics, FMF-related clinical and genetic characteristics, and COVID-19 outcomes were obtained. A total of 822 patients with FMF (mean age of 36 years) were included in the study. Fifty-nine of them (7%) had a COVID-19 diagnosis confirmed by real-time PCR test or chest CT findings. Most FMF patients with COVID-19 (58) had mild and moderate disease activity. All patients were on colchicine treatment. However, 8 of them (13.6%) were not compliant with colchicine use and 9 of them (15.3%) were colchicine resistant. Twelve FMF patients with COVID-19 were hospitalized. There were 4 patients requiring oxygen support. COVID-19 related complications were observed in 2 patients (1 thromboembolism, 1 acute respiratory distress syndrome). Hospitalized COVID-19 patients with FMF were older than non-hospitalized patients (median ages: 51 and 31 years, respectively; p: 0.002). Other FMF-related characteristics were similar between the groups. FMF-related characteristics were not found to be associated with poor outcomes in COVID-19. Thus, FMF may not be a risk factor for poor COVID-19 outcomes.

Association Between Familial Mediterranean Fever and Cachexia in Females.

OBJECTIVES: This study aims to investigate the association between familial Mediterranean fever (FMF) and cachexia in females. PATIENTS AND METHODS: The study included 32 female FMF patients (median age 27.50 years; range, 18 to 50 years) and 30 female healthy controls (median age 32 years; range, 18 to 50 years). Patients were classified according to Tel-Hashomer criteria. Circumference of arm, waist, and thigh was recorded. Short form 36 (SF-36) and Multidimensional Assessment of Fatigue (MAF) scale were applied. Composition of the body was measured with dual X-ray absorption. Muscle strength was measured with an isokinetic dynamometer, and strength of hand grip was measured from dominant hand with a hand dynamometer. C-reactive protein, erythrocyte sedimentation rate, fibrinogen and serum creatinine kinase (CK) levels were recorded. RESULTS: Body mass index was significantly higher in controls. Twelve patients and one control had cachexia. CK level was significantly higher in patients than controls. Mass of muscle without fat was significantly higher in patients than controls. Peak torque values of extension and flexion at the velocity of 60°/second [Newton meter (Nm)], and value of total work during extension at the velocity of 240°/second (Nm) in isokinetic measures were significantly higher in controls. MAF score was significantly higher in patients with cachexia than patients without cachexia where the subscale scores of SF-36, except the vitality score, were significantly lower in patients with cachexia. However, Tel-Hashomer score was significantly higher in patients with cachexia. CONCLUSION: This study pointed at a significant association between cachexia and FMF in females. Muscle endurance was not affected in FMF patients with cachexia; however, decreased muscle strength, impaired quality of life and increased fatigue were observed in these patients.

Familial mediterranean fever: assessment of clinical manifestations, pregnancy, genetic mutational analyses, and disease severity in a national cohort.

The aims of this study were to investigate the main clinical and laboratory features, including pregnancy and genetic analysis, of Turkish Familial Mediterranean Fever (FMF) patients and to analyze the relationships between genotypic features, age of disease onset, clinical findings, and disease severity. A study was planned within a national network of 22 different centers. Demographics, clinical and laboratory findings, attack characteristics, drugs, pregnancy and birth history, disease severity, and gene mutation analyses were evaluated. Disease severity, assessed using a scoring system developed by Pras et al., was evaluated in relation to gene mutations and age of disease onset. A total of 979 patients (643 females and 336 males; mean age: 35.92 ± 11.97 years) with FMF were included in the study. Of a total of 585 pregnancies, 7% of them resulted in preterm birth and 18.1% resulted in abortions. During pregnancy, there was no FMF attack in 61.4% of patients. Of the MEditerranean FeVer (MEFV) mutations, 150 (24.3%) cases were homozygous, 292 (47.3%) cases were heterozygous, and 175 (28.4%) were compound heterozygous. Patients with homozygous gene mutations had more severe disease activity, earlier age of disease onset, higher rates of joint and skin involvement, sacroiliitis, and amyloidosis. Patients with compound heterozygous genotype displayed severe disease activity in close resemblance to patients with homozygous mutation. In addition, patients with compound heterozygous mutations had higher rates of protracted febrile myalgia and elevated fibrinogen levels. In 63.9% of compound heterozygous patients, age of onset was < 20 years, with greater disease severity, and high rates of attack frequency and colchicine resistance. Our results suggest that indicators for disease severity include early onset of disease and homozygous gene mutations. Furthermore, patients with compound heterozygous mutations displayed significant presentations of severe disease activity.

Ultrasound-defined remission for good functional status in rheumatoid arthritis.

BACKGROUND & OBJECTIVES: It has been shown that joint damage due to subclinical synovitis progresses despite apparent clinical remission in rheumatoid arthritis (RA). Hence, finding more objective methods to investigate subclinical synovitis has become a current issue. Ultrasonography (US) has been among the most investigated methods. This study was conducted to detect whether there was subclinical inflammation in RA patients in clinical remission by power Doppler ultrasonography (PDUS) and to evaluate the effects of this inflammation on upper extremity function. METHODS: Forty five RA patients fulfilled the remission criteria of disease activity score 28 using erythrocyte sedimentation rate (DAS28-ESR), were enrolled in the study. Bilateral wrist, 2nd and 3th metacarpophalangeal and proximal interphalangeal joints and 2nd and 5th metatarsophalangeal joints were examined by PDUS. Upper extremity function was assessed with Michigan Hand Outcomes Questionnaire (MHQ) and handgrip strength. The pain was evaluated by visual analogue scale (VAS). RESULTS: In 29 of 45 RA patients in clinical remission, synovitis was detected by PDUS at least in one joint. VAS and DAS28-ESR scores were significantly lower and total MHQ, some subgroup scores of MHQ (overall hand function, activity of daily living and work performance) and grip strength of the dominant hand were higher in patients with PD signal negativity. INTERPRETATION & CONCLUSIONS: PDUS showed a crucial role in determining the subclinical synovitis. Subclinical synovitis negatively affects the upper extremity function. Ultrasound-defined remission may be considered for good functional status and real remission in patients with RA.

Ovarian reserve is preserved in Behçet's disease.

AIM: To compare ovarian reserve with anti-müllerian hormone (AMH) levels, antral follicle counts (AFCs) and ovarian volume in patients with Behçet's disease (BD) and healthy subjects. METHOD: This study included 35 women with BD (mean age: 34.4 ± 5.3 years) and 35 healthy controls (mean age: 34.1 ± 6.0 years). Venous blood samples collected from groups on menstrual cycle Days 2-4 were analyzed using the enzyme-linked immunosorbent assay to measure follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2 ), prolactin (PRL) and AMH levels. The AFCs and ovarian volumes were estimated by ultrasonography on the same day. The body mass index (BMI), duration and severity of the disease, medications, and number of children were recorded. RESULTS: There was no statistically significant difference in the mean age, median gravida/para/abortus and the number of live births and mean BMI between the patient and control groups (P > 0.05). No statistically significant difference in the median FSH, LH, E2, PRL levels, right and left ovarian volumes, and right and left ovarian AFCs was observed among the patients and the controls (P > 0.05). There was no statistically significant difference in the mean AMH levels (P = 0.468). Bonferroni correction showed no statistically significant correlation between AMH levels and age, BMI, FSH, LH, E2, PRL levels, right and left ovarian AFCs, right and left ovarian volume (P > 0.025) among the controls and the patients. CONCLUSION: Our study results suggest that ovarian reserve is preserved in patients with BD. In addition, AMH levels of the BD patients were similar to levels of healthy subjects.

Evaluation of Ovarian Reserve with Anti-Müllerian Hormone in Familial Mediterranean Fever.

Objective. To investigate ovarian reserves in attack-free familial Mediterranean fever (AF-FMF) patients at the reproductive age by anti-Müllerian hormone (AMH), antral follicle count (AFC), ovarian volume, and hormonal parameters. Methods. Thirty-three AF-FMF patients aging 18-45 years and 34 healthy women were enrolled and FSH, LH, E2, PRL, and AMH levels were measured in the morning blood samples at 2nd-4th days of menstruation by ELISA. Concomitant pelvic ultrasonography was performed to calculate AFC and ovarian volumes. Results. In FMF patient group, median AMH levels were statistically significantly lower in the M69V mutation positive group than in the negative ones (P = 0.018). There was no statistically significant difference in median AMH levels between E148Q mutation positive patients and the negative ones (P = 0.920). There was also no statistically significant difference in median AMH levels between M680I mutation positive patients and the negative ones (P = 0.868). No statistically significant difference was observed in median AMH levels between patients who had at least one mutation and those with no mutations (P = 0.868). We realized that there was no difference in comparisons between ovarian volumes, number of follicles, and AMH levels ovarian reserves when compared with FMF patients and healthy individuals. Conclusions. Ovarian reserves of FMF pateints were similar to those of healthy subjects according to AMH. However, AMH levels were lower in FMF patients with M694V mutation.