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The article focuses on a research which aims to investigate the prevalence of systemic lupus erythematosus (SLE) gene mutation among familial Mediterranean fever (FMF) gene patients. It states that none of SLE patients fulfilled revised Tel-Hashomer criteria. It mentions that the carrier rate for MEF was slightly lower in SLE group.

Introduction: Exertional leg pain (ELP) and enthesitis are musculoskeletal findings in familial Mediterranean fever (FMF). They are not accepted as principal treatment targets. In this study, we assessed the effectiveness of treatments on ELP and enthesitis., Material and Methods: We have included 218 FMF patients to the study. We retrospectively compared the FMF attacks' frequency, duration and intensity (FMF attack VAS score) and levels of ELP VAS and enthesitis VAS scores between pre-treatment stage and while patients were on treatment at the last visit., Results: Forty-nine (22.5%) and 52 (23.9%) of the patients had enthesitis and ELP respectively. All patients were on colchicine treatment. Serositis attacks respond the treatments significantly. Moreover, both ELP VAS scores (p=0.002) and enthesis VAS scores (p=0.17) were improved with treatment. But only improvement in ELP VAS scores was significant., Conclusion: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Introduction: Exertional leg pain (ELP) and enthesitis are musculoskeletal findings in familial Mediterranean fever (FMF). They are not accepted as principal treatment targets. In this study, we assessed the effectiveness of treatments on ELP and enthesitis., Material and Methods: We have included 218 FMF patients to the study. We retrospectively compared the FMF attacks' frequency, duration and intensity (FMF attack VAS score) and levels of ELP VAS and enthesitis VAS scores between pre-treatment stage and while patients were on treatment at the last visit., Results: Forty-nine (22.5%) and 52 (23.9%) of the patients had enthesitis and ELP respectively. All patients were on colchicine treatment. Serositis attacks respond the treatments significantly. Moreover, both ELP VAS scores (p=0.002) and enthesis VAS scores (p=0.17) were improved with treatment. But only improvement in ELP VAS scores was significant., Conclusion: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Objective: Familial Mediterranean fever (FMF) is an auto-inflammatory disease that is also characterized with some of the common musculoskeletal features of spondyloarthritis (SpA). Enthesitis is the hallmark of SpA. Recently, it was postulated that exertional leg pain is a possible sign of lower extremity enthesitis associated with FMF severity. In this study, we have evaluated the association between the enthesitis, enthesitis score and disease severity in FMF patients., Methods: We enrolled 238 FMF patients that fulfilled the modified Tel-Hashomer criteria. We assessed the presence of enthesitis at the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES) defined sites with standard palpation method. Then, FMF patients dichotomised two groups as enthesitis group and controls. Herein, we evaluated the enthesis extensity with MASES. FMF disease severity was determined via the international severity scoring system for FMF (ISSF). Firstly, we have compared demographic properties, disease-related features and ISSF scores of the groups. Then, we have correlated ISSF with MASES in enthesitis group., Results: We showed that 54 (22.6%) of 238 patients had enthesitis. The demographic features were similar between the groups. The enthesitis group had higher ISSF scores (p < 0.001); higher frequency of fever (p = 0.004), exertional leg pain (p < 0.001), myalgia (p < 0.001) and arthritis (p = 0.01); and more intense, widespread, frequent and longer attacks compared with controls. Moreover, there was a weak correlation between ISSF and MASES in the patients with enthesitis., Conclusion: Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other musculoskeletal manifestations resemble SpA. Key points •More than one-fifth of the patients with FMF would suffer from enthesitis. •The FMF patients with enthesitis had higher ISSF scores; higher frequency of fever, exertional leg pain, myalgia and arthritis; and more intense, widespread, frequent and longer attacks as compared with controls. •Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other SpA-like musculoskeletal feature.

Objective: To determine predictors and optimal duration of sustained remission (SR) in patients with rheumatoid arthritis (RA)., Methods: A total of 428 consecutive patients with RA visiting our clinic routinely between 2012 and 2013 were evaluated. Seventy seven of these patients in DAS28 remission were enrolled and followed up for 62.2 ± 9.9 months. Patients in remission ≥ 6 months (SR) and shorter (non: N-SR) were compared in terms of demographic-clinical data and the psychosocial factors. At enrollment, 1st and 5th years, patients in DAS28, SDAI, and Boolean remission were determined., Results: Sixty three patients were in SR and 14 in N-SR. Lower baseline DAS28 and HAQ scores, anti-CCP were positive predictors of SR. Although the presence of anxiety, depression, fibromyalgia, and fatigue were lower in the SR group, there was no significance. Patients in DAS28 remission (100%) at baseline reduced to 64% at 1st and 42.6% at 5th years. Patients satisfying SDAI and Boolean remission at these three visits were 49%, 44%, and 32.4% vs 41%, 28%, and 20.6%, respectively. If the duration of remission is defined as 6 months, the remission rates of SDAI at inclusion and fifth years' visits were similar but Boolean remission rates differed significantly and if it is accepted as ≥ 12 months, both the SDAI and Boolean remission rates were not different., Conclusion: Low DAS28 and HAQ scores at baseline, anti-CCP were positive predictors of SR. Instead of 6 months, remission duration for ≥ 12 months would probably help us to predict SR independently from the chosen criteria; Boolean or SDAI.

Objectives: Fatigue is a common symptom of chronic inflammatory diseases. The objective of this study was to investigate fatigue in patients with Behçet's syndrome (BS) and to examine the relationship between fatigue and disease activity, quality of life, anxiety and depression., Methods: This is a cross-sectional study of 123 BS patients and 71 healthy controls in Turkey. All subjects completed the Multidimensional Assessment of Fatigue (MAF) questionnaire, Short form-36 (SF-36), Hospital Anxiety and Depression (HADS) scale and Health Assessment Questionnaire (HAQ). Disease activity among BS patients was assessed using the Behçet Syndrome Activity Scale (BSAS) and the physician's global assessment (PGA)., Results: BS patients had significantly higher MAF, HADS-depression (HADS-D) and HADS-anxiety (HADS-A) scores than the healthy controls (P < 0.001). Both the physical and mental components of the SF-36 scale were impaired in BS patients (P = 0.0001). BS patients with active disease, depression and anxiety had significantly higher MAF scores compared to BS patients without active disease, depression and anxiety (P = 0.0001). MAF scores showed positive correlations with HADS-A, HADS-D, HAQ scores and negative correlations with SF-36 mental and physical components. In regression analyses, depression, anxiety and physical dysfunction were significantly associated with fatigue, after adjusting for age, sex, SF-36 physical and mental scores, HAQ, HADS-A, HADS-D and BSAS scores (P < 0.05). Decreases in SF-36 physical and mental scores were significant predictive factors for high MAF score in healthy controls (P < 0.05)., Conclusions: Fatigue is common in clinically active BS patients compared with healthy controls and inactive BS patients. Depression, anxiety and physical dysfunction were significantly associated with fatigue., (© 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Introduction: The prevalence of Sjögren's syndrome (SS) in patients with the diagnosis of SpA has been reported to be higher than normal population. Yet, the vice-versa is unclear. In this study, we aimed to investigate the prevalence of IBP, radiologic sacroiliitis and SpA in patients with primary SS., Methods: 85 patients followed at the rheumatology clinics of the Marmara and Kocaeli Universities with the diagnosis of primary SS between November 2011 and August 2012 were included in this study. The control group consisted of 100 age-and gender-matched patients. Inflammatory back pain and axial SpA were diagnosed according to the assessment of spondylo arthritis International Society (ASAS) criteria., Results: 83 patients were (97%) female and 2 (3%) were male. Mean age of the patients was 49.1 (±11) years. Mean disease duration was 7.3 (±4) years. The patient and control groups were comparable in terms of age and gender (p > 0.05). Inflammatory back pain was observed in 21 (24.7%) of 85 primary SS patients and in 4 (4%) of 100 control subjects (p < 0.001), radiographic sacroiliitis was demonstrated in 9 (10.5%) of primary SS patients and 2 (2%) of the control subjects (p = 0.025). Remaining SpA findings were not encountered in either group., Conclusion: inflammatory back pain and radiologic sacroiliitis is increased in patients with primary SS. Whether IBP, SI joint inflammation and radiologic sacroiliitis is due to the co-existence of SpA and primary SS or IBP is an underdiagnosed clinical feature of SS deserves further studies of large patient numbers.

Aim: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. One of the common characteristics of this disease is its young age predominance. Nearly 90% of patients experience disease flares during early adult age periods. Currently there are limited data for the comparison of early versus late onset FMF and therefore the primary aim of this study was to investigate these two subsets with regard to their certain demographic, clinical and genetic differences., Methods: Early (≤ 20 years, Group 1) and late (> 20 years, Group 2) onset FMF patients were identified from the national FMF registry that involves 2246 patients from 15 adult rheumatology clinics located in different geographical areas of Turkey., Results: Of the 2246 patients, 1633 (72.7%) were aged ≤ 20 years old (Group 1) and the remaining 613 were older than 20 years (Group 2). Delay in diagnosis was longer in Group 1 and fever, peritonitis, pleuritis, erysipelas-like erythema (ELE), arthritis, family history of FMF and amyloidosis were more common in Group 1. On the other hand, sex distribution, rates of amyloidosis, vasculitis and kidney failure were not different between the groups. Among patients with available genotypes, homozygous and heterozygous M694V mutations were significantly higher and heterozygous E148Q mutation was significantly lower in Group 1 compared to Group 2., Conclusion: Patients with FMF whose symptoms start before 20 years of age seem to have severe symptoms and M694V mutation may be responsible for the early expression of the disease., (© 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Gallstone (GS) formation is a multifactorial process and one of the associated factors is hyperinsulinemia. The aim of this cross-sectional study was to determine the association between size and count of gallstones, and insulin levels and resistance. The study group composed of 84 patients who have ultrasonographically confirmed gallstone(s). Insulin level of all participants were measured and insulin resistance was calculated with the homeostasis model assessment of insulin resistance (HOMA-IR) index. All p-values &lt; 0.05 were considered statistically significant. There were 28 patients with single stone and 56 with multiple stones. Mean insulin level was 12.54 &plusmn;11.66 ml/U (median 9.91 ml/U, IQR 6.33) and 56.3% of patients had insulin resistance. Mean stone size was 7.82 &plusmn;7.52 mm (median 6 mm and IQR 11.75). There was a non-significant association and correlation between insulin level and size of GS (p=0.129; r =0.16). There was significant difference between single stone group and multiple stones group, according to the insulin level.

The LupusQoL is a disease-specific health-related quality of life (HRQoL) measure for patients with lupus. We conducted this study to compare the efficiency of LupusQoL-TR (validated Turkish version of the LupusQoL questionnaire) with the 36-item Short-Form Health Survey (SF-36), a generic quality of life (QoL) scale, in Turkish patients with lupus. Both questionnaires were conducted at a single visit to the clinic. Disease activity was measured with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Associations between the LupusQoL-TR and SF-36 domains were examined while also examining age, disease duration, and disease activity for each questionnaire. Descriptive statistics, Spearman's correlation coefficients, and Students t test were performed to analyze the data. A total of 113 consecutive patients with lupus (F/M 108:5, mean age 40.6 ± 11.9 years, mean disease duration 8.5 ± 7.0 years) were included, and 69 % of these were active. The median SLEDAI score was 2 (0-24), the mean global LupusQoL-TR score was 60.9 ± 23.3, and the mean SF-36 score was 41.2 ± 9.0. There was a significant correlation between LupusQoL-TR and SF-36 mean scores (r = 0.83; p < 0.001). QoL assessed by LupusQoL-TR and SF-36 did not correlate with disease activity (r = -0.11; p = 0.244 and r = -0.03; p = 0.721, respectively). LupusQoL-TR and SF-36 questionnaires were beneficial instruments in evaluating HRQoL in Turkish lupus patients. However, LupusQoL-TR and SF-36 were not associated with SLEDAI scores, which suggested that QoL might be affected by other factors besides disease activity, especially in clinically inactive or mildly active patients.

Objective: Remission is the primary aim in the treatment of patients with rheumatoid arthritis (RA). In this study, we aimed to evaluate biomarker profiles of patients in remission by different criteria and compare these profiles with controls., Methods: Serum levels of calprotectin, interleukin 6 (IL-6), type II collagen helical peptide, C-terminal crosslinking telopeptide of type I collagen generated by matrix metalloproteinases (ICTP), matrix metalloproteinase 3 (MMP-3), resistin, and leptin were measured by ELISA in 80 patients. The patients were in Disease Activity Score at 28 joints with erythrocyte sedimentation rate (DAS28-ESR) remission, and had these characteristics: female/male 54/26, mean age 51.4 ± 12.1 years, mean disease duration 11.4 ± 8.1 years, rheumatoid factor positivity 68.7% (n = 55), anticyclic citrullinated peptide positivity 60.7% (n = 48). These patients were also evaluated for the American College of Rheumatology/European League Against Rheumatism (Boolean) and Simple Disease Activity Index (SDAI) remissions. Additionally, 80 age-, sex-, and comorbidity-matched individuals without rheumatic diseases were included in the study as controls., Results: At recruitment of 80 patients in DAS28 remission, 33 patients (41.2%) were found in Boolean remission and 39 patients (48.7%) were in SDAI remission. Serum MMP-3, ICTP, resistin, and IL-6 levels of the 80 patients in DAS28 remission were statistically significantly higher than the controls. Patients in Boolean and SDAI remissions had significantly higher serum ICTP, resistin, and IL-6 levels in comparison with the controls., Conclusion: The 3 commonly used remission criteria of RA are almost similar with regard to patients' biomarker levels. Biomarker profiles of patients may provide complementary information to clinical evaluation of remission and may help to determine the patients under the risk of progression.

Objectives: The aim of our study was to evaluate quality of life (QoL) in patients with systemic lupus erythematosus (SLE) and assess the impact of disease activity and psychological distress on health-related quality of life (HRQoL) in Turkey., Methods: The Medical Outcomes Study Short Form (SF) -36 was used in a cohort of 113 consecutive patients with SLE and 123 age- and gender-matched healthy subjects to measure HRQoL. Patients' disease activity was assessed with SLE disease activity index (SLEDAI) and psychological distress was evaluated by the Hospital Anxiety and Depression Scale (HADS) for all participants. Patients' demographic and clinical data were recorded at the time of HRQoL and HADS testing. Multiple logistic regression analysis was performed to explore the relationships between demographics, disease duration, disease activity as well as psychological (anxiety and depression) variables and the HRQoL., Results: SLE patients have lower quality of life than healthy controls. No relationship between HRQoL and SLE activity or disease duration were observed. Patients with anxiety and/or depression reported worse SF-36 scores than those without psychological distress. The results of multivariate analysis suggested that HADS-A, HADS-D scores and working status were associated with the impairment of HRQoL., Conclusions: HRQoL is impaired in patients with SLE and is associated with mood disorders. Physicians should pay close attention to detect anxiety and depression and manage them in order to improve the quality of life in patients with SLE.

Objective: To investigate the effect of depression, anxiety, fatigue, and fibromyalgia (FM) on the remission status in patients with rheumatoid arthritis (RA), defined according to the 28-joint count Disease Activity Score (DAS28)-erythrocyte sedimentation rate (ESR) and the Boolean-based new American College of Rheumatology/European League Against Rheumatism remission criteria., Methods: The subjects were patients with RA who participated in a hospital-based observational cohort. Patients who met the DAS28-ESR remission criteria at their latest visit were invited to participate in our study. The patient groups fulfilling or not fulfilling the Boolean remission criteria were identified and compared with each other with regard to the presence of depression, anxiety, fatigue (0-50), and FM. The relationship between psychosocial factors and Simplified Disease Activity Index (SDAI) remission, which is the index-based definition of remission in RA, was also investigated., Results: A total of 87 out of 428 patients (20%) with RA met the DAS28-ESR remission criteria and 32 (37%) of these also met the Boolean remission criteria, while 55 (63%) did not. Forty patients were also in SDAI remission. In the Boolean remission group, 2 patients had depression and 2 had anxiety (p = 0.004). In the Boolean nonremission group, 19 patients had depression and 13 had anxiety (p = 0.04). Continuous scales of anxiety (3.34 ± 3.76 vs 5.83 ± 4.70, p = 0.012) and depression (2.18 ± 2.75 vs 4.63 ± 4.10, p = 0.001) were also lower in the Boolean remission group in comparison with the nonremission group. Though FM syndrome was detected in only 1 patient of the Boolean remission group and in 7 patients of the Boolean nonremission group (p = 0.249), patients' polysymptomatic distress scores of FM in the Boolean remission group were significantly lower than those of the nonremission group (3.12 ± 3.25 vs 6.27 ± 5.19, p = 0.001). The mean fatigue scores were 9.5 ± 10.6 in the Boolean remission group and 16.8 ± 12.8 in the Boolean nonremission group (p = 0.006). In multivariate analysis, patient's global assessment (PtGA) and depression were found as the independent discriminators of Boolean-based definition. Similar relationships were also observed between psychosocial factors and SDAI remission., Conclusion: In patients with RA who do not fulfill the Boolean remission criteria, to avoid overtreatment, assessment of anxiety, fatigue, FM, and especially depression must be considered if PtGA scores and disease activity variables are significantly different.

Behçet's disease (BD) is a chronic, multi-systemic disorder that can affect all sizes of arteries and veins. Vascular involvement of BD is generally observed in young men and is an important cause of morbidity and mortality. Arterial lesions can appear as aneurysms, stenosis and occlusions in BD. We, here, present the case of a woman who developed a peripheral arterial aneurysm in her sixth decade, 20 years after disease onset. BD patients with aneurysms should be consulted at any age for pre- and post-operative assessment for immunosuppressive treatment to reduce recurrences, complications and disease activation.

Objectives: Coccydynia is defined as pain in or around the tail bone area. The most common cause of coccydynia is either a trauma such as a fall directly on to the coccyx or repetitive minor trauma. The etiology remains obscure in up to 30% of patients. The literature on the contribution of rheumatic diseases to coccydynia is scarce. Our objective was to investigate the prevalence of coccydynia in ankylosing spondylitis (AS) patients., Methods: One hundred and seven consecutive patients with AS were evaluated for coccydynia were enrolled between January and November 2012 for a cross-sectional analysis. Seventy-four consecutive patients were followed for mechanical back pain as controls and the AS patients were interviewed for the presence of coccydynia. The data collected was evaluated on SPSS® version 11.5 and Microsoft Excel® Programmes., Results: Prevalence of coccydynia in AS (38.3%) was significantly higher than the control group (p<0.0001) in both female and male AS patients (female AS vs. control=40.9% vs. 18.4%, p=0.015 and male AS vs. control=36.5% vs. 8.0%, p=0.005). Both genders were affected equally in the AS group whereas coccydynia was slightly more frequent in female patients in the control group., Conclusions: Coccydynia is a previously neglected symptom of AS and it is almost three times more common in AS than in non-specific chronic low back pain. Our observation may implicate that inflammatory diseases have a role in the etiology of coccydynia, especially in those without a history of recent or past trauma and coccydynia may be a factor associated with the severity of AS as well.