Importance of hematological markers in familial Mediterranean fever in terms of disease severity and amyloidosis.

There are limited follow-up parameters for familial Mediterranean fever (FMF) related to disease severity and amyloidosis. Some hematological markers are emerging to assess inflammation. In this study, we hypothesized that some hematological parameters could be used to determine disease severity and amyloidosis in FMF. We included 274 adult FMF patients, and evaluated the relationship between neutrophil lymphocyte ratio (NLR), monocyte-lymphocyte ratio (MLR) and platelet-lymphocyte ratio (PLR), platelet counts and leukocyte counts, mean erythrocyte haemoglobin concentration (MCHC) and mean erythrocyte haemoglobin (MCH) with disease severity and amyloidosis. First, we classified patients according to disease severity and presence of amyloidosis. We then compared the parameters within the groups. In addition, we determined predictive cut-off values with ROC analysis. Finally, we correlated the change in ISSF scores with the change in hematological parameters of 52 patients with follow-up hematological indices after six months. The patients with severe-moderate group had higher CRP levels (p < 0.001), white blood cell (p = 0.002) and neutrophil counts (p = 0.004) and, conversely, lower MCHC levels (p = 0.001) than patients with mild disease severity. FMF patients with amyloidosis had higher neutrophil (p = 0.04) and monocyte count (p = 0.02), increased NLR (p = 0.01) and lower MLR (p = 0.02) levels than those without. In addition, MCHC levels were also lower in the severe-moderate group in the follow-up analyses after sixth months (p = 0.03). MCHC, neutrophil and monocyte counts, NLR, MLR may be associated with poor prognosis in FMF patients. These parameters can be used in conjunction with acute phase reactant and clinical features to assess disease status.

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.

Beyond expectations: disease duration and psychological burden in psoriatic arthritis.

This study aims to investigate the relationship between disease duration and psychological burden in PsA and to identify the risk factors associated with psychological distress. Patients with PsA who met CASPAR classification criteria enrolled by Turkish League Against Rheumatism (TLAR)-Network. Patients were categorized into three groups based on disease duration: early stage (< 5 years), middle stage (≥ 5, < 10 years), and late stage (≥ 10 years). All patients underwent clinical and laboratory assessment using standardized protocol and case report forms. The associations between psychological variables and clinical parameters were assessed by a multivariate analysis. Of the 1113 patients with PsA (63.9% female), 564 (%50.7) had high risk for depression and 263 (%23.6) for anxiety. The risk of psychological burden was similar across all PsA groups, and patients with a higher risk of depression and anxiety also experienced greater disease activity, poorer quality of life, and physical disability. Multivariate logistic regression revealed that female gender (OR = 1.52), PsAQoL (OR = 1.13), HAQ (OR = 1.99), FiRST score (OR = 1.14), unemployment/retired (OR = 1.48) and PASI head score (OR = 1.41) were factors that influenced the risk of depression, whereas the current or past enthesitis (OR = 1.45), PsAQoL (OR = 1.19), and FiRST score (OR = 1.26) were factors that influenced the risk of anxiety. PsA patients can experience a comparable level of psychological burden throughout the course of their disease. Several socio-demographic and disease-related factors may contribute to mental disorders in PsA. In the present era of personalized treatment for PsA, evaluating psychiatric distress can guide tailored interventions that improve overall well-being and reduce disease burden.

Diagnostic delay in psoriatic arthritis: insights from a nationwide multicenter study.

This study aimed to investigate the duration of diagnostic delay in patients with psoriatic arthritis (PsA) and identify potential contributing factors using a comprehensive, population-based approach. Data were obtained from the Turkish League Against Rheumatism (TLAR)-Network, involving patients who met the CASPAR criteria. Diagnostic delay was defined as time interval from symptom onset to PsA diagnosis, categorized as ≤ 2 years and > 2 years. Temporal trends were assessed by grouping patients based on the year of diagnosis. Various factors including demographics, clinical characteristics, disease activity, quality of life, physical function, disability, fatigue, and well-being were examined. Logistic regression models were used to identify factors associated with diagnostic delay. Among 1,134 PsA patients, mean diagnostic delay was 35.1 months (median: 12). Approximately 39.15% were diagnosed within 3 months, and 67.02% were diagnosed within 24 months. Patients experiencing longer delays had higher scores in Psoriatic Arthritis Quality of Life Questionnaire (PsAQoL), Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), patient's global assessment (PtGA) and physician's global assessment (PhGA). Diagnostic delay has decreased over time, with median delay falling from 60 to 24 months throughout pre-2010 and 2015-2019 terms. Several factors were identified as significant contributors to delayed diagnosis, including lower levels of education (OR = 2.63), arthritis symptoms preceding skin manifestations (OR = 1.72), low back pain at first visit (OR = 1.60), symptom onset age (OR = 0.96), and psoriasis subtype (OR = 0.25). Timely diagnosis of PsA is crucial for effective management and improved outcomes. Despite recent improvements, about one-third of PsA patients still experience delays exceeding 2 years. By identifying influential factors such as education level, arthritis symptoms preceding skin manifestations, initial visit symptoms, age of symptom onset, and psoriasis subtype, healthcare practitioners may create specific techniques to help in early detection and intervention.

Impact of obesity on quality of life, psychological status, and disease activity in psoriatic arthritis: a multi­center study.

This article aims to evaluate the possible effect of obesity on quality of life, psychological status, and other clinical variables in Psoriatic arthritis (PsA). PsA patients have been recruited by the Turkish League Against Rheumatism-Network from various centers in Turkey in this cross-sectional study. Patients with a body mass index (BMI) ≥ of 30 kg/m2 were considered obese. Differences among patients with regard to obesity status were assessed with health-related quality of life measures (PsA Quality of Life Questionnaire [PsAQoL]), psychological status (Hospital Anxiety and Depression Scale [HADS]), and disease activity parameters (the Disease Activity index for PSoriatic Arthritis [DAPSA], Disease Activity Score 28-C-reactive protein [DAS28-CRP], Bath Ankylosing Spondylitis Disease Activity Index [BASDAI], Psoriasis Area and Severity Index [PASI]), physical functions (Ankylosing Spondylitis Functional Index [BASFI], Health Assessment Questionnaire [HAQ], and Health Assessment Questionnaire for the spondyloarthropathies [HAQ-S]). Pain was assessed using visual analog scale of pain (VAS-P), and fatigue was evaluated using visual analog scale of fatigue (VAS-F) and Functional Assessment of Chronic Illness Therapy (FACIT). A total of 1033 patients with PsA, 650 (62.9%) non-obese and 383 (37.1%) obese were included in the study. The PsAQoL, HADS-Anxiety, HADS-Depression, DAPSA, DAS28-CRP, BASDAI, BASFI, HAQ and HAQ-S scores of the obese group were higher than the non-obese group (p < 0.05). VAS-P and PASI scores were similar between group of patients with and without obesity. Obese patients had higher median scores of VAS-F and FACIT than non-obese patients (p < 0.05). Linear regression analysis showed that BMI affects the quality of life, depression, and disease activity. Consequently, obesity has significant associations with higher disease activity, lower QoL, risk of anxiety, depression, and fatigue. Therefore, obesity should also be taken into account in the management of PsA patients.

Assessment of proarrhythmic ventricular electrophysiological remodeling in patients with rheumatoid arthritis.

INTRODUCTION: Rheumatoid arthritis (RA) is related to cardiovascular disease and results in increased mortality rates. Ischemia, autonomic nervous system dysfunction, impaired cardiac ionic currents, and genetic predisposition may be the underlying mechanisms. Proarrhythmic ventricular electrophysiological remodeling detected on the basis of Tp­e interval, Tp-e/QT, and Tp-e/QTc ratios plays a key role in the prognosis. Our aim was to assess proarrhythmic ventricular electrophysiological remodeling in patients with RA, a well-known chronic inflammatory disorder. MATERIALS AND METHODS: A total of 163 patients with RA and 47 patients as a control group were included in this retrospective study. Proarrhythmic ventricular electrophysiological remodeling markers were evaluated in both groups along with baseline demographic and clinical variables. Patients using medication or with chronic disorders that can affect ventricular repolarization markers were excluded. RESULTS: The patients with RA had prolonged Tp­e interval (66 ms [44-80]; 80 ms [78-96], p < 0.001) and increased Tp-e/QT ratio (0.18 [0.12-0.22]; 0.22 [0.20-0.24], p < 0.001) and Tp-e/QTc ratio (0.16 [0.11-0.19]; 0.20 [0.17-0.22], p < 0.001) compared to the control group. CONCLUSION: The Tp­e interval and Tp-e/QT ratio, which may help to clarify the pathophysiological mechanisms of ventricular arrhythmias, were increased in patients with RA.

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.

The effect of gender on disease activity and clinical characteristics in patients with axial psoriatic arthritis.

OBJECTIVES: In this study, we aimed to evaluate the effect of gender on clinical findings, disease activity, functional status and quality of life in patients with axial involvement in Turkey. METHODS: Patients with PsA who met the CASPAR classification criteria were enrolled consequently in this cohort. Turkish League Against Rheumatism (TLAR)-Network was formed with the participation of 25 centres. The demographic variables, fatigue, diagnostic delay, the beginning of peripheral arthritis, enthesitis, dactylitis and spine involvement, inflammatory low back pain, BASFI, HAQ, HAQ-s, visual analogue scale-pain (VAS-pain), anxiety, depression and disease activity parameters (ESR, DAS28, BASDAI) were recorded. Axial involvement was assessed according to clinical and radiological data according to modified New York (MNYC) or Assessment of SpondyloArthritis international Society (ASAS) criteria. RESULTS: A total of 1018 patients with PsA were included in this study. Of the 373 patients with axial involvement, 150 were male (40.2%) and 223 (59.8%) were female. Spondylitis was detected in 14,7% of men and 21,9% of women in all patients. Pain score (VAS) (p < .002), fatigue (p < .001), ESR (p < .001), DAS28 (p < .001), BASDAI score (p < .001), PsAQoL (p < .001), HAQ score (p < ,01), HAQ-S score (p < .001), anxiety (p < .001), depression (p < .024), FACIT (p < .001) and FiRST (p < .001) scores were statistically significantly worse in women than males with axial PsA. However, quality of life was better (p < .001) and PASI score (p < .005) were statistically worse in male patients than in female patients with axial involvement. CONCLUSION: This study has shown that the burden of disease in axial PsA has significant difference between genders. Disease activity, physical disability, functional limitation, depression and anxiety scores were higher in female patients, while quality of life were better and PASI score were higher in male patients. Therefore, we suggest that new strategies should be developed for more effective treatment of axial PsA in female patients.

The impact of fatigue on patients with psoriatic arthritis: a multi-center study of the TLAR-network.

Fatigue is a substantial problem in patients with psoriatic arthritis (PsA) that needs to be considered in the core set of domains. This study aimed to evaluate fatigue and its relationship with disease parameters, functional disability, anxiety, depression, quality of life, and correlation with disease activity as determined by various scales. A total of 1028 patients (677 females, 351 males) with PsA who met the CASPAR criteria were included [Turkish League Against Rheumatism (TLAR) Network multicenter study]. The demographic features and clinical conditions of the patients were recorded. Correlations between fatigue score and clinical parameters were evaluated using the Disease Activity Score 28 (DAS28), Disease Activity in Psoriatic Arthritis (DAPSA), Clinical DAPSA (cDAPSA), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Fibromyalgia Rapid Screening Tool (FiRST), minimal disease activity (MDA), and very low disease activity (VLDA). Fatigue was assessed with the Functional Assessment of Chronic Illness Therapy (FACIT-F) and a 10-point VAS (VAS-F). The mean age of the patients was 47 (SD: 12.2) years, and the mean disease duration was 6.4 (SD: 7.3) years. The mean VAS-F score was 5.1 (SD: 2.7), with fatigue being absent or mild, moderate, and severe in 12.8%, 24.6%, and 62.5% of the patients, respectively. Fatigue scores were significantly better in patients with DAS28 remission, DAPSA remission, cDAPSA remission, MDA, and VLDA (p < 0.001). Fatigue scores significantly increased with increasing disease activity levels on the DAS28, DAPSA, and cDAPSA (p < 0.001). VAS-F scores showed correlations with the scores of the BASDAI, BASFI, PsAQoL, HAD-A, FiRST, pain VAS, and PtGA. FiRST scores showed fibromyalgia in 255 (24.8%) patients. FACIT-F and VAS-F scores were significantly higher in patients with fibromyalgia (p < 0.001). In regression analysis, VLDA, BASDAI score, FiRST score, high education level, HAD-Anxiety, and BMI showed independent associations with fatigue. Our findings showed that fatigue was a common symptom in PsA and disease activity was the most substantial predictor, with fatigue being less in patients in remission, MDA, and VLDA. Other correlates of fatigue were female gender, educational level, anxiety, quality of life, function, pain, and fibromyalgia.

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.

Enthesitis and its relationship with disease activity, functional status, and quality of life in psoriatic arthritis: a multi-center study.

Psoriatic arthritis (PsA) is an inflammatory arthritis with distinct phenotypic subtypes. Enthesitis is assigned as a hallmark of the disease, given its significant relations to disease activity and quality of life. Our objective is to evaluate the prevalence of enthesitis and its association with some clinical parameters, particularly quality of life, using data from a national registry. Patients with PsA meeting ClASsification criteria for Psoriatic Arthritis (CASPAR) were enrolled by means of a multi-centre Turkish League Against Rheumatism (TLAR) Network Project. The following information was recorded in web-based case report forms: demographic, clinical and radiographic data; physical examination findings, including tender and swollen joint counts (TJC and SJC); nail and skin involvement; Disease Activity Score-28 for Rheumatoid Arthritis with Erythrocyte Sedimentation Rate (DAS 28-ESR); Bath Ankylosing Spondylitis Disease Activity Index (BASDAI); Maastricht Ankylosing Spondylitis Enthesitis Score (MASES); Psoriasis Area Severity Index (PASI); Bath Ankylosing Spondylitis Radiology Index for the spine (BASRI-s); Health Assessment Questionnaire (HAQ); Bath Ankylosing Spondylitis Functional Index (BASFI); Health Assessment Questionnaire for the spondyloarthropathies (HAQ-s); Psoriatic arthritis quality of Life scale (PsAQoL); Short Form 36 (SF-36); Hospital Anxiety Depression Scale (HADS); Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F); and Fibromyalgia Rapid Screening Tool (FiRST) scores. The patients were divided into two groups, namely with and without enthesitis, based on the triple Likert-type physician-reported statement of 'active enthesitis', 'history of enthesitis' or 'none' in the case report forms. Patients with active enthesitis were compared to others in terms of these clinical parameters. A total of 1130 patients were enrolled in this observational study. Of these patients, 251 (22.2%) had active enthesitis according to the clinical assessment. TJC, HAQ-s, BASDAI, FiRST and PsAQoL were significantly higher whereas the SF-36 scores were lower in patients with enthesitis (p < 0.05). Chronic back pain, dactylitis, and tenosynovitis were more frequent in the enthesopathy group (59.4%/39%, 13.1%/6.5% and 24.7%/3.4%, respectively). Significant positive correlations between the MASES score and the TJC, HAQ, DAS 28-ESR, BASDAI, FiRST and PsAQoL scores, and a negative correlation with the SF-36 score were found. When linear regression analysis was performed, the SF-36 MCS and PCS scores decreased by - 9.740 and - 11.795 units, and the FiRST scores increased by 1.223 units in patients with enthesitis. Enthesitis is an important involvement of PsA with significant relations to quality of life determined with PsAQoL and SF-36 scores. Our study found higher frequency of dactylitis and chronic back pain, and worse quality of life determined with SF-36 and PsAQoL scores in patients with enthesitis.

Performance of myotonometer in the assessment of skin involvement in systemic sclerosis.

OBJECTIVES: A clinically practical tool to assess skin biomechanical properties rapidly and accurately is still lacking. Our aim was to examine the intra- and inter-observer reproducibility of a myotonometer for objective skin property assessment in systemic sclerosis (SSc), comparing it with the modified Rodnan skin score (mRSS), and distinguishing patients from healthy controls. METHOD: Thirty-four patients (21 limited and 13 diffuse SSc), and 31 age and gender-matched healthy controls were enrolled. Skin tone and stiffness were measured at four different anatomical sites (the forearm, hand, leg, and foot) using a myotonometer. The correlation between the mRSS and skin properties was assessed. Also, hand functionality was evaluated for possible correlations between the variables. The differences in skin properties between dcSSc and lcSSc patients, and healthy controls were assessed using variance analysis. RESULTS: Intra- and inter-examiner reproducibility were excellent (ICC = 0.70 to 0.98) for tone and stiffness except for non-dominant hand tone, which showed good reliability (ICC = 0.64 to 0.74). Stiffness and tone values of the hands, forearms, and feet significantly correlated with mRSS total score (r = 0.40 to 0.71, p < 0.05). Additionally, tone and stiffness of the hands and forearms moderately correlated with hand function (p < 0.05). Tone and stiffness values increased in patients with dcSSc compared to healthy controls, or patients with lcSSc, at the hands, forearms, and legs (p < 0.05). CONCLUSIONS: Our findings emphasize the potential utility of the myotonometer for assessing skin properties and differentiating SSc patients from controls, demonstrating its promise as a valuable clinical evaluation tool in this context. Key Points •The myotonometer displayed excellent intra- and inter-examiner reproducibility for assessing skin properties. •Skin tone and stiffness parameters well correlated with the mRSS scores. •The myotonometer can distinguish patients with diffuse cutaneous SSc from healthy controls.

Pandemic of the century: COVID-19 in inflammatory rheumatic diseases of a national cohort with 3,532 patients.

Objectives: This study aimed to assess the clinical outcomes and risk factors for severe coronavirus disease 2019 (COVID-19) in patients with inflammatory rheumatic disease (IRD) of a national cohort. Patients and methods: The multicenter cross-sectional study was carried out between July 15, 2020, and February 28, 2021. Data collection was provided from a national network database system, and 3,532 IRD patients (2,359 males, 1,173 females; mean age: 48.7±13.9 years; range; 18 to 90 years) were analyzed. Demographics, clinics about rheumatic disease, comorbidities, smoking status, being infected with COVID-19, and the course of the infection were questioned by rheumatology specialists. Results: One hundred seventeen patients were infected with COVID-19, the hospitalization rate due to COVID-19 was 58.9%, and the mortality rate was 1.7%. There was no difference between the COVID-19 positive and negative groups in terms of rheumatic disease activities and receiving drugs. It was observed that patients with COVID-19 had worse compliance with isolation rules, and bacillus Calmette-Guérin (BCG) vaccination was less common. The mean age and the rate of smoking of hospitalized COVID-19 patients were higher than those without hospitalization. Conclusion: In this cohort, in which real-life data were analyzed, COVID-19 rates in IRD patients were similar to the general population for the same period. Compliance with the isolation rules and BCG vaccination attracted attention as components that reduce the risk of COVID-19 infection. The risk factors for hospitalization were older age and smoking.

Evaluation of peripheral nerve involvements in patients with familial Mediterranean fever.

Objectives: The aim of this study was to evaluate possible peripheral and autonomic nerve involvement in familial Mediterranean fever (FMF) patients with nerve conduction studies, sympathetic skin response (SSR) and RR interval variability (RRIV). Patients and methods: The comparative case series was conducted with 76 participants between November 2017 and December 2018. Forty-six FMF patients, [12 with amyloidosis (5 males, 7 females; mean age: 44.7±13.9 years) and 34 without amyloidosis (14 males, 20 females; mean age: 35.9±8.7 years)], and 30 healthy volunteers (11 males, 19 females; mean age: 38.4±10 years) were included in this study. Nerve conduction parameters, SSR latency and amplitude from palmar and plantar responses, and RRIV at rest and deep breathing were studied in all the subjects. Neuropathic symptoms of the patient group were evaluated using the survey of autonomic symptoms scale and the neuropathy disability score. Results: Nerve conduction studies of the patient group revealed polyneuropathy in seven (15.21%) patients and carpal tunnel syndrome in six (13.04%) patients. The mean amplitudes of SSR measured from the soles were significantly lower than the control group (p=0.041). The mean values of RRIV during rest and hyperventilation were lower in the patient group compared to the control group, but no statistically significant difference was found (p=0.484, p=0.341). Conclusion: We detected that the prevalence of carpal tunnel syndrome in our patient population (13.04%) was higher than in the general population. Most of the changes in the range of parameters of SSR and RRIV determined in the patient group did not reach statistical significance, suggesting subclinical dysautonomia in FMF patients.

Anti-mutated citrulline vimentin antibodies may be higher in Ankylosing spondylitis, but may not have a role in diagnosing disease and may not be associated with disease severity.

OBJECTIVE: Ankylosing spondylitis (AS) is a rheumatologic disease with severe morbidity and mortality. Many studies in the literature showing that serum antibodies against anti-mutated citrullinated vimentin (anti-MCV ab) can be elevated in rheumatoid arthritis (RA) patients. However, there is little data in the literature about the levels of anti-MCV antibodies in AS patients. We designed the study to evaluate the role of anti-MCV antibody in the diagnosis of AS and to investigate whether it is associated with disease activity parameters. METHODS: There were three separate groups in our study. The number of participants in these groups is 60 patients in the AS group, 60 patients in the RA group, and 50 healthy participants in the control group. The anti-MCV ab levels of the participants were measured by enzyme-like immune assay method. We compared anti-MCV levels between groups. We then evaluated its role in the diagnosis of AS and evaluated its relationship with disease activity parameters. RESULTS: The anti-MCV antibody levels of both AS (p=0.006) and RA (p>0.001) patients were found to be significantly higher than controls. Anti-MCV antibody was higher than predefined threshold level (20 IU/mL) in 4 of 60 (6.7%) AS patients. Anti-MCV levels are similar in patients with or without a -acceptable symptom state (PASS). There is also no appropriate anti-MCV cutoff level with respect to PASS and a highly sensitive and specific level for diagnosis of AS. CONCLUSION: Although AS patients has higher anti-MCV levels than controls, it may have a limited ability to AS diagnosis and to predict severity of the disease.

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort.

INTRODUCTION: Familial Mediterranean fever (FMF) is one of the common autoinflammatory diseases with multisystemic manifestation. Pleuritis is the only known pulmonary involvement of FMF; however, as far as we know, thoracic involvements in pleural, parenchymal, bronchial, and vascular structures have not been evaluated yet. METHOD: We included 243 consecutive FMF patients who applied to our clinic within the last 5 years and were requested to have a thorax CT for any reason and 122 trauma patients without any comorbidity. An experienced radiologist evaluated the thorax CT images blindly according to the relevant guidelines. We then presented the common incidental pulmonary and mediastinal findings on the thorax CT. Additionally, we compared patients with and without lung involvement according to demographic and disease-related parameters. RESULTS: In our study, 167 of 243 patients (68.7%) had at least one of the pulmonary findings on their thorax CT. The most common pulmonary findings were apical fibrosis in 96 (39.5%) patients, parenchymal fibrotic changes in 48 (19.8%) patients, and a solitary parenchymal nodule smaller than 4 mm in 33 (13.6%) patients. All demographic, genetic, and disease-related characteristics, including the frequency of spondyloarthropathy, were similar in patients with and without pulmonary findings. CONCLUSIONS: We showed that the most common incidental pulmonary finding in our FMF cohort was apical fibrosis on thoracic CT. Our data did not show causality between FMF and apical fibrosis; therefore, more studies are needed to evaluate the frequency and clinical significance of apical fibrosis in FMF. Key Points • More than two-thirds of familial Mediterranean fever (FMF) patients in our study group who underwent a thoracic scan for any reason had pulmonary and mediastinal findings on thorax computed tomography (CT). • In our FMF cohort, the most common incidental pulmonary finding on their thorax CT was apical fibrosis. • All demographic and disease-related characteristics, including the frequency of spondyloarthritis, were similar between patients with and without pulmonary and mediastinal findings.

The impact of nail psoriasis on disease activity, quality of life, and clinical variables in patients with psoriatic arthritis: A cross-sectional multicenter study.

AIM: Nail involvement is common in psoriatic arthritis. This study assesses clinical characteristics, nail psoriasis prevalence, and impact of nail psoriasis on disease activity in patients with psoriatic arthritis (PsA). METHOD: This cross-sectional multicenter study was conducted by the Turkish League Against Rheumatism using PsA patients recruited from 25 centers. Demographic and clinical characteristics of PsA patients, such as disease activity measures, quality of life, and nail involvement findings were assessed during routine follow-up examinations. Patients were divided into two groups according to the presence or absence of nail psoriasis and compared using the χ2 test or Fisher exact test for categorical variables and the t-test or Mann-Whitney U test for continuous variables. RESULTS: In 1122 individuals with PsA, 645 (57.5%) displayed nail psoriasis. The most frequent features of fingernails were ridges (38%), followed by pitting (21%) and onycholysis (19%). More females were present in both groups (with and without nail psoriasis; 64% vs 67%, P < 0.282). Patients with nail psoriasis were older, indicated more pain and fatigue, experienced greater swelling, tender joint counts, and skin disease severity, and had a higher disease activity score compared with those without nail psoriasis (all P < 0.05). CONCLUSION: We demonstrate an increased prevalence of nail psoriasis observed in patients with psoriatic arthritis. Patients with nail involvement experience increased disease activity, lower quality of life, and diminished mental and physical status compared with those without nail involvement.

Familial mediterranean fever patients may have unmet needs for the treatments of exertional leg pain and enthesitis.

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.

Quality of life, disease activity and preferences for administration routes in rheumatoid arthritis: a multicentre, prospective, observational study.

Objective: We aimed to evaluate quality of life (QoL), disease activity, compliance to treatment, patient and physician preferences for route of administration (RoA), status of health and pain in RA patients starting advanced treatments or needing a switch, and the factors associated with patient preferences. Methods: A multicentre, prospective, observational and 1-year follow-up study was conducted, between 2015 and 2020, in adult RA patients using advanced treatments for the first time or needing a switch in their current treatments. All the data collected were entered into electronic case report forms. DAS in 28 joints with ESR [DAS28-4(ESR)], EuroQol 5-Dimensional Questionnaire (EQ-5D), HAQ Disability Index (HAQ-DI), Compliance Questionnaire for Rheumatology (CQR-19), Work Productivity and Activity Impairment Instrument (WPAI) and Patient Global Assessment-Visual Analogue Scale (PGA-VAS) questionnaires were used for longitudinal assessments. Results: Four hundred and fifty-nine patients were enrolled. Three hundred and eight patients (67.1%) attended the final study visit at 12 months and were included for comparative analyses. Irrespective of RoA, the disease activity and QoL improved significantly at 12 months, whereas compliance worsened. At baseline and 12 months, EQ-5D and DAS28-4(ESR) scores were significantly correlated (P < 0.001). The WPAI scores changed significantly in favour of better outcomes over 12 months after initiation of advanced treatment or switching (P < 0.001). A higher proportion of patients preferred an oral RoA, in comparison to physicians (53.6% vs 31.4%; P < 0.001). Patient and physician RoA preferences were independent of gender, age, disease duration, advanced treatment type and the EQ-5D-3L, DAS28-4(ESR), HAQ-DI, PGA-VAS and CQR-19 scores at baseline. Conclusion: The oral route was more frequently preferred by patients compared with physicians, although patients' preference rates showed a slight increase towards the end of the treatment, which might be an important factor for RA outcomes. Better control of disease activity and QoL were achieved at 12 months, regardless of RoA.

Inconsistencies of the Disease Activity Assessment Tools for Psoriatic Arthritis: Challenges to Rheumatologists.

OBJECTIVE: Currently, concerning the evaluation of psoriatic arthritis (PsA), there is no agreement on a standardized composite index for disease activity that includes all relevant domains. The present study sought to assess the rates of remission (REM)/low disease activity (LDA) and disease states [minimal disease activity (MDA), very low disease activity (VLDA)] as defined by diverse activity scales (DAPSA, DAS28-ESR) in an attempt to display discrepancies across these assessment tools for peripheral PsA. METHODS: The study involved 758 patients (496 females, 262 males; mean age 47,1 years) with peripheral PsA who were registered to the Turkish League Against Rheumatism (TLAR) Network. The patients were assessed using the DAS28-ESR, DAPSA, MDA, and VLDA. The overall yield of each scale was assessed in identifying REM and LDA. The presence or absence of swollen joints was separately analysed. RESULTS: The median disease duration was 4 years (range 0-44 years). According to DAPSA and DAS28-ESR, REM was achieved in 6.9% and 19.5% of the patients, respectively. The rates of MDA and VLDA were 16% and 2.9%, respectively. Despite the absence of swollen joints, a significant portion of patients were not considered to be in REM (296 (39.1%) patients with DAS28-ESR, 364 (48%) with DAPSA, and 394 (52%) with VLDA). CONCLUSION: Patients with peripheral PsA may be assigned to diverse disease activity levels when assessed with the DAS28-ESR, DAPSA, MDA and VLDA, which would inevitably have clinical implications. In patients with PsA a holistic approach seems to be necessary which includes other domains apart from joint involvement, such as skin involvement, enthesitis, spinal involvement, and patient-reported outcomes.