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Objective: This study aimed to evaluate the prevalence of sarcopenia and its clinical significance in Turkish women with SLE, exploring the association between muscle mass, muscle strength and SLE disease activity., Methods: A cross-sectional study was conducted at Gazi University Hospital's Department of Rheumatology from January to December 2020. It involved 82 patients with SLE, diagnosed according to the 2019 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria, and 69 healthy controls. Sarcopenia was assessed using hand grip dynamometry (hand grip strength (HGS)) and bioelectrical impedance analysis for muscle mass, with sarcopenia defined according to the 2018 European Working Group on Sarcopenia in Older People criteria and specific cut-offs for the Turkish population. The main outcomes measured were the presence of sarcopenia and probable sarcopenia, HGS values, skeletal muscle mass index and SLE Disease Activity Index 2000 (SLEDAI-2K)., Results: Among the patients with SLE, 51.2% met the criteria for probable sarcopenia and 12.9% were diagnosed with sarcopenia. The mean HGS was significantly lower in the SLE group (21.7±4.9 kg) compared with controls, indicating reduced muscle strength. The prevalence of anti-double-stranded DNA (anti-dsDNA) antibodies was 82.9%. Multivariate regression analysis identified height and levels of anti-dsDNA antibodies as independent predictors for developing probable sarcopenia. No significant association was found between clinical parameters, including SLEDAI-2K scores, and sarcopenia status., Conclusions: Sarcopenia is prevalent among Turkish women with SLE, with a significant proportion showing reduced muscle strength. The study found no direct association between sarcopenia and SLE disease activity or clinical parameters. These findings underscore the importance of including muscle strength assessments in the routine clinical evaluation of patients with SLE to potentially improve management and quality of life., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The present study aims to determine the frequency of vascular calcification in Takayasu arteritis (TA) and the risk factors for it and to evaluate its relation with atherosclerotic predictors such as metabolic syndrome (MS), left ventricular mass index (LVMI) and carotid intima-media thickness (CIMT)., Methods: A cross-sectional study was conducted in patients with TA; MS was defined according to the US National Cholesterol Education Program/Adult Treatment Panel III (NCEP/ATP III) criteria. The study included 49 TA patients (22 with MS, 27 without MS) and 31 healthy controls (HCs). Non-contrast computed tomography measured calcification in coronary arteries, aorta, and branches., Results: Forty-seven patients (95.9%) were female and mean age was 33.45 ± 8.53 years. Total calcification score (mean ± SD; 5223.9 ± 18041.1 AU vs. 35.87 ± 72.70 AU (p = 0.05)), CIMT, and LVMI were found to be significantly higher in TA patients than HCs (p < 0.05). While there was no significant difference between the total calcification score of MS (+) TA patients and MS (-) TA patients, in both patient groups, the total calcification score was found to be significantly higher than HCs. MS (+) and MS (-) groups were found to have significantly higher CIMT and LVMI values than the control group, in addition, MS (+) patients were found to have significantly higher LVMI and CIMT values than MS (-) group (p < 0.05)., Conclusion: Vascular calcification, CIMT, and LVMI are elevated in all TA patients, with greater impact in the presence of MS., (Copyright © 2024 Hellenic Society of Cardiology. Published by Elsevier Inc. All rights reserved.)

Objective: Behçet's Disease (BD) is a chronic multisystem vasculitis that manifests with destructive inflammation affecting the eyes, central nervous system, and blood vessels. The pathology of vein involvement in BD is poorly characterized. Magnetic resonance (MR) venography gives more comprehensive information about deep veins and adjacent tissues. In this study, we aimed to characterize vein involvement and evaluate the diagnostic utility of MR venography in BD., Methods: Sixty-five BD patients who fulfilled the International Study Group (ISG) criteria and 20 healthy control subjects were enrolled. Inferior vena cava (IVC), common iliac veins (CIV), external (EIV) and internal iliac veins (IVV), common femoral veins (CFV), femoral veins (FV), and greater saphenous veins (GSV) of BD patients and healthy controls were evaluated with MR venography and ultrasonography for the presence pathologic features, luminal thrombi, vessel wall changes, and perivascular abnormalities., Results: 33 vascular and 32 non-vascular BD patients (mean age 39.3 ± 11.3 years and 48 [73.8%] male) were enrolled. MR venography revealed diffuse concentric thickening of the walls of IVC, CIV, EIV, IIV, CFV, FV, and GSV in BD (healthy controls vs. BD p<0.05 for all vein segments). MR venography provided additional information about veins and perivascular tissues like contrast enhancement, enlarged lymph nodes, and seminal vesicle vascularization, which were remarkably more frequent in vascular BD than non-vascular BD and healthy controls., Conclusion: The results of our study suggest that the involvement of the venous system is diffuse and generalized in BD, and demonstration of venulitis might help diagnose the disease., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome., Methods: This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries., Results: Since its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access., Conclusions: This International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder., (Copyright © 2022 Sota, Vitale, Więsik-Szewczyk, Frassi, Lopalco, Emmi, Govoni, de Paulis, Marino, Gidaro, Monti, Opris-Belinski, Pereira, Jahnz-Rózyk, Gaggiano, Crisafulli, Iannone, Mattioli, Ruffilli, Mormile, Rybak, Caggiano, Airò, Tufan, Gentileschi, Ragab, Almaghlouth, Aboul-Fotouh Khalil, Cattalini, La Torre, Tarsia, Giardini, Ali Saad, Bocchia, Caroni, Giani, Cinotti, Ruscitti, Rubegni, Dagostin, Frediani, Guler, Della Casa, Maggio, Recke, von Bubnoff, Krause, Balistreri, Fabiani, Rigante and Cantarini.)

Background/objective: Core muscle endurance (CME), which is the ability of sustaining the activity of trunk muscles, has been shown to be lower in patients with ankylosing spondylitis (AS). The aim was to investigate the possible relationship between CME times and balance, fatigue, physical activity (PA) level, and thoracic kyphosis angle., Methods: Fifty-one patients with AS with a mean age of 41.0 years (interquartile range, 25/75 years; 29.0/51.0 years) were included in the study. Core muscle endurance times were assessed by using trunk extension, trunk flexion, and side bridge tests. Overall stability index, anteroposterior stability index, mediolateral stability index, and limits of stability were evaluated with the Biodex Balance System. Fatigue and PA levels were surveyed using Fatigue Severity Scale and International Physical Activity Questionnaire, respectively. Thoracic kyphosis angle was measured by using a digital inclinometer. Additionally, CME times were compared for "high-fatigue" versus "low-fatigue" and as "low PA" versus "moderate/high PA" groups. Spearman correlation coefficients and Mann-Whitney U test were used for statistical analysis., Results: Significant correlations were detected between overall stability index, anteroposterior stability index, Fatigue Severity Scale, International Physical Activity Questionnaire, and all CME tests (p < 0.05) and between mediolateral stability index and side bridge test (p < 0.05). Limits of stability correlated only with side bridge test (p < 0.05). Core muscle endurance significantly differed between high-fatigue and low-fatigue groups (p < 0.05), except trunk flexor test (p > 0.05). No significant differences were observed between low PA and moderate/high PA groups (p > 0.05), except side bridge test (p < 0.05)., Conclusions: Core muscle endurance times seem to be related to PA level, fatigue, and balance but not with thoracic kyphosis angle. Assessing CME in patients with AS might help in planning individualized exercise programs., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Anakinra and canakinumab are the most commonly used agents in colchicine resistant/intolerant patients. In this study we investigated long-term efficacy and safety of anakinra and canakinumab., Methods: In this retrospective study, we enrolled 101 adult patients with familial Mediterranean fever (FMF). Clinical and laboratory parameters before and after treatment with anakinra/canakinumab and the side effects observed during the treatment were recorded. All patients received anakinra initially and switched to canakinumab, in case of inadequate response/intolerance., Results: The median (IQR) duration of treatment with anti-IL-1 agents was 35 (24-47.5) months. 101 patients were treated with anakinra and 27 patients with canakinumab. The autoinflammatory diseases activity and attacks decreased with both anakinra and canakinumab. Anakinra was effective in decreasing proteinuria and canakinumab was not effective in decreasing proteinuria in anakinra unresponsive patients. The modified FMF score was achieved in 76.2% of anakinra and 88.9% of canakinumab group. Injection site reactions (ISRs, n:15) was the most common reason of discontinuation of anakinra and most of ISRs developed in first 3 months of treatment. One severe skin rash, two anaphylactic reactions and one severe neutropenia were observed with anakinra; in the first, eighth, twelfth and fiftieth months, respectively. No severe side effects or side effect-related discontinuation of canakinumab were observed., Conclusions: Anakinra and canakinumab seem to be effective in long-term management of FMF patients. Canakinumab had a favourable safety/tolerability profile. Anakinra is also generally safe, but the serious side effects that may be observed in the short and long-term use should be taken into account.

Introduction: Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis. Some patients suffer from associated inflammatory conditions and damage related to FMF that may potentially impair work productivity which have not been studied to date., Methods: Consecutive FMF patients who were attending a tertiary referral center and age-and sex-matched healthy subjects enrolled into the study. Disease activity was assessed with autoinflammatory disease activity index (AIDAI) and patient global assessment. Damage was evaluated using Autoinflammatory Disease Damage Index (ADDI). Quality of life (QoL) and work productivity were determined with 36-Item Short Form Health Survey (SF-36) and Work Productivity and Activity Impairment Specific Health Problem v2.0 (WPAI:SHP), respectively., Results: There were 111 FMF patients, 60 female (54%), mean age 32.7±8.7 years. There were significant impairments in all domains of the SF-36 QoL in FMF patients. Of the 111 patients enrolled, 65 (58.6%) were employed in a paid work. Mean% ±SD impairment in work productivity both assessed as absenteeism (9.3±23.2% vs. 0.7±2.6, p=0.013) and presenteeism (35.2±32.6% vs. 9.6±14.7, p<0.001) were significantly higher in FMF patients compared to healthy subjects. Impairment in work productivity was correlated with the number of attacks, disease activity, colchicine resistance, and disease-associated damage. Impairment was most significant in colchicine-resistant FMF patients but lower in those on interleukin (IL)-1 antagonist treatments., Conclusions: FMF causes significant work impairment and reduced QoL which is associated with disease activity and damage. The use of IL-1 antagonists may help to improve work productivity and QoL in FMF patients with frequent attacks. Key points • Work productivity is impaired in patients with FMF. • Disease activity was an independent predictor for impaired work productivity. • IL-1 antagonists may improve work productivity and quality of life in FMF patients with frequent attacks.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schönlein purpura, Behçet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.