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Objective: To compare the effects of telerehabilitation vs home-based exercise programs for knee osteoarthritis (KOA). Method: Patients diagnosed with moderate/mild KOA were enrolled in the study and randomized into two groups. The patients in the telerehabilitation group did their exercises via video conference simultaneously, accompanied by a physiotherapist, while the patients in the control group were given a brochure showing how to do the exercises and explaining how to do each exercise. Patients completed 30-s chair stand test (30 CST), Knee Injury and Osteoarthritis Outcome Score (KOOS), Numeric Rating Scale (NRS), International Physical Activity Questionnaire Short Form (IPAQ-SF), Hospital Anxiety and Depression Scale (HADS), TAMPA Kinesiophobia Scale (TKS), Fatigue Severity Scale (FSS) twice before and after 8-week treatment, and Quality Indicators Questionnaire for Physiotherapy Management of Hip and Knee Osteoarthritis (QUIPA) and Exercise Adherence Rating Scale (EARS) after treatment only. Moreover, the number of painkillers that patients used in the last 15 days was recorded before and after treatment, and patient satisfaction with treatment was questioned after treatment. Results: Forty-eight patients were included in the study. The mean age of patients was 55.83 ± 6.93 years, and 43 (89.6%) patients were women. No significant differences were determined between groups in terms of baseline characteristics. After the 8-week follow-up, telerehabilitation group demonstrated better 30 CST, IPAQ-SF, KOOS, QUIPA, treatment satisfaction, and total and C subscale of EARS scores increment and greater NRS, HADS, TKS, and FSS score reduction than the control group. It was determined that there was a statistically significant difference between the telerehabilitation and control groups for all of the specified parameters; however, no statistically significant difference was found for the B subscale of EARS. Conclusion: This study indicated that telerehabilitation is superior to self-management. Moreover, through this innovative and population specific web-based approach for KOA, a vast number of patients who have internet access could be reached. Thus, patients with KOA received effective treatment. Key Points • Telerehabilitation is superior to self-management. • Telerehabilitation is a quality practice with the potential to reduce symptoms in patients with knee OA. [ABSTRACT FROM AUTHOR]

In a previous study, it has been shown that the population of Th17 lymphocytes was increased in patients with FMF. IL-21 and IL-23 play significant roles in the production and differentiation of Th17 cells. In this study, we aimed to evaluate serum levels of IL-21 and IL-23 in FMF patients both at diagnosis and after treatment, and to compare these levels with those of healthy controls. Twenty-seven newly diagnosed patients with FMF in attack-free periods and twenty-seven healthy volunteers enrolled in the study. The groups were comparable with respect to age and gender. IL-21 and IL-23 levels in serum samples from patients at the time of diagnosis, in remission after treatment, and from the control groups were analysed using the ELISA method. There was no significant difference between the cytokine levels of the patient group at the time of diagnosis and the cytokine levels of the control group (for IL-21, p: 0.28 and for IL-23, p: 0.56). Similarly, there was no significant difference between the patients' cytokine levels at the time of diagnosis and after treatment (for IL-21, p: 0.99 and for IL-23, p: 0.08). Interleukin levels at the time of diagnosis did not differ among patient groups based on the presence of clinical findings or the M694V genotype. Our results suggest that IL-21 and IL-23 do not play a role in the pathogenesis of the disease. However, while interpreting these findings, it should be considered that patients with active episodes were excluded and cytokine levels were not measured in tissue samples. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the impact of familial Mediterranean fever (FMF) features on the clinical course and outcomes of coronavirus disease 2019 (COVID-19) and clinical course of FMF after COVID-19. Methods: Consecutive FMF patients with COVID-19 were enrolled from three referral hospitals. Clinical features of FMF and detailed COVID-19 information were obtained from patient interviews and medical records. Results: Seventy-three FMF patients were included in the study. 94.5% of patients had clinical symptoms of COVID-19. We found 24.7% hospitalization, 12.3% respiratory support, 4.1% intensive care unit admission, 6.8% complication, and 1.4% mortality rate in patients. The risk factors of hospitalization for respiratory support were male gender [OR: 7.167 (95% CI: 1.368-37.535)], greater age [OR: 1.067 (95% CI: 1.016-1.121)], and non-adherence to colchicine treatment before the infection [OR: 7.5 (95% CI: 1.348-41.722)]. One-third of patients had reported attacks after COVID-19. The patterns of triggered attacks were fever, peritonitis, pleuritis, transient arthritis, chronic knee mono-arthritis, and protracted febrile myalgia. Conclusions: FMF characteristics were not associated with worse outcomes of COVID-19. Colchicine non-adherence was the risk factor of hospitalization for oxygen support. The rate of FMF attacks after COVID-19 is prominently increased, with some of them being protracted and destructive. [ABSTRACT FROM AUTHOR]

Aim Cognitive dysfunction is a neurologic manifestation in primary Sjögren syndrome ( PSS). On the other hand, several antibodies are related to cognitive dysfunction. The aim of this study is to assess the cognitive dysfunction of PSS patients via detailed neurologic tests. Moreover, its associations with antibodies were also evaluated. Method Twenty-eight female patients with PSS and 17 healthy controls comprised the study groups. Short-term memory, long-term memory, verbal learning, visual memory, visual spatial perception, attention, verbal frequency function, executive functions and information processing speed were evaluated with neurologic tests in both of the study groups. Furthermore, anti- N-methyl-D-aspartate ( NMDA) type anti-glutamate-receptor antibody, anti-ribosomal-p and antiganglioside antibodies were assessed in the study groups. Results The attention, data processing speed, verbal learning, short-term verbal memory and visuo-spatial perception performances were lower in the patients with PSS when compared to the healthy controls. The difference reached statistical significance in Paced Auditory Serial Addition Test ( P < 0.01), Serial Digit Learning Test ( P < 0.01), clock drawing ( P = 0.03), Auditory Verbal Learning Test immediate verbal memory ( P = 0.01) and Benton Judgement of Line Orientation Test ( P = 0.03). Even if antiganglioside antibodies were more likely to be present in the PSS group when compared to the healthy controls, no relationship was found between its positivity and cognitive dysfunction. Conclusion Results of this study suggest that cognitive dysfunction is quite prevalent in PSS patients without being associated with studied antibodies. [ABSTRACT FROM AUTHOR]

Objective: To report a patient with coeliac disease (CD) associated with primary biliary cirrhosis (PBC) who presented with myopathy without classical symptoms of CD. Clinical Presentation and Intervention: A 42-year-old woman presented with inability to walk and marked loss of motor function. She had elevated liver enzymes with a cholestatic pattern. Antimitochondrial antibody M2 band, anti-endomysial antibody, antigliadin IgA and IgM were positive. Histopathologic findings of the liver revealed PBC and duodenal biopsy was consistent with CD. She was also found to have osteomalacia. She showed slow response to gluten-free diet, but accelerated full recovery with vitamin D replacement. Conclusion: In PBC patients with subclinical CD and myopathy, vitamin D status can provide a basis for treatment. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Hydroxychloroquine (HCQ) use is associated with less disease activity, flares, damage and improved survival in Systemic Lupus Erythematosus (SLE). However, its effect on patient reported health outcomes (PROs) such as quality of life (QOL) is not known. Methods: International data from Study on Outcomes of Lupus (SOUL) from 2,161 SLE patients were compared by HCQ use. Disease activity and damage were assessed using SELENA-SLEDAI and SLICC-ACR/SDI. QOL was evaluated using LupusPRO and Lupus Impact Tracker (LIT). Linear regression analyses were performed with LupusPRO summary scores health related HRQOL, non-health related NHRQOL and LIT as dependent and HCQ use as independent variable. Analyses were undertaken to test mediation of effects of HCQ use on QOL through disease activity. Results: Mean age was 40.5 ± 12.8 years, 93% were women. Sixty-three (1363/2161) percent were on HCQ. On univariate analysis, HCQ use was associated with (a) better QOL (LupusPRO-HRQOL: β 6.19, 95% CI 4.15, 8.24, P ≤ 0.001, LupusPRO NHRQOL: β 5.83, 95% CI 4.02, 7.64, P ≤ 0.001) and less impact on daily life (LIT: β −9.37, 95% CI −12.24, −6.50, P ≤ 0.001). On multivariate and mediational analyses, the effects of HCQ on QOL were indirectly and completely mediated through disease activity. Conclusions: HCQ use in SLE is associated with better patient reported health outcomes (LupusPRO-HRQOL and NHRQOL and impact on daily life), and the effects are mediated through disease activity. This information can facilitate patients and physician's communication with decision-making regarding the use of HCQ for SLE management. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items. Methods This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months' follow-up. Patients were stratified based on whether they had persistent inflammation. We used logistic regression analysis to investigate independent predictors of persistent inflammation and the associated individual damage items. Results One hundred and forty-two (15%) patients had persistent inflammation. Active FMF (54%) was the most prominent reason for the persistent inflammation. Spondylarthritis (16%), other inflammatory arthritis (8%) and IBD (2%) were other frequent reasons. Male gender, history of exertional leg pain, inflammatory comorbidities, M694V homozygosity, colchicine resistance, lower education levels and musculoskeletal attack dominance were found to be the independent predictors of persistent inflammation. Earlier disease onset led to a tendency towards persistent inflammation. Patients with persistent inflammation were more likely to suffer damage. There is an increased risk of developing proteinuria, amyloidosis and renal insufficiency. Conclusion We identified, for the first time, the predictors of persistent inflammation in adult FMF patients and related individual damage items of the Autoinflammatory Disease Damage Index. Persistent inflammation is insidious and one of the chief causes of damage; therefore, especially patients with these predictors should be followed up more closely. If detected, underlying inflammatory comorbidities should be assessed meticulously as early detection and proper treatment strategies may favourably impact the natural history of the disease. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to translate and adapt the Mouth Handicap in Systemic Sclerosis (MHISS) Questionnaire into the Turkish language and evaluate its validity and reliability in Turkish systemic sclerosis (SSc) patients. Method: The MHISS was translated according to Beaton guidelines. Patients being diagnosed with SSc, being between 18‐65 years old and receiving no treatment between test‐retest assessments were included to study. Test‐retest reliability was evaluated, comparing the results of two administrations, with Spearman's correlation. Internal consistency was assessed by Cronbach's α. Validity of the questionnaire was assessed by comparison with mouth opening, total scores of Disabilities of Arm, Shoulder and Hand Questionnaire (DASH) and Health Assessment Questionnaire (HAQ). Construct validity was tested by factor analysis. Results: Forty‐five SSc patients were included in the study. The Turkish version of the MHISS (MHISS‐T) met set criteria of reliability and validity. Internal consistency (Cronbach's α = 0.863) and test‐retest reliability were excellent (r =.88). The correlations between MHISS‐T and inter‐incisor distance, MHISS‐T and HAQ and MHISS‐T and DASH were negatively and statistically significant (r = −0.739, P <.001), very good and statistically significant (r =.664, P <.001), good and statistically significant (r =.570, P <.001), respectively. Regarding factor analysis, MHISS‐T has three subscales. Conclusion: Our results demonstrated that the Turkish version of the MHISS‐T has excellent test‐retest reliability and very good validity. As a result of this study we determined that MHISS‐T is a valid and reliable instrument to measure mouth disabilities in Turkish‐speaking SSc patients. [ABSTRACT FROM AUTHOR]

Aim: Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown etiology characterized by excessive collagen production, endothelial cell injury, microvascular obliteration, cutaneous fibrosis and progressive visceral disease. The hands are frequently involved during the progression of the disease, with symmetrical skin thickening as a prominent feature. Modified hand mobility in scleroderma (mHAMIS) test is a measurement method to assess hand mobility in patients with SSc. Knowing the inter‐rater reliability of the instrument is important in order for the results from different examiners to be accurately interpreted. The aim of this study was to test inter‐rater reliability of the mHAMIS test. Method: Hand mobility for both hands was assessed in 25 female patients with SSc by 2 physiotherapists who have different years of experience. Patients who had flexion contracture in at least 1 finger and undergone hand surgery in the last year due to any injuries, were excluded from the study since hand mobility was prevented. Inter‐rater reliability was determined using intra‐class correlation coefficients (ICCs). Result: The ICCs were excellent between raters for dominant and non‐dominant hands. The values were 0.92 and 0.93, respectively. Conclusion: The inter‐rater reliability of the mHAMIS was found to be excellent. This research contributes to the literature by proving that the test can be used without causing bias in clinical trials. [ABSTRACT FROM AUTHOR]

Introduction/objectives: Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis, but some patients may experience long-term complications of disease such as infertility/subfertility. The published data about FMF-associated infertility is still limited. The aim of this study is to investigate the frequency and to determine potential factors for FMF-associated infertility/subfertility. Methods: We enrolled 971 adult patients with FMF. We defined infertility as the failure to conceive after 12 months of regular, unprotected intercourse. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics and genotype data (if available), disease complications, laboratory parameters, and treatment features were recorded. Results: There were 582 subjects eligible for the present study (mean age 41.05 ± 10.6 years, 65.8% female). MEFV mutations were available in 482 subjects, and 74.9% of subjects were harboring M694 V mutation (25.1% homozygous for M694 V). Infertility was present in 64 patients (14.6% of females and 4% of males). Multivariate analysis showed female sex [odds ratio (OR), 4.47; 95% confidence interval (CI95%) 1.75–11.42; p = 0.002], FMF disease onset < 20 years [OR, 2.99; (CI95% 1.04–8.61);p = 0.04], disease severity (ISSF) [OR, 4.81; (CI95% 2.28–10.17); p < 0.001], and colchicine nonresponse [OR, 2.80; (CI95% 1.17–6.74) p = 0.021] were the independent predictors of infertility. We also observed reversal of infertility in five patients who were treated with IL-1 β antagonists. Conclusion: Disease severity, FMF disease onset < 20 years, colchicine nonresponse, and female sex were found to be the independent predictors of infertility. The value of effective therapeutic interventions must be determined to treat infertility in these patients. Key Points •The prevalence of infertility increased in female patients with FMF. • Female sex, FMF disease onset < 20 years, disease severity, and colchicine nonresponse were risk factors for FMF-associated infertility. • With effective treatment of FMF, reversal of infertility was observed in five patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to assess the impact of Familial Mediterranean Fever (FMF) attacks on daily physical activity and detect FMF attacks using a connected activity tracker. Methods: Patients with FMF according to the Tel-Hashomer criteria were included in this prospective observational study. Attack-related data were collected weekly via phone call to avoid memory bias. Median steps in attack and attack-free days were calculated and compared using the Wilcoxon rank test. Sensitivity and specificity threshold for capturing attacks was set to two thirds of median steps per day in the whole observation period. Results: Twelve patients participated in the study. The median age of patients was 26 (18–32) years, and 7 (58.3%) of them were male. Patients with attacks (n = 10) walked a median of 6990 (4552–11,531) steps per day in attack-free days, whereas this number decreased to a median of 1841 (590–4783) steps in attack days (p = 0.005). The activity tracker captured 42 of 45 attack days and 312 of 361 attack-free days. The cutoff value had 93% sensitivity and 86% specificity for capturing attacks. Conclusions: FMF attacks significantly impair the physical activity of patients. Activity tracking may be a reasonable method to document FMF attacks. This might prevent errors due to memory bias and help accurately identify and treat patients with FMF. [ABSTRACT FROM AUTHOR]

Objectives: Takayasu arteritis and Spondyloarthritis are two distinct inflammatory diseases that affect the same age periods. Increasing number of reports on co-incident Takayasu arteritis-spondyloarthritis cases in literature raised the hypotheses about their association. The purpose of this study is to evaluate the incidence of spondyloarthropathy spectrum diseases in Takayasu arteritis patients.Methods: Detailed clinical and demographic features of Takayasu arteritis patients were recorded and all were screened meticulously for the presence of spondyloarthropathy features following recommendations of Assessment of SpondyloArthritis international Society. Patients were questioned for inflammatory back pain, enthesitis, uveitis, inflammatory bowel disease, peripheral arthritis, and investigated accordingly with HLA-B27, plain X-rays and sacroiliac magnetic resonance imaging.Results: A total of 69 Takayasu arteritis patients (65 female, 94.2%) were enrolled. After detailed investigation, 14 (20.3%) Takayasu arteritis patients fulfilled the Assessment of SpondyloArthritis international Society criteria for Spondyloarthropathy. Two of 14 (14.2%) spondyloarthropathy patients were positive for HLA-B27. Type 1 and type 2 Takayasu arteritis were more common in patients with diagnosis of both Takayasu arteritis and spondyloarthropathy than those without spondyloarthropathy. Most of patients with diagnosis of both these diseases required biologic therapies than patients with diagnosis of Takayasu arteritis alone (64.3% vs 29.1%, P = 0.014) due to refractory Takayasu arteritis.Conclusion: Our results suggest a significant association between Takayasu arteritis and spondyloarthropathy. Possible shared genetic or immunopathogenic processes may explain this association, which merits further investigations. [ABSTRACT FROM AUTHOR]

Background: Familial Mediterranean fever (FMF) patients suffer from chronic complications of disease such as AA amyloidosis, chronic arthritis, and spondylitis. Reduced quality of life (QoL) is a feature of chronic diseases but it is also impaired in patients with FMF. Despite the regular use of colchicine at a maximal dose, about 10% of patients do not respond well or resistant to colchicine (crFMF). IL-1 inhibitors have been shown to be effective in controlling attacks in crFMF patients. Herein, we aimed to investigate QoL changes of crFMF patients with IL-1 inhibitors.Methods: All patients were prospectively monitored for the frequency, duration, severity of attacks, patient global assessments (Visual Analog Scale; VAS), and laboratory features. Either anakinra or canakinumab was used as IL-1 antagonist treatments. Demographic information, MEFV gene mutations, attack characteristics, and previous treatments were registered. Short form-36 (SF-36) quality of life scale was implemented by the interviewer for evaluating the QoL before and 3 months after the treatment.Results: A total of 44 patients were included in this study. Striking improvements were detected in frequency, duration, and VAS severity of attacks (p < 0.001). In the comparison of pre- and post-treatment, SF-36 sub-components significant improvements were observed on physical function, role limitation due to physical difficulty, role limitation due to emotional problem, energy, emotional well-being, social function, pain, general health, and health change.Conclusions: In conclusion, IL-1 antagonists prevent attacks and improve QoL of crFMF. [ABSTRACT FROM AUTHOR]

Background: Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Colchicine is still the only approved drug for the prevention of amyloidosis in FMF and utility of IL-1 inhibitors in crFMF cases who already has amyloidosis remain to be elucidated. Herein, we evaluated efficacy and safety of IL-1 inhibitors in patients with crFMF-associated AA amyloidosis in a relatively large single center study. Methods: Medical records of FMF patients complicated with AA amyloidosis in our dedicated FMF center were retrospectively reviewed and those patients who ever treated with IL-1 inhibitors were enrolled into the study. Patient global, physician global assessments (on 0-10cm visual analog scale), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum creatinine and 24-h urinary protein excretion values for each visit were recruited from computer-based hospital records. Treatment response of patients were assessed with clinical symptoms, serum albumin, CRP and ESR values. Renal outcome parameters were analyzed on those not receiving renal replacement therapy. Results: Seventeen patients were identified with crFMF-amyloidosis that ever treated with IL-1 inhibitors. Background colchicine therapy was continued in all patients in maximal-tolerated dose along with IL-1 inhibitors. All patients benefit from IL-1 antagonists assessed by patient and physician global assessments. Inflammatory markers, CRP and ESR, were significantly reduced in all and normalized in 12 out of 17 patients. More importantly, the amount of proteinuria was remarkably improved following IL-1 inhibitor therapy (1606mg/day to 519 mg/day, p=.008). Both anakinra and canakinumab were well-tolerated without severe side effects. All patients were initially treated with anakinra but switched to canakinumab in seven patients (one leukopenia, four injection site reaction, two inefficacy). Conclusion: We evaluated the clinical and laboratory responses to IL-1 inhibitors in crFMF-associated amyloidosis patients. We found significant decreases in CRP, ESR and proteinuria after IL-1 inhibitor therapy. This study confirmed that IL-1 inhibitors are effective for controlling attacks and inflammatory activity in FMF patients complicated with AA amyloidosis. Moreover, they reduce or stabilize amount of proteinuria and preserve renal function in short-term follow-up. Prolonged prospective clinical trials are warranted to assess their long-term efficacy in this particular patient group. [ABSTRACT FROM AUTHOR]

To evaluate the efficacy of on-demand use of anakinra in patients with crFMF. The Gazi FMF cohort was established in the year 2010, and from that date, 689 patients with FMF diagnosed according to the Tel Hashomer criteria were registered. Attack type, duration, severity, and their impact on life were collected either by disease diaries or a mobile phone application (FMF AIDD, AppStore, and Playstore). A retrospective cohort analysis was made from records of patients who have ever been treated with IL-1 inhibitors. A total of 78 patients were treated with IL-1 inhibitors in our cohort. Among these, 15 patients were identified who received on-demand anakinra. Rationale for on-demand use was prominent prodrome or trigger for attacks and patient's personal claim. Six patients were switched from regular use and nine were directly started as on-demand use. All were using background colchicine in maximum tolerated doses. None of the patients had evidence of persistently elevated acute phase reactants or proteinuria. The median duration of on-demand anakinra use was 6 (3-36) months. Pre- and post- on-demand anakinra periods were compared. Patient reported attack severity (p = 0.002), duration (p = 0.001), frequency (p = 0.001), absenteeism (p = 0.002), and presenteeism (p = 0.002) were significantly improved. On-demand anakinra prevented progression of prodromes to full-blown attacks which was demonstrated by decrease in the rate of attack/prodrome ratio (p = 0.02). On-demand anakinra can be continued in ten subjects on long-term. On-demand anakinra significantly improved FMF attacks in certain patients which suggest this approach would be of benefit in daily practice in selected patients. [ABSTRACT FROM AUTHOR]

Avascular necrosis (AVN), also known as osteonecrosis, is characterized by death of the osteocytes due to inadequate blood supply caused by various mechanisms. The hip is the most common affected joint followed by knee. Incidence of AVN in rheumatic diseases is variable and high corticosteroid (CS) therapy is a known major risk factor for development of AVN. Data on the AVN in Behçet disease (BD) are limited. The purpose of this study is to examine the clinical and treatment characteristics of BD patients with diagnosis of AVN. Retrospective medical records of 337 BD patients were reviewed. Nine BD patients with AVN were detected. The clinical data of these patients with AVN have been reviewed. All patients had MRI of the symptomatic joints compatible with AVN. All of the nine patients who were diagnosed with AVN were male. Median duration of BD was 7 years. Median time between diagnosis of BD and detection of AVN was 3 years (1-16 years). Multiple joints were involved in seven patients. Six patients had bilateral knee AVN. Six patients had vascular BD. The median time interval between initial CS dose and AVN development was 24 months (range = 2-100). The median highest daily CS dose was 64 mg/day (range = 32-80) and median cumulative CS dose prior to AVN was 18 g. All of patients had intravenous pulse steroids. CS treatment, smoking and vascular involvement may predispose to AVN in patients with BD. According to this cohort, AVN in BD frequently tended to be in the knee joint and bilateral. [ABSTRACT FROM AUTHOR]

Background:Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis. Methods:Medical records of granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener’s Granulomatosis (BVAS/WG). Serum ferritin and other acute phase markers were measured at initial presentation. Results:Serum ferritin levels were found to be the highest in GPA patients with alveolar hemorrhage, median (IQR) 1041 (1281) μg/L. Patients with renal disease also had high levels of ferritin and it was correlated with concurrent glomerular filtration rate (r = −0.65,p < .001). Serum ferritin is also correlated well with the BVAS/WG scores (r = 0.79,p < .001). Conclusions:Measurement of serum ferritin might help in assessing disease activity of GPA. [ABSTRACT FROM AUTHOR]

LupusPRO is a disease-targeted patient-reported outcome measure that was developed and validated from and among US patients with systemic lupus erythematosus (SLE). We herein report the results of the cross-cultural adaptation and validation study of the Turkish translated version of the LupusPRO. Turkish LupusPRO and the Medical Outcomes Study Short Form (SF-36) (Turkish) were administered to the Turkish lupus patients. Disease activity was ascertained using the physician global assessment (PGA), Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI), and flare (defined by LFA-Lupus Foundation of America). Disease damage was assessed with Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI). Also, second Turkish LupusPRO tests were given to the patients to be completed within 2-3 days and sent back to us. Internal consistency reliability, test-retest reliability, and convergent and criterion validity (against disease activity or health status) were tested. All reported p values are two-tailed. The conceptual framework of the LupusPRO was evaluated using confirmatory factor analysis appropriate for categorical data. One hundred two SLE subjects (94 % women) were enrolled. The median (IQR) age and mean disease duration (±SD) were 38.5 (18) years and 60.3 (±56.3) months, respectively. The mean ± SD, SLEDAI, and SDI scores were 3.1 ± 3.7 and 0.52 ± 0.75, respectively. There were 25 patients who had flares at the time of study. Forty-two patients with no change in their health status completed and sent back the second LupusPRO test and were included in the test-retest analysis. Test-retest reliability of LupusPRO domains ranged from 0.87 to 0.97, while internal consistency reliability of the domains ranged from 0.63 to 0.94. Convergent validity with corresponding domains of SF-36 was present. Health-related quality-of-life domains performed well against disease activity measures (PGA, total SLEDAI, LFA flare, and SF-6D-overall health status), establishing its criterion validity. Item-to-factor loadings representing the hypothesized item-to-scale relationships were satisfactory. The model fit for the hypothesized item-to-scale relationships was also satisfactory. The Turkish version of the LupusPRO is valid and appears to perform comparably to the English and Spanish language versions. It can be used as a patient-reported outcome parameter in clinical trials, as well as longitudinal studies for testing responsiveness to change. [ABSTRACT FROM AUTHOR]

Giriş: Mezenterik arterit, bağırsak perforasyon, gastrik veya duodenal ülser, nekrotizan enterokolit, spontan peritonit, pankreatit ve karaciğer veya dalağın spontan rüptürü gibi çeşitli komplikasyonlar sistemik lupus eritematozuslu (SLE) hastalarda rapor edilmiştir. Ancak safra kesesi hastalığı birlikteliği ile ilgili veriler sınırlıdır. Biz de kliniğimizde takip ettiğimiz akalkülöz kolesistit olgu sını sunmak istiyoruz. Olgu Sunumu 1: Otuz beş yaş kadın hasta gebeliğinin 21. haftasında sağ üst kadran ağrısı ve transaminaz yüksekliği ile acil servise başvurdu. Yedi ve 8. haftalarda iki düşük öyküsü olup 17. gestasyonel haftadan beri derin ven trombozu nedeniyle asetilsalisilikasit + heparin kullanmaktaydı. Obstetrik patoloji saptanmayan hasta kolesistit tanısı alarak opere edildi. Operasyon materyali biyopsisi akalkülöz kolesistit ile uyumluydu. Cerrahi tarafından karaciğerde peteşiyal lezyonları görülen hasta otoantikor pozitifliği + mukokutanöz bulgular + aile öyküsü ile SLE + anti-fosfolipid antikor sendromu (APS) tanısı aldı. Sağlıklı doğum yapan hasta semptomsuz takip edilmektedir Olgu Sunumu 2: Acil servise karın ağrısı, ateş ile başvuran 54 yaş kadın hastanın pansitopeni olması nedeniyle servise kabul edildi. Özgeçmişinde SLE tanısı mevcuttu. Servis yatışı sırasında afazik olan hastanın sol parietotemporal bölgede akut enfarkt tespit edildi. Anti-fosfolipid antikorları pozitif saptandı. Transaminaz ve kolestaz enzimleri progresif artan, anti-agregan tedavi başlanan hastanın bakılan ultrasonografide kolesistit lehine kese duvar kalınlığında artış izlendi. Genel cerrahi birimince kese operasyonu gerekli görülmedi. Akalkülöz kolesitit tanısı alan hasta varfarin başlanarak taburcu edildi. Tartışma: SLE'de AAK'nin tek atak bulgusu olduğu, kompleman düşüşüne eşlik ettiğini gösteren birkaç pivotal yayın mevcuttur, ancak bu birlikteliğin patogenezi net olarak aydınlatılmamıştır. Önceki olgulardaki safra kesesi histolojik bulguları vaskülit, trombüs ve fibrinoid nekroz paternlerini ortaya çıkarmıştır. SLE nin alevlenmesi safra kesesinde arterit ve venülite yol açabilir. Pozitif anti-fosfolipid antikorları varlığı trombüs kaynaklı AAK'ye yol açabilir. Sonuç: AAK küçük arter oklüzyonu ve daha az oranda yetersiz venöz dolumla ilgilidir, bu da SLE de damar tutulumu ilişkili sıklığının artabileceğini düşündürmektedir. [ABSTRACT FROM AUTHOR]

Enthesopathy is pathology of bony insertions of tendons, ligaments or joint capsules. It is a frequent finding in rheumatic diseases, like ankylosing spondylitis (AS) and Behçet's disease. Musculoskeletal complaints are common in patients with familial Mediterranean fever (FMF), and these could be a clinical manifestation of enthesopathy. Hence, we investigated the possible association between FMF and enthesopathy. Fifty-six patients with FMF and 11 patients with FMF-associated spondyloarthropathy (FMFS) were enrolled. Forty-seven healthy individuals and 36 patients with AS formed the healthy and diseased control groups. Musculoskeletal complaints were meticulously questioned, and all patients underwent a detailed physical and ultrasonographic (US) examination of the lower limbs. US scorings of enthesopathy was performed according to the Glasgow Ultrasound Enthesitis Scoring System (GUESS). Demographic data, disease characteristics, MEFV genotypes and HLA B27 results were retrieved from the medical records. Patient-reported pain and physical examination findings consistent with enthesopathy were frequent in all groups with the highest prevalence in the FMFS group. Heel was the most common region affected in all patient groups. FMF patients harboring M694 V variant had higher GUESS scores compared to patients with other variants (2.78 ± 2.43 vs. 1.37 ± 1.67, p = 0.026). There was no statistically significant difference in the mean ± SD GUESS scores between healthy subjects and those FMF patients with genetic variants other than M694 V (1.38 ± 1.42 vs. 1.37 ± 1.67, p > 0.05). Enthesopathy may not be a feature of general FMF population; rather, it might be specifically associated with the presence of M694 V variant. Our results further support the previous evidence regarding M694 V mutation and spondyloarthropathy association. [ABSTRACT FROM AUTHOR]

Ulcerative colitis (UC) is an inflammatory disease of the colonic mucosa. The presence of gene responsible for FMF, MEFV, which frequently causes inflammation, may aggravate the clinical course of UC. We aimed to determine the prevalence of MEFV mutations in UC patients and its impact on the clinical course. Four groups were formed as group 1 UC with distal disease, group 2 UC with pancolonic disease, group 3 UC with total colectomy, and group 4 Rheumatoid Arthritis (RA) patients. Eleven mutations of FMF gene were investigated. The mean age of group 1, 2, 3, and 4 were 46.7 ± 13.9, 43.8 ± 12.9, 44.8 ± 14.2, and 45.8 ± 10.9 years, respectively. The mutations were identified in 19 of the 54 UC patients (35.2%). Homozygous E148Q in 2 patients (3.7%) and heterozygous in 17 patients (31.5%) (E148Q 11.1%, M694V 5.6%, V726A 5.6%, K695R 1.8%, M680I 1.8%, and compound heterozygous 5.6%) were determined. Frequencies of MEFV mutations in group 1, 2, and 3 were 30, 27.3, and 58.3%, respectively. The mutations were identified in 3 of the 20 RA patients (15%). All of them were heterozygous. The rate of MEFV mutations were higher in group 3 than in group 4 ( P = 0.018), and the number of attacks that were treated with steroid in all UC patients with mutation positive was higher than in mutation negative ( P = 0.016). FMF gene mutations may be identified in UC patients up to 58.3%. It may be suggested that the UC patients with severe form should be identified for MEFV mutations before the judgment of colectomy. [ABSTRACT FROM AUTHOR]

The aim of the present study was to investigate the health-related quality of life (HRQOL) and mood conditions in familial Mediterranean fever (FMF) patients. Ninety FMF patients (F/M 60/30, median age 29) and 67 control subjects (F/M 46/21, median age 30) were included in this study. HRQOL was assessed with short form-36 (SF-36) and mood conditions were assessed with hospital anxiety depression scale (HADS). FMF patients had significantly lower mean scores on SF-36 physical components compared to the control group. However, mental components were comparable between groups. FMF patients were significantly more likely to have depression and anxiety compared to the control group [30 (33%) vs. 8 (12%), respectively, χ = 9.58, OR (95% CI) = 3.7 (1.5-8.7), p < 0.01 for depression and 48 (53%) and 11 (16%), respectively, χ = 22.31, OR (95% CI) = 5.8 (2.7-12.5), p < 0.001 for anxiety]. When frequency of anxious subjects was adjusted for the presence of concomitant depressive status as a confounding factor, the difference between the groups remained statistically significant [ χ = 11.86, OR (95% CI) = 5.4 (2.1-13.7), p < 0.01]. However, the difference of depression status between groups was not statistically significant when adjusted for the presence of concomitant anxiety status [ χ = 0.08, OR (95% CI) = 1.3 (0.5-3.8), p = 0.78] and FMF was found to be independently associated with only anxiety [OR (95% CI) = 7.1 (2.3-20.3)]. In addition, pure anxious FMF subgroup had significantly lower scores of mental health and mental component summary when compared to normal mood subgroup. In conclusion, FMF might adversely affect HRQOL. Depression and anxiety are more frequent in FMF patients than healthy subjects. [ABSTRACT FROM AUTHOR]

We investigated the association between familial Mediterranean fever (FMF) and osteoporosis (OP) in adult patients. Thirty-five attack-free FMF patients (28 females, 7 males; mean age 36.9 ± 5.7 years) were individually matched to control subjects on the basis of age (within 2 years) and sex. All patients were taking regular colchicine. Subjects having any condition that can cause decreased bone mineral density (BMD) were excluded from the study. BMD was measured at the spine and femur by dual X-ray absorptiometry (DXA). Data was given as the median (IQR). T scores of the spine were −0.700 (−1.097 to −0.262) and −0.450 (−0.830 to 0.112) in FMF patients and healthy controls, respectively ( p > 0.05). T scores of the femur neck were −0.900 (−1.480 to −0.570) and −0.430 (−1.472 to 0.247) in FMF patients and healthy controls, respectively ( p > 0.05). Total femur T scores were significantly lower in FMF patients than healthy controls (−0.780 [−1.222 to −0.085] vs. −0.100 [−0.765 to 0.537], respectively, p = 0.021). Total femur T scores were significantly decreased in adult patients with FMF. Ongoing subclinical inflammation may be associated with decreased bone mineral content in those patients. [ABSTRACT FROM AUTHOR]

There is morphologic evidence of subclinical atherosclerosis in Behçet’s disease (BD) patients obtained by high-resolution B-mode ultrasonography (US). Vascular endothelial growth factor (VEGF) is a potent angiogenetic factor and a marker for endothelial dysfunction. VEGF could contribute to the pathological events in BD. VEGF could also be an important factor in the progression of atherosclerosis. In this study, we investigated whether there is correlation between intima-media thickness (IMT) of the carotid arteries and serum VEGF levels in BD patients and healthy controls. Twenty-one patients with BD (male/female: 15/6, mean age: 35.8 ± 8.6 years) were individually matched to control subjects on the basis of age (within 3 years) and sex. Carotid IMT of the subjects was measured by high-resolution B-mode US. Mean IMT values of common carotid arteries were 0.86 ± 0.18 mm for patients with BD, and 0.57 ± 0.14 mm for healthy controls ( p < 0.001). Mean VEGF levels were 130.41 ± 58.28 pg/ml for patients with BD and 82.69 ± 25.03 pg/ml for healthy controls ( p < 0.001). There was no correlation between VEGF levels in the control group and in the BD group, but there was a significant correlation between VEGF levels and mean carotid IMT in the whole group ( r = 0.317, p < 0.05). In conclusion, elevation of VEGF appears as a feature of the inflammatory reaction during the course of BD, not a direct determinant of subclinical atherosclerosis. On the other hand, the significant correlation between carotid IMT and serum VEGF levels in the whole group suggests that association between VEGF levels and carotid IMT warrants further investigation with larger sample sizes. [ABSTRACT FROM AUTHOR]

Subclinical atherosclerosis can be demonstrated by measuring the intima-media thickness (IMT) of the carotid arteries by high-resolution B-mode ultrasonography (US). Endothelial injury appears a key event in the atherogenesis. Endothelial cell activation and/or injury are the characteristic features of Behçet’s disease (BD). In this study, we investigated morphologic evidence of subclinical atherosclerosis in the BD patients by using high-resolution B-mode US. Thirty-four patients with BD without arterial involvement (male/female 21/13; mean age 34.6±8.5 years) were individually matched to control subjects on the basis of age (within 2 years) and sex. Subjects with diabetes mellitus, hypertension, evidence of myocardial infarction or cerebrovascular disease, and patients on long-term steroids (i.e., >6 months) were excluded from the study. Mean IMT values of the right carotid arteries were 0.81±0.17 mm for patients with BD, and 0.54±0.13 mm for healthy controls ( P<0.001). Mean IMT values of the left carotid arteries were 0.82±0.16 mm for patients with BD, and 0.55±0.12 mm for healthy controls ( P<0.001). The overall prevalence of carotid atherosclerotic plaques was higher among the patients than the controls [prevalence of plaques were 17.6% (6/34) in BD patients and 0% in healthy controls, P<0.05]. In conclusion, our data indicate morphologic evidence of subclinical atherosclerosis in patients with BD. [ABSTRACT FROM AUTHOR]

Highlights: [•] Antimicrobial peptides may initiate an inflammatory response. [•] Antimicrobial peptides are involved in the pathogenesis of many inflammatory disorders. [•] Antimicrobial peptides, LL37 and alpha defensin are active in familial Mediterranean fever. [ABSTRACT FROM AUTHOR]

Background. Systemic amyloidosis is a potentially fatal condition, unless diagnosed and treated before development of irreversible organ damage. Demonstration of amyloid deposits within tissue biopsies is only definitive diagnostic method, which makes appropriate selection of biopsy site essential. Herein, we evaluated efficacy of minimally invasive minor salivary gland biopsy (MSGB) for the diagnosis of amyloidosis. Methods. We analyzed 37 biopsies taken from 35 patients. Suggestive findings for amyloidosis were significant proteinuria, renal impairment, refractory diarrhea, neuropathy, and restrictive cardiomyopathy. Minor salivary gland was the initial biopsy site in all subjects. When MSGB was negative but there was a high suspicion for amyloidosis, a kidney, duodenum, or rectal biopsy was performed for further investigation. Results. Mean age of patients was 45.4 and 21 were female. In 11 patients amyloidosis was diagnosed with MSGB. In overall 18 patients were diagnosed with amyloidosis. Sixteen of them were identified as being of AA type and two were AL type amyloidosis. The sensitivity of minimally invasive MSGB is 61.1% for diagnosing amyloidosis in this study. Conclusion. MSGB is a safe and simple method for the diagnosis of amyloidosis which can be performed in an outpatient setting. We suggest extensive use of this minimally invasive method. [ABSTRACT FROM AUTHOR]

Background. Systemic amyloidosis is a potentially fatal condition, unless diagnosed and treated before development of irreversible organ damage. Demonstration of amyloid deposits within tissue biopsies is only definitive diagnostic method, which makes appropriate selection of biopsy site essential. Herein, we evaluated efficacy of minimally invasive minor salivary gland biopsy (MSGB) for the diagnosis of amyloidosis. Methods. We analyzed 37 biopsies taken from 35 patients. Suggestive findings for amyloidosis were significant proteinuria, renal impairment, refractory diarrhea, neuropathy, and restrictive cardiomyopathy. Minor salivary gland was the initial biopsy site in all subjects. When MSGB was negative but there was a high suspicion for amyloidosis, a kidney, duodenum, or rectal biopsy was performed for further investigation. Results. Mean age of patients was 45.4 and 21 were female. In 11 patients amyloidosis was diagnosed with MSGB. In overall 18 patients were diagnosed with amyloidosis. Sixteen of them were identified as being of AA type and two were AL type amyloidosis. The sensitivity of minimally invasive MSGB is 61.1% for diagnosing amyloidosis in this study. Conclusion. MSGB is a safe and simple method for the diagnosis of amyloidosis which can be performed in an outpatient setting. We suggest extensive use of this minimally invasive method. [ABSTRACT FROM AUTHOR]

Articular attack is a common feature of familial Mediterranean fever (FMF). FMF arthritis commonly resolves without any sequale within a few weeks. However, approximately 10% of the patients develop protracted arthritis persisting for months to years. Treatment with colchicine may not be effective and nonsteroidal antiinflammatory drugs or second line agents may be needed for the management of protracted arthritis. In this paper, we describe a 22-year-old patient with FMF who was complicated with protracted arthritis in the knee and shoulder joints and bilateral sacroiliitis. He was successfully treated by intramuscular gold 50 mg weekly. However, gold treatment was discontinued 8 months later because of the development of asymptomatic proteinuria. In conclusion, FMF should be considered in the evaluation of peripheric oligoarthritis, particularly in patients with Mediterranean origin. Intramuscular gold might be an effective agent. However, care should be taken regarding the development of proteinuria. [ABSTRACT FROM AUTHOR]

Protracted febrile myalgia syndrome (PFMS) is a very rare but severe manifestation of familial Mediterranean fever (FMF) which is characterized by severe debilitating pain in large muscle groups that may last for several weeks. Colchicine is ineffective and treatment is largely supportive. Demonstration of crucial role of interleukin-1 (IL-1) in the pathogenesis of FMF has increased the use of IL-1 blockers in colchicine resistant or intolerant patients. Herein, we reported successful use of an IL-1 inhibitor, anakinra, in treatment of two patients with PFMS. [ABSTRACT FROM AUTHOR]