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Rheumatic diseases cause deformities in the hands and affect daily living activities. Therefore, assessment of hand disabilities is important in rheumatic disease. The aim of this study was to test the validity and reliability of the Turkish version of the A Score For Assessment and Quantification of Chronic Rheumatic Affections of the Hands (SACRAH). A translation and back-translation of the SACRAH were performed, according to the Beaton guidelines. Patients who were between 18-65 years old, who were literate in Turkish, who had rheumatic disease diagnosis and whose hands were affected, were included in the study. Patients who were using a splint during daytime were excluded from the study. They completed the Turkish version of Disabilities of the Arm, Shoulder and Hand Questionnaire (DASH-T) once and the final version of the SACRAH Questionnaire twice with a 7 days’ interval. The internal consistency (Cronbach’s α) and reliability (test-retest reliability) of the questionnaire were assessed. Besides, correlations between SACRAH and DASH-T scores were analyzed using the Spearman correlation coefficient. One hundred and twenty patients participated in the study. The Turkish version of the SACRAH met set criteria of reliability and validity. Internal consistency was excellent (Cronbach’s α=0.88) and test-retest reliability were very good (r=0.73). SACRAH showed a positive and statistically significant correlation with DASH-T scores (r=0.83, p

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Purpose: Sleep disturbance is among the most important geriatric syndromes, and its evaluation is part of the routine comprehensive geriatric assessment (CGA). Previous studies have demonstrated that older patients with rheumatoid arthritis (RA) have poorer sleep quality than younger control patients. However, there needs to be more data on the sleep quality of older patients with RA with age-matched controls., Methods: Totally 100 participants, 50 older RA patients classified according to the ACR criteria, and 50 age- and gender-matched control patients without RA were included in the study. All patients underwent CGA, including assessing their functionality, depressive, cognitive, and nutritional status. In addition, sleep quality was assessed by the Pittsburg Sleep Quality Index (PSQI), and RA disease activity by a rheumatologist using the Disease Activity Score 28 (DAS28-CRP), and quality of life with the RA QoL questionnaire (RAQoL)., Results: The median age was 70 years (min-max: 65-86), and 62.5% were female. Co-morbidities and comprehensive geriatric assessment parameters were similar between the two groups. Median PSQI global score was higher in patients with RA than controls [9 (min-max: 1-20) vs. 5 (min-max: 1-13), p = 0.029). When the patients were categorized with respect to being 'poor sleepers' (PSQI score > 5), 62% of patients with RA and 38% of controls were poor sleepers (p = 0.016). The patients classified as poor sleepers were more likely to have a diagnosis of RA, higher DAS28-CRP and RAQoL scores, lower grip strength, and be a woman. PSQI global scores were significantly positively correlated with DAS28-CRP scores (r = 0.514, p < 0.001), RAQoL scores (r = 0.689, p < 0.001), number of medications used (r = 0.292, p = 0.003), and YDS scores (r = 0.407, p < 0.001), and significantly negatively correlated with handgrip strength (r =  - 0.351, p = 0.001)., Conclusion: The results suggest that older patients with RA might have poorer sleep quality compared to age- and gender-matched controls. Moreover, sleep quality correlated with RA disease activity and QoL in old age., (© 2023. The Author(s), under exclusive licence to European Geriatric Medicine Society.)

Competing Interests: The authors declare no conflict of interest.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc., Methods: Forty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed., Results: Twenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34)., Conclusions: Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease., Trial Registration: This study is not a clinical trial study.

Objective : P r odrome is defined by manifestations that precede a familial Mediterranean fever (FMF) attack and predict its emergence. We aimed to determine the frequency, characteristics, and clinical determinants of prodrome in patients with FMF. Method : This cross-sectional study was conducted in a tertiary rheumatology clinic. During the clinical interview, all patients completed a standardized questionnaire about the pre-attack period. Prodrome was defined as the presence of any recurrent pre-attack manifestation occurring at least 4 h before an attack. Patients were classified according to whether they had prodrome of any kind of attack. Results : The study enrolled 401 patients aged 37.7 ± 11.0 years (mean ± sd). Male gender, M694V / M694V , homozygous MEFV mutation, peritonitis, pleuritis, and arthritis were more frequent in prodrome-positive patients. Altogether, 141 patients (35.2%) had prodrome. Male gender and ever having attack types of peritonitis or arthritis were independent clinical determinants of prodrome [relative risk (95% confidence interval): 1.72 (1.07-2.76), p = 0.02; 4.27 (1.80-10.1), p = 0.001; 1.77 (1.04-3.04), p = 0.04, respectively]. Age, MEFV mutations, pleuritis, and erysipelas-like erythema were not clinical determinants. Conclusions : All FMF patients, particularly males and patients who had peritonitis or arthritis at any time, should be questioned about prodrome. Prodrome should be analysed in terms of elucidating the pathogenesis of FMF and as an opportunity for a secondary prevention strategy for impending attacks. This study may shed light on prodrome for future cytokine or drug studies with the purpose of developing new cost-effective treatment protocols irrespective of colchicine resistance.

Objective: We aimed to present our single-center real-life experience of canakinumab use in adult patients with familial Mediterranean fever (FMF)., Methods: Data were derived from the Gazi FMF cohort, which was established in 2010. From that year, all patients with FMF were registered. The impact of FMF on their lives was tracked by either an FMF diary or mobile phone application (FMF-AIDD, free to download in App Store and Google Play). The records of patients who were treated with canakinumab were reviewed., Results: Twenty-three adult patients with FMF (65% female) were enrolled in this study. The median age was 32 years (min-max, 24-58 years), and the disease duration was 26 years (14-59 years). A total of 86% of patients harbored homozygous or compound heterozygous exon 10 MEFV mutations. Indications for interleukin 1 inhibitor use were colchicine resistance (n = 12) or intolerance (n = 2), amyloidosis (n = 7), and chronic manifestations of the disease (n = 2). All patients used anakinra before. The median duration of canakinumab use was 7 months (min-max, 1-44 months). Pre- and post-canakinumab periods were compared. Attack severity, duration, frequency, C-reactive protein level, and erythrocyte sedimentation rate were significantly improved (p ≤ 0.01), whereas serum creatinine and alanine aminotransferase levels remained the same (p = 0.27 and p = 0.74, respectively). Canakinumab achieved complete disease remission in 14 patients (60%). Canakinumab was discontinued in 7 patients. The reasons for discontinuation were pregnancy (n = 2), dominance of axial spondyloarthropathy (n = 2), inflammatory bowel disease, patient's refusal, and weight gain., Conclusions: Canakinumab is effective in the prevention of FMF bouts without severe adverse effects. Treatment with canakinumab in an individualized dose and interval may be a reasonable choice for colchicine- and anakinra-resistant or -intolerant adult patients with FMF and those with chronic manifestations of disease.

Background: Granulomatosis with polyangiitis (GPA) is a granulomatous necrotizing vasculitis with high morbidity and mortality. Anti-neutrophil cytoplasmic antibody is a valuable diagnostic marker, however its titer lacks predictive value for the severity of organ involvement. Platelet to lymphocyte ratio (PLR) and mean platelet volume (MPV) has been regarded as a potential marker in assessing systemic inflammation. We aimed to explore the value of PLR and MPV in the assessment of disease activity and manifestations of disease in GPA., Methods: 56 newly diagnosed GPA patients and 53 age-sex matched healthy controls were included in this retrospective and cross-sectional study with comparative group. Complete blood count was performed with Backman Coulter automatic analyzer, erythrocyte sedimentation rate (ESR) with Westergen method and C-reactive protein (CRP) levels with nephelometry. The PLR was calculated as the ratio of platelet and lymphocyte counts., Result: Compared to control group, ESR, CRP and PLR were significantly higher and MPV significantly lower in GPA patients. In patients group, PLR was positively correlated with ESR and CRP (r = 0.39, p = 0.005 and r = 0.51, p < 0.001, respectively). MPV was negatively correlated with ESR and CRP (r = - 0.31, p = 0.028 and r = - 0.34 p = 0.014, respectively). Patients with renal involvement had significantly higher PLR than patients without renal involvement (median:265.98, IQR:208.79 vs median:180.34 IQR:129.37, p = 0.02). PLR was negatively correlated with glomerular filtration rate (r = - 0.27, p = 0.009). A cut-off level of 204 for PLR had 65.6% sensitivity and 62.5 specificity to predict renal involvement., Conclusion: PLR exhibit favorable diagnostic performance in predicting renal involvement in patients with GPA.

Rheumatic diseases cause deformities in the hands and affect daily living activities. Therefore, assessment of hand disabilities is important in rheumatic disease. The aim of this study was to test the validity and reliability of the Turkish version of the A Score For Assessment and Quantification of Chronic Rheumatic Affections of the Hands (SACRAH). A translation and back-translation of the SACRAH were performed, according to the Beaton guidelines. Patients who were between 18-65 years old, who were literate in Turkish, who had rheumatic disease diagnosis and whose hands were affected, were included in the study. Patients who were using a splint during daytime were excluded from the study. They completed the Turkish version of Disabilities of the Arm, Shoulder and Hand Questionnaire (DASH-T) once and the final version of the SACRAH Questionnaire twice with a 7 days' interval. The internal consistency (Cronbach's α) and reliability (test-retest reliability) of the questionnaire were assessed. Besides, correlations between SACRAH and DASH-T scores were analyzed using the Spearman correlation coefficient. One hundred and twenty patients participated in the study. The Turkish version of the SACRAH met set criteria of reliability and validity. Internal consistency was excellent (Cronbach's α=0.88) and test-retest reliability were very good (r=0.73). SACRAH showed a positive and statistically significant correlation with DASH-T scores (r=0.83, p<0.001). Our results show that the Turkish version of the SACRAH has excellent test-retest reliability and validity. As a result of this study we determined that SACRAH is a valid and reliable instrument for assessing functional status and subjective manual function in Turkish-speaking patients.

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.

Background : Chronic inflammation, as determined by persistently elevated acute-phase reactants in attack-free periods, can occasionally be observed in patients with familial Mediterranean fever (FMF) and is suggested to be a risk factor for the development of amyloidosis. We aimed to investigate the underlying causes of chronic inflammation in FMF patients and its association with amyloidosis in long-term follow-up. Method : Electronic medical records of FMF patients who had regular follow-up for ≥ 5 years in our cohort were utilized. As part of routine evaluation, detailed history, physical examination, and pertinent laboratory and radiographic investigations were performed in all patients to determine potential causes of elevated C-reactive protein (CRP) levels. Results : The study included 146 FMF patients who had no evidence of amyloidosis at baseline and had regular follow-up for ≥ 5 years. Thirty-seven patients (25.3%) were found to have chronic inflammation in the disease course. Twenty-five (67.5%) of them had either very frequent attacks or chronic manifestations of disease. In the entire study group, amyloidosis developed in five patients (3.42%) during the 5 year follow-up, four in the FMF with chronic inflammation group (10.8%), and only one of the 109 patients without chronic inflammation (odds ratio 13.09, 95% confidence interval 1.41-121.2). Conclusions : The results suggest that persistently high CRP levels during the attack-free periods may be a strong risk factor for the development of amyloidosis in patients with FMF. The vast majority of FMF patients with chronic inflammation had active FMF.

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.

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.

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.

Importance: Systemic lupus erythematosus (SLE) is a thoroughly examined multi-organ disease whose common ocular complications are also well documented. However, SLE choroidopathy, being a rare ocular complication, is needed to be better understood, in order to make the differential diagnosis from clinical conditions such as central serous chorioretinopathy and provide adequate prompt treatment., Objective: To provide a clear understanding for the clinical course of SLE choroidopathy by the help of enhanced depth imaging optical coherence tomography, fluorescein angiography, and indocyanine angiography., Design: The study is designed as a case presentation, consisting of the physical examination results and the data gathered by the relevant screening methods for each visit, applied by the same ophthalmologists and technicians., Setting: The visits were organized as same day examinations as needed, in a university hospital which works as a referral center.

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.