A hand exercise mobile app for people with rheumatoid arthritis in Turkey: design, development and usability study.

To design and develop a smartphone application for a structured hand exercise programme for patients with rheumatoid arthritis (RA) in Turkey and to test its usability. We followed a two-stage process: (1) Design and Development and (2) Usability testing. In stage 1, we used a qualitative user-centered design approach. We conducted a focus group (8 therapists and people with RA) to discuss the content, features and design to produce a prototype of the application. In a second focus group session, the participants tested the prototype, provided feedback and further revisions were made. In stage 2, 17 participants with RA used the app for 4 to 6 weeks. The System Usability Scale and the adapted Usability, Satisfaction and Ease to Use Questionnaires were used to measure usability, ease of use. Semi-structured interviews were conducted to explore user experiences with the application with 17 participants. In stage 1, the following themes were identified from the focus groups (a) login techniques (b) self-monitoring (c) exercises, (d) exercise diary, (e) information, (f) behavioral change and encouragement (g) exercise adherence. In stage 2, 3 themes were determined from interviews: (a) learning and accuracy, (b) ease of use, (c) motivation and adherence. USE and SUS scores indicated that users reported a high level of usability, satisfaction and ease of use. A mobile app for hand exercise for people with RA was developed using a mixed-method and iterative design. Participants perceived the mobile app as easy to use with high levels of satisfaction.

The epidemiology of dermatomyositis in northwestern Thrace region in Turkey: epidemiology of dermatomyositis in Turkey.

Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence in Turkey. Patients diagnosed with DM at the Trakya University Medical Faculty, Department of Rheumatology from November 2004 to November 2014 were reviewed retrospectively. Patients' clinical and demographic features, laboratory data, treatment modalities, follow-up durations, disease courses, outcomes, and complications were evaluated. Our study included 23 patients with DM; 14 were females and 9 were males (female/male: 1.55). Over the course of the study, the annual incidence of DM was 3.7 per million (95% CI 0-18.8) person years, and the overall prevalence was 32.2 per million (95% CI 18.1-46.3). Incidence in women was higher (4.6/1,000,000 person years) compared to men (2.9/1,000,000 person years). The frequencies of most common findings were as follows: heliotrope rash (82.6%), Gottron papules (87%), proximal myopathy (78.3%), and facial erythema (60.9%). In our hospital-based study, the frequency of DM was lower than those reported in North America; however, they were similar to European countries.

The epidemiology of Takayasu arteritis: a hospital-based study from northwestern part of Turkey.

Takayasu arteritis (TA) is a chronic, inflammatory large vessel vasculitis that affects aorta and its main branches. We aimed to evaluate the incidence and prevalence of TA in the northwestern part of Turkey. We retrospectively evaluated 23 TA patients followed by our clinic. Clinical features, treatments and responses were recorded. Our hospital is the single tertiary referral center for rheumatic diseases for a mixed rural and urban population of 620,447 people for >16 years (306,036 males, 314,411 females). Nineteen of the 23 patients were females (82.6 %) and four were males (17.4 %). The annual incidence rate for TA was 0.34/100,000. The overall prevalence of TA in our region was 3.3/100,000 (95 % CI 1.9-4.8) in individuals >16 years. The most common findings at the time of presentation were blood pressure difference (73 %) and headache (60.4 %). The most common angiographic type was type 1 (12 patients, 52.2 %). Median follow-up period was 48 months (range 10-132). Three (13 %) of the patients had stent replacements to different vascular sites. One patient had an operation for aortic aneurysm, and aortic valve replacement surgery has been made. One patient had renal artery bypass operation. Eleven patients (47.8 %) had recurrency at follow-up period and two patients (8.7 %) died. In northwestern part of Turkey, the annual incidence and prevalence of TA were higher than western population, but similar to East Asian data.

Investigation of factors affecting physical activity level in patients with primary Sjögren's syndrome.

OBJECTIVES: This study aimed to determine physical activity levels and understand the factors influencing an active lifestyle among patients with primary Sjögren's syndrome (pSS). METHODS: Ninety-seven patients participated in this multicentric study. Physical activity levels were assessed using the International Physical Activity Questionnaire-Short Form (IPAQ-SF). The Inflammatory Arthritis Facilitators and Barriers (IFAB) questionnaire was used to evaluate perceived barriers and facilitators to physical activity. RESULTS: Forty-six patients were physically inactive and the rest of them were moderately active. Commonly identified barriers included a lack of motivation, fatigue, and pain. Conversely, knowledge of the health and mood benefits for physical activity emerged as a key motivator. Patients with better scores on facilitators and lower scores on barriers exhibited higher physical activity levels (p < 0.05). Notably, a high level of perceived facilitators of physical activity (odds ratio [OR]: 1.02; 95% confidence interval [CI], 1.00 ­ 1.05) and reduced pain (OR: 0.81; 95% CI: 0.69 ­ 0.95) were linked to an active lifestyle. CONCLUSIONS: This study emphasizes the role of motivation and awareness of the benefits of physical activity for health and mood in driving physical activity for patients with primary Sjögren's syndrome. Tailored physical activity programs that address psychological aspects and disease-related pain, and fatigue should be designed to counter sedentary lifestyles in pSS patients.

Inflammatory Arthritis Facilitators and Barriers (IFAB) for physical activity questionnaire: cross-cultural adaptation into Turkish and evaluation of its psychometric properties.

PURPOSE: We aimed to conduct the translation and cross-cultural adaptation of the Inflammatory arthritis Facilitators and Barriers (IFAB) questionnaire into Turkish language for patients with axial spondyloarthritis (axSpA) and analyze the psychometric properties of the IFAB-Turkish version (IFAB-Tr). METHODS: Data from ninety-three patients with axSpA were analyzed who completed the IFAB-Tr, Health Assessment Questionnaire (HAQ), Hospital Anxiety and Depression Scale (HADS) and Bath Ankylosing Spondylitis Functional Index (BASFI). Internal consistency, test-retest reliability, convergent validity, floor and ceiling effect, measurement error were examined. Exploratory factor analysis (EFA) and confirmatory factor analysis were conducted. RESULTS: Small modification was needed in the translation and cultural adaptation process. Internal consistency of the IFAB-Tr total score was 0.71 and test-retest reliability was excellent for IFAB-Tr (ICC = 0.90). A moderate negative correlation was found between IFAB-Tr total score and HAQ, HADS, BASFI scores. No floor and ceiling effect was detected. Minimal detectable change was 10.34 points. EFA revealed two factors which accounted for 55% of the variance. CONCLUSIONS: The IFAB was successfully translated into the Turkish language and seems suitable for evaluating barriers and facilitators for physical activity in rheumatic diseases and could be used in clinical settings before designing a physical activity intervention.IMPLICATIONS FOR REHABILITATIONThe Turkish version of the Inflammatory arthritis FAcilitators and Barriers (IFAB) is a valid and reliable tool to assess barriers and facilitators to physical activity for patients with axial spondyloarthritis.More work is needed to assess all psychometric properties of the IFAB questionnaire in other inflammatory rheumatic disorders.The Inflammatory arthritis FAcilitators and Barriers questionnaire in Turkish is an easy and quick way to determine potential barriers to physical activity in clinical and research settings.

Identifying frailty and its associated factors in patients with axial spondyloarthritis.

BACKGROUND AND AIM: Frailty is an elderly people phenomenon; however, younger adults with comorbidities may show a higher trajectory of frailty toward older age. The frailty status of middle-aged patients with axial spondyloarthritis (axSpA) remains little researched. We aimed to identify frailty status and to investigate the association of clinical, sociodemographic, and psychosocial factors with frailty. METHODS: This study included 114 patients aged between 40 and 65 years. The patients were classified as frail, pre-frail, and robust according to Fried's phenotype and the Kihon Checklist (KCL). Physical function, quality of life, depression, anxiety, and disease-related factors were assessed. Between-group comparisons and multinomial logistic regression analysis were performed. RESULTS: Frailty and pre-frailty prevalences were 20.2% and 49.1% for Fried's phenotype and 36.0% and 33.3% for the KCL. Frail adults had impaired physical function and increased disease activity compared to pre-frail and robust patients. Adjusted multinomial logistic regression analysis revealed that disease activity (odds ratio [OR] = 1.62, 95% CI = 1.12-2.34) and Short Physical Performance Battery (OR = 0.32, 95% CI = 0.18-0.56) were associated with Fried's phenotype-determined frailty. Disease activity (OR = 1.91, 95% CI = 1.11-3.26), Bath Ankylosing Spondylitis Functional Index (OR = 2.70, 95% CI = 1.56-4.67), and depression (OR = 1.55, 95% CI = 1.18-2.02) were associated with the KCL determined frailty. CONCLUSION: Frailty and pre-frailty are commonly detected in middle-aged individuals with axSpA. Our findings highlight a possible need for frailty screening and preventive programs targeting middle-aged axSpA patients.

Pre-sarcopenia is associated with health-related quality of life in patients with primary Sjögren's syndrome.

OBJECTIVES: Primary Sjögren's syndrome leads to pain and fatigue that may cause impaired muscle function and muscle mass. This study aimed to determine the presence of pre-sarcopenia and to clarify associated factors with pre-sarcopenia in primary Sjögren's syndrome patients. METHOD: This cross-sectional study recruited 49 patients with primary Sjögren's syndrome and 49 age- and gender-matched healthy controls. Sarcopenia was assessed according to European Working Group on Sarcopenia in Older People in 2018 (EGSWOP2) criteria by evaluating muscle mass, gait speed, and grip strength. Physical function was also assessed with five times sit-to-stand (FTSTS) test. Quality of life (QoL), psychological symptoms, and nutritional status were evaluated using Sarcopenia Quality of Life (SarQoL) questionnaire, Hospital Anxiety and Depression Scale (HADS), and Mini Nutritional Assessment Long Form (MNA-LF), respectively. RESULTS: Pre-sarcopenia was detected in 28.5% of patients with primary Sjögren's syndrome and 6.1% of healthy controls (p = 0.006). Patients with pre-sarcopenia were older and had worse scores in terms of depression, QoL, pain scores, grip strength, and gait speed compared with those without pre-sarcopenia. The SarQoL scores and grip strength were significantly correlated with gait speed, pain, FTSTS test, and depression. Logistic regression analyses identified that the independent predictive factor for pre-sarcopenia was QoL (OR: 0.79, 95% CI: 0.65-0.95; p = 0.015). CONCLUSIONS: Maintaining adequate muscle mass and muscle function in patients with primary Sjögren's syndrome may contribute toward improving health-related QoL. Also, controlling depression and malnutrition risk may help to reduce the risk of development of sarcopenia. Key Points • Pre-sarcopenia is detected in 28.4% of the patients with primary Sjögren's syndrome which is higher than healthy controls. • Pre-sarcopenia is associated with health-related quality of life in primary Sjögren's syndrome. • Psychological symptoms and low physical performance are more common in pre-sarcopenic patients.

Alterations in right ventricular mechanics in patients with Behcet's disease.

Manifest myocardial involvement is somewhat rare in patients with Behcet's disease (BD), although echocardiographic studies suggest that subclinical alterations in left ventricular (LV) contractility is rather common. Data on right ventricular (RV) involvement in BD is rather scarce. This study aims to determine whether RV systolic performance is affected in BD patients, and to understand the clinical and echocardiographic correlates of RV contractility in these patients. Forty-five patients who fulfilled criteria for BD and 45 age and gender matched controls were enrolled. All participants underwent a comprehensive echocardiographic examination, including deformation imaging, to characterize RV mechanics. Conventional morphologic and echocardiographic indicators of RV morphology and function were not different between groups, but RV apical strain and RV free wall strain (FWS) were significantly lower in BD patients as compared to the controls (P < 0.001 and P = 0.02, respectively). The only significant correlates of FWS were tricuspid regurgitation velocity and related indices in healthy controls, while FWS correlated with LV global longitudinal strain (GLS), morphologic measures of left and right atria and ventricles, and with conventional measures of right ventricular contractility. The relationship between FWS and GLS remained statistically significant after adjusting for other clinical and echocardiographic parameters (ß = 0.379, P = 0.01). In patients with BD, there is a subclinical alteration in RV contractility and the degree of alteration in the RV systolic performance paralleled that of LV. Thus, present results support the presence of RV involvement in these patients.

Drug Induced Thrombotic Microangiopathy with Certolizumab Pegol

Background: Certolizumab pegol is used to treat ankylosing spondylitis, Crohn's disease, psoriatic arthritis, and rheumatoid arthritis. Unlike other monoclonal antibodies such as infliximab and adalimumab, certolizumab does not contain an Fc fraction and hence does not induce complement activation. In this report, we describe the case of a patient with thrombotic microangiopathy caused due to certolizumab pegol, with a brief description about the pathophysiological approach to thrombotic microangiopathy. Case Report: A-39-year-old man suffering from ankylosing spondylitis for the past 10 years presented with fatigue. He had been on certolizumab pegol treatment for 6 months, starting with 400 and 200 mg every 2 weeks. He had significant nonimmune hemolytic anemia and thrombocytopenia without a disseminated intravascular coagulopathy. Schistocytes were observed in more than 10% of the erythrocytes per field. Plasma exchange along with corticosteroid treatment was started. There was a dramatic improvement within a week, and after 10 sessions of plasma exchange, the patient was discharged on corticosteroids with a tapering plan. ADAMTS13 enzyme activity was determined to be normal. Conclusion: The development of drug-induced thrombotic microangiopathy may be either immune-mediated or dose-dependent toxicity-mediated Anti-drug antibodies and their immunological aspects are still unclear and yet to be elucidated.

A rare cause of fever in an adult: a case of familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent fever attacks and serositis. Nonspecific manifestations of the FMF can mimic many common acquired disorders such as infections and acute abdomen. This can delay recognition for many years and lead to comprehensive assessments and even unnecessary surgeries. Untreated FMF may lead to serious complications such as end-stage renal disease and malabsorption due to amyloid deposits in the kidneys and the digestive system. Colchicine has been used successfully to treat FMF since the 1970s. CASE PRESENTATION: A 30-year-old male was admitted to our hospital with the complaints of fever, nausea, vomiting, and generalized myalgia and weakness for 15 days. The day after hospitalization, the patient had abdominal pain. Approximately a month before, the patient was treated for a diagnosis of urinary tract infection, with similar complaints. MEFV gene mutation analysis revealed homozygosity for the R202Q mutation. FMF was considered in the patient due to the presence of recurrent febrile serositis attacks and R202Q homozygous mutation in the FMF gene analyses. Colchicine was started 3×0.5 mg/day by consulting rheumatology on day 8 of admission. After the colchicine treatment, the patient's complaints markedly improved and the inflammatory markers returned to normal levels. At his follow-up visit at 6 months, the patient remained asymptomatic. CONCLUSION: We present a case of adult-onset FMF accompanied by peritonitis as a disease among the rare causes of fever in an adult who was treated with colchicine. Based on this case, we suggest that FMF should be kept in mind in the differential diagnosis of patients with periodic fever syndromes.

PECAM-1 gene polymorphisms and soluble PECAM-1 level in rheumatoid arthritis and systemic lupus erythematosus patients: any link with clinical atherosclerotic events?

Genetic polymorphisms of platelet endothelial cell adhesion molecule-1 (PECAM-1) were found to play roles in atherosclerotic events. We determined PECAM-1 polymorphisms, soluble PECAM-1, and CD40L levels in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) and evaluated their associations with clinical atherosclerotic complications. We included 100 RA patients, 81 SLE patients, and 94 healthy controls. The clinical features about the patients were obtained from medical records. Past cardiovascular complications were recorded. The most frequent gene polymorphisms of PECAM-1 were studied in our genetics laboratory. Soluble PECAM-1 and CD40L levels in serum were determined with ELISA. The frequencies of 373C (rs668) and 1688A (rs12953) alleles were higher in RA patients when compared to controls (p values, 0.028 and 0.016). RA and SLE patients had significantly higher allele frequencies for 2008A (rs1131012) when compared to controls (p values, 0.016 and 0.001). SLE patients had significantly more frequent AA genotype for rs1131012 polymorphism than RA patients and controls (p values, 0.007 and <0.001). Soluble PECAM-1 level was significantly higher in RA patients than in SLE patients and healthy controls (p values <0.001). Atherosclerotic complications were more frequent in SLE patients with AG genotype (rs12953) than those with AA genotype (p = 0.021). SLE patients with CC genotype (rs668) had a significantly lower frequency of atherosclerotic complications than those with CG genotype (p = 0.045). Nevertheless, in multivariate analysis, there was no association between genotype and atherosclerotic complications. We found associations between various PECAM-1 polymorphisms in RA and SLE; PECAM-1 and soluble CD40 ligand (sCD40L) levels were significantly higher in RA patients than in SLE and control groups. PECAM-1 polymorphisms in SLE were protective against atherosclerotic complications.