Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed. RESULTS: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. CONCLUSIONS: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.

The Effects of 2 Different Soft Tissue Mobilization Techniques on Delayed Onset Muscle Soreness in Male Recreational Athletes: A Single-Blinded Randomized Controlled Trial.

CONTEXT: Soft tissue mobilization is frequently employed for delayed onset muscle soreness (DOMS) management. Foam roller and percussive massage are 2 popular soft tissue mobilization methods preferred by various professionals. However, their effects on DOMS symptoms are controversial and there are no studies comparing these 2 methods. The aim of the present study was to compare the acute effects of soft tissue mobilization with a foam roller or a percussive massage device on DOMS in young male recreational athletes. DESIGN: A parallel, single-blinded, randomized controlled trial. METHODS: Thirty-six participants (median [interquartile range 25/75]; age: 20.0 [19.3/21.0] y) were randomly allocated to percussive massage group (n = 12), foam roller group (n = 12), and control group (n = 12). First, a fatigue protocol targeting quadriceps femoris was performed. Then, participants received soft tissue mobilization with foam roller/percussive massage or rested for 10 minutes according to their groups. Pain and fatigue were evaluated by a visual analog scale, and the skin surface temperature of over the quadriceps femoris was measured with thermal camera imaging. Evaluations were performed at baseline, following fatigue protocol, at 24th hour, and at 48th hour. Changes from the baseline at 24th and 48th hours were compared between groups. RESULTS: No significant between-group differences were observed at the assessments performed at 24th or 48th hour regarding the changes from baseline in pain (P value for 24th hour = .905, P value for 48th hour = .733), fatigue (P value for 24th hour = .895, P value for 48th hour = .606), or skin surface temperature measurements (P values for 24th hour = between .300 and .925, P values for 48th hour = between .311 and .750). CONCLUSIONS: Soft tissue mobilizations applied with foam roller or percussive massage device do not seem to be superior to passive resting in alleviating DOMS symptoms in recreational athletes.

The effect of testosterone level on metabolic syndrome: a cross-sectional study.

BACKGROUND: Metabolic syndrome (MS) may reduce circulating testosterone and, at the same time, low testosterone levels may lead to MS. Thus, identifying problems regarding sex hormones and examining their effects on the pathogenesis of MS is important to prevent serious complications of the condition, such as diabetes or cardiovascular diseases. AIMS: This study aimed to investigate the correlations between MS-related parameters and androgen levels. METHODS: A total of 108 males [median age 48.5 years (min/max = 21/77 years)] were included in the study. Blood pressure and anthropometric measurements (body mass index, waist circumference, hip circumference, thigh circumference, neck circumference, and length of index and ring finger) were performed. Biochemical analysis was assessed. Additionally, total testosterone, free testosterone, and sex hormone binding globulin levels were investigated. RESULTS: Weak negative correlations were observed between testosterone levels and several anthropometric measures/glucose metabolisms (p < 0.05). The highest correlation was between total testosterone levels and body mass index (rho= -0.390, p < 0.001) CONCLUSION: According to our results, controlling weight, one of the preventable risk factors, can have a positive effect on testosterone levels and, therefore, on the cardiovascular system through different mechanisms.

Systemic Sclerosis Quality of Life Questionnaire (SScQoL): translation into Turkish and assessing its psychometric properties.

BACKGROUND: Systemic sclerosis (SSc) is a chronic autoimmune disease that affects the connective tissues and leads to physical, emotional, and social challenges for patients. Evaluating health-related quality of life (HRQoL) with a disease-specific tool may be preferable for improving patient care and treatment outcomes. The aim of this study was to translate the Systemic Sclerosis Quality of Life Questionnaire (SScQoL) into Turkish and to investigate its psychometric properties. METHODS: Eighty-six patients with SSc (mean age 51.8 ± 11.7 years, 80 females) participated in the study. Convergent validity was explored by correlation analyses between Turkish SScQoL and Short-Form 36 (SF-36), European Quality of Life Survey-5 Dimensions (EQ-5D), EQ-5D Visual Analog Scale (EQ-VAS), and Scleroderma Health Assessment Questionnaire (SHAQ). Cronbach's alpha was calculated to test internal consistency. Turkish SScQoL was readministered after 7-14 days to fifty-eight patients for determining test re-test reliability. Intraclass correlation coefficients in 95% confidence interval (ICCs [95%CI]) were calculated to examine the agreement between two assessments. Values greater than 15% and an absolute skewness value < 1 were recognized as the presence of a floor or ceiling effect. RESULTS: SScQoL correlated significantly with SF-36 subdomains (r = -0.347 to -0.618, p < 0.01), EQ-5D (r = -0.535, p < 0.01), EQ-VAS (r = -0.636, p < 0.01), and SHAQ global score (r = 0.521, p < 0.01). SScQoL demonstrated excellent internal consistency (Cronbach's alpha = 0.917), and good to excellent test-retest reliability (ICC [95%CI] = 0.85 [0.76-0.91]). No floor/ceiling effects were observed. CONCLUSION: The Turkish version of SScQoL seems to have adequate psychometric properties and can be used to evaluate HRQoL in clinical and research settings. Key points • Turkish version of SScQoL is a valid and reliable tool for measuring health-related quality of life of patients with systemic sclerosis. • SScQoL is the only diseases-specific quality of life measurement for systemic sclerosis available in Turkish. • Patients with limited and diffuse SSc seem to be similar in terms of self-reported health-related quality of life.

Assessing dyspnea-related kinesiophobia in patients with systemic sclerosis (SSc): validity and reliability of Turkish Breathlessness Beliefs Questionnaire for SSc.

INTRODUCTION/OBJECTIVE: The respiratory system is often affected by systemic sclerosis (SSc), a connective tissue disease characterized by fibrosis, vasculopathy, and inflammation. As a result, especially exertional dyspnea may occur in SSc patients. Evaluation of attitudes towards dyspnea is important in terms of preventing negative consequences such as kinesiophobia. However, no validated tool is available for assessing dyspnea-related kinesiophobia for patients with SSc. The aim of the present study was to perform the Turkish validation of the Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc), which was adapted from the Tampa Kinesiophobia Scale, and to investigate its relationships with physical and clinical characteristics. METHOD: Fifty patients with SSc (47 females) were included in the study. The patients were evaluated regarding pulmonary function tests, respiratory muscle strength, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue. The patients were re-evaluated 1 week later for the test-retest reliability of the BBQ-SSc. RESULTS: Internal consistency (Cronbach's alpha: 0.862) and test-retest validity (ICC: 0.831; 95% CI: 0.702-0.907) of the 12-item Turkish BBQ-SSc were found to be good. Principal component analysis confirmed the two-dimensional structure of the questionnaire. The scores of the questionnaire were associated with duration of illness, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue (p < 0.05). CONCLUSIONS: According to our results, the 12-item Turkish BBQ-SSc is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients. Key Points • There are no structured tools available for assessing dyspnea-related kinesiophobia in patients with systemic sclerosis (SSc) • The 12-item Turkish Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc) is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients.

Non-steroidal anti-inflammatory drug use is determined by disease activity in axSpA and decreased by biologicals: a longitudinal analysis.

OBJECTIVE: To evaluate non-steroidal anti-inflammatory drug (NSAID) use and Assessment in Spondyloarthritis International Society (ASAS)-NSAID scores in patients with axial spondyloarhritis (axSpA) in a longitudinal study. METHODS: In total, 429 patients with axSpA (59% male; 63.6% with AS) were included in this study. Data about disease activity, C-reactive protein (CRP) levels, and NSAID use and dosage were collected at 0, 12, 24, and 52 weeks retrospectively. The relationship with NSAID use /ASAS-NSAID scores and other factors were tested using generalized estimating equations (GEE). RESULTS: At baseline (0 weeks), 92.8% of patients in biologic disease-modifying anti-rheumatic drugs (bDMARDs) group and 82.1% of patients in conventional treatment group were treated with NSAIDs. At baseline, the proportion (p=0.03) and the median (IQR) ASAS-NSAID scores were higher in bDMARDs group [100 (50) vs 50 (83.4); p<0.001]. During follow-up, NSAID use and ASAS-NSAID scores decreased significantly in patients treated with bDMARDs (p<0.001) and the reduction remained stable throughout the follow-up However, neither NSAID use (p=0.06) nor ASAS-NSAID scores changed in conventional treatment group (p=0.15). In bDMARD-treated patients, ASDAS-CRP and BASFI scores were independent determinants for NSAID use, and BASDAI and PGA were determinants for NSAID dosage. There was no independent significant predictor for ASAS-NSAID scores; PGA was the only significant predictor for NSAID use in the conventional treatment group. CONCLUSION: Concurrent biologic treatment was associated with low NSAID intake in patients with axSpA, and NSAID use was determined mainly by disease activity and partly by function during bDMARD treatment.

Investigating performance-based hand functions in patients with systemic sclerosis.

OBJECTIVE: Systemic sclerosis (SSc) is a connective tissue disorder characterized by collagen deposits in various organs. Skin involvement is one of the most common symptoms and along with vascular damage, may deteriorate hand functions. However, the status of hand functions has generally been investigated using patient-reported measures in patients with SSc. The aim of the present study was to investigate performance-based hand functions in patients with SSc using the Sollerman Hand Function Test (SHFT). METHODS: A total of 39 patients with SSc (33 females) were included in the study. Twenty-four patients were classified as limited cutaneous SSc (lcSSc), while 15 patients were classified as diffuse cutaneous SSc (dcSSc). Hand-related physical characteristics were evaluated using the Modified Hand Mobility in Scleroderma Test, grip strength, and pinch strengths. The Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire, and Scleroderma Health Assessment Questionnaire were used as patient-reported measures. Performance-based hand functions were evaluated using SHFT. RESULTS: No significant differences were observed between lcSSc and dcSSc subtypes regarding performance-based and patient-reported hand functions (p > 0.05). SHFT scores significantly correlated with hand-related physical characteristics and patient-reported hand functions (p < 0.05). The highest correlation was determined between SHFT and the Duruoz Hand Index (rho: -0.652, p < 0.001). CONCLUSION: According to our results, performance-based hand functions seem not to be affected by disease subtype. Performance-based hand functions may partially be captured by the patient-reported outcomes, especially the Duruoz Hand Index, in patients with SSc.

Investigating the lower extremity-related anaerobic exercise capacity and functional status in adult patients with familial Mediterranean fever.

OBJECTIVES: No studies examined the lower extremity-related anaerobic exercise capacity or functional status in adult patients with FMF. METHODS: Twenty-four patients with FMF (12 males) and 24 age-sex-matched healthy controls (13 males) were included in the study. Lower extremity-related anaerobic exercise capacity was assessed by using Wingate Anaerobic Test. Lower extremity-related functional status was examined by using 9-Step Stair Climb Test, 10-Repetition Chair Stand Test, and Six-Minute Walking Distance. Muscle strength of hip flexors, hip extensors, knee flexors, and knee extensors were evaluated by using a hand-held dynamometer. RESULTS: Patients with FMF had significantly poorer results in all anaerobic exercise capacity parameters and functional status assessments (P < 0.05), except muscle strength measurements (P > 0.05). Both average and peak anaerobic exercise capacities correlated significantly with all muscle strength measurements, 9-Step Stair Climb Test, 10-Repetition Chair Stand Test times, and Six-Minute Walk Distances (P < 0.05) in patients with FMF. CONCLUSION: Lower extremity-related anaerobic exercise capacity and functional status seem to be diminished in adult patients with FMF. Evaluating these parameters may be beneficial for planning more appropriate and individualized treatment regimens such as patient education and exercise counselling for patients with FMF.

Is the international physical activity questionnaire (IPAQ) a valid assessment tool for measuring physical activity of patients with axial spondyloartritis?

BACKGROUND: Determining the level of physical activity (PA) is an essential part of patient evaluation in axial spondylarthritis (axSpA). Subjective and objective methods are both frequently used methods for evaluating PA. Although subjective methods are cost-effective and easy to use, their accuracy for measuring PA is still questionable. OBJECTIVE: To investigate the concurrent criterion validity of a self-reported questionnaire (IPAQ-Short Form) when compared to an accelerometer (Actigraph wGT3X-BT) for measuring PA level in patients with axSpA. DESIGN: Cross-sectional design. METHODS: Fifty-eight patients with axSpA with a median age of 39.0 (IQR 25/75: 30.0/46.0) years were included in the study. An accelerometer (Actigraph wGT3X-BT) was attached to the waist of patients at their first visits and was removed at their second visits, seven days later. Patients were asked to complete the International Physical Activity Questionnaire Short Form (IPAQ) at their second visits. RESULTS: No significant correlations were determined between IPAQ and accelerometer (p > 0.05), except for the moderate PA (rho: 0.367, p < 0.05), and total PA (rho: 0.330, p < 0.05). It was also observed that IPAQ was underestimating energy expenditure for all types of PA. CONCLUSION: IPAQ might not be a valid tool for measuring PA level in patients with axSpA. Disease-specific subjective methods for determining the PA should be developed and validated for those patients.

Comparison of physical activity levels among different sub-types of axial spondyloarthritis patients and healthy controls.

OBJECTIVES: The aim was to compare the physical activity levels among radiographic axial spondyloarthritis (axSpA) patients, non-radiographic axSpA patients, and healthy controls and investigating the possible relationships between physical activity level and clinical features. METHODS: Thirty-four patients with radiographic axSpA (24 male), 33 patients with non-radiographic axSpA (23 male), and 35 age and sex-matched healthy controls (24 male) were included. The patients were assessed with Bath Ankylosing Spondylitis Disease Activity Index, Bath Ankylosing Spondylitis Functional Index, Bath Ankylosing Spondylitis Metrology Index, Ankylosing Spondylitis Quality of Life Questionnaire, Hospital Anxiety and Depression Scale, Tampa Scale of Kinesiophobia. Physical activity was measured by using an accelerometer (Actigraph wGT3X-BT). RESULTS: Physical and disease-related characteristics were comparable between groups (p > .05). Radiographic axSpA patients showed lesser physical activity compared to non-radiographic axSpA patients and healthy controls (p < .05). No difference was detected between non-radiographic axSpA patients and healthy controls (p > .05). Physical activity levels were correlated with different clinical features for each sub-type of axSpA. Decreased spinal mobility is the most correlated disease characteristic with lower physical activity level for both sub-types. CONCLUSION: It seems that disease sub-type in axSpA may alter the physical activity levels. Increasing physical activity levels might need different approaches for different sub-types of axSpA.

The role of smoking in the development and progression of structural damage in axial SpA patients: A systematic review and meta-analysis.

OBJECTIVE: The objective of this study was to determine whether smoking was associated with the cumulative radiographic spinal damage and radiographic progression in patients with ankylosing spondylitis (AS). Thus, we conducted a systematic review and meta-analysis of the available studies to date. METHODS: An electronic search was conducted from inception to June 21, 2016, in EMBASE, the MEDLINE/PubMed Cochrane Central Register of Controlled Trials databases. Cross-sectional and longitudinal cohort studies investigating the association between smoking and cumulative spinal structural damage or radiographic progression were included. The outcome of interest was the presence of syndesmophytes in cross-sectional studies and radiographic progression in longitudinal studies. The quality assessment was done using the Agency for Healthcare Research and Quality checklist. The authors of potentially relevant studies were contacted regarding the unpublished data. Data from eligible cross-sectional studies were extracted and arranged in a 2x2 table. The odds ratios (ORs) and 95% confidence intervals (CIs) for the dichotomous outcome of interest were computed. RESULTS: The combined data of eight eligible cross-sectional studies for the assessment of association between smoking and cumulative spinal structural damage suggested a significant association (OR, 2.02; 95% CI, 1.51-2.70). No significant heterogeneity was detected between studies (I2=23.0%, p=0.25). The heterogeneity of the longitudinal study data did not permit us to undertake a meta-analysis. Hence, a qualitative review was performed. CONCLUSION: The results of our meta-analysis show that smoking is associated with increased cumulative spinal structural damage in patients with AS. Therefore, rheumatologists should encourage patients with AS to quit smoking.

Role of the mTOR pathway in minor salivary gland changes in Sjogren's syndrome and systemic sclerosis.

BACKGROUND: To examine the activity of the mammalian target of rapamycin (mTOR) pathway and its regulators, transforming growth factor (TGF)-ß1 and phosphatase and tensin homolog (PTEN), in minor salivary gland biopsies of Sjogren's syndrome (SS) and systemic sclerosis (SSc) patients. METHODS: We retrospectively evaluated SS, SSc, and SS-SSc overlap patients admitted to our outpatient rheumatology clinic between January 2007 and December 2015 who underwent a minor salivary gland biopsy. Patient demographics and some clinical features were obtained from hospital records. Immunohistochemistry was used to analyze total mTOR, total PTEN, and TGF-ß1 expression in the biopsied tissues. The biopsy specimens were also examined for the presence and degree of fibrosis. RESULTS: Minor salivary gland biopsies of 58 SS, 14 SSc, and 23 SS-SSc overlap patients were included in the study. There was no significant difference in mTOR expression between these groups (P = 0.622). PTEN protein was expressed in 87.2% of patients with SS, 57.9% with overlap syndrome, and 100% of the SSC patients, and these differences were statistically different (P = 0.023). Although ductal epithelial TGF-ß1 expression was similar between the groups (P = 0.345), acinar cell expression was found to be more frequent in the SSc (72.7%) and overlap patients (85.7%) in comparison with the SS cases (58.2%; P = 0.004). CONCLUSION: mTOR may be one of the common pathways in the pathology of both SS and SSc. Hence, there may be a role for mTOR inhibitors in the treatment of both diseases. Additionally, PTEN and TGF-ß1 expression may be a distinctive feature of SSc.

Saliva levels of caspase-1, TNF-α, and IFN-γ in primary Sjögren's syndrome: oral mucosal abnormalities revisited

Background/aim: Abnormalities in oral mucosal immunity contribute to complex pathogenesis of primary Sjögren's syndrome (pSjS). We aimed to measure saliva and serum levels of caspase-1, tumor necrosis factor-alpha (TNF-α) and interferon gamma (IFN-γ) in patients with pSjS. Materials and methods: We studied 43 pSjS patients fulfilling the AECG criteria and 30 age/sex-matched healthy controls, as well as 39 rheumatoid arthritis (RA) patients as a disease control group. ESSDAI scores were less than seven in all patients with pSjS, indicating low disease activity. Quantitative analyses were made in serum and whole saliva samples. The statistical analysis was performed using SPSS 19.0. Results: While no significant difference was found in serum measurements, saliva levels of TNF-α and caspase-1 were significantly higher in pSjS patients versus healthy controls when using the Mann-Whitney U test. On the other hand, in the pSjS group, saliva levels of TNF-α and caspase-1 were also significantly higher compared to the RA group using Student's t-test. In the pSjS group, those parameters did not show any correlation with disease duration, seropositivity, and smoking. Conclusion: Despite low disease activity, saliva TNF-α and caspase-1 levels were found to be significantly higher in the pSjS group, which may suggest a possible advantage of local anticytokine treatments in selected cases.

Segmental arterial mediolysis mimics systemic vasculitis.

Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.

Imaging findings of pulmonary granulomatosis with polyangiitis (Wegener's granulomatosis): lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy.

BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. METHODS: This retrospective study included 48 patients, aged 28-73 (mean, 47.3) years, who showed either histopathological diagnosis of GPA (n = 39) or elevated levels of the cytoplasmic anti-neutrophilic cytoplasmic antibody serum marker (n = 9) between January 2003 and December 2013. All patients received a chest computed tomography (CT), and the types of pulmonary lesions were defined and evaluated. RESULTS: Among the 48 patients, 33 had abnormal pulmonary findings on CT. The most commonly detected pulmonary lesion types were nodules and masses (n = 126) observed in 24 patients. Cavitation, necrosis, spiculation and invasion of the fissure, pleura or diaphragm were observed in 14, 9, 10 and 6 patients, respectively. Consolidation was found in 14 patients and thickening of bronchial wall in 8 patients. CONCLUSIONS: Pulmonary lesion types of GPA have a wide spectrum, potentially mimicking a high number of diseases including malignancy, infection and noninfectious inflammatory diseases. A spiculated lung lesion invading the fissure, pleura or diaphragm is mostly present in malignancy, but it can be also seen in GPA.

Incidence of Cyclophosphamide-induced Urotoxicity and Protective Effect of Mesna in Rheumatic Diseases.

OBJECTIVE: To assess bladder toxicity of cyclophosphamide (CYC) and uroprotective effect of mesna in rheumatic diseases. METHODS: Data of 1018 patients (725 women/293 men) treated with CYC were evaluated in this retrospective study. All of the following information was obtained: the cumulative CYC dose, route of CYC administration, duration of therapy, concomitant mesna usage, and hemorrhagic cystitis. Cox proportional hazard model was used for statistics. RESULTS: We identified 17 patients (1.67%) with hemorrhagic cystitis and 2 patients (0.19%) with bladder cancer in 4224 patient-years. The median time for diagnosis to hemorrhagic cystitis was 10 months (4-48) and bladder cancer was 8 years (6-10.9). There were 583 patients (57.2%) who received mesna with intravenous CYC therapy. We observed similar incidence rate for hemorrhagic cystitis in both patient groups concomitantly treated with or without mesna [9/583 (1.5%) vs 8/425 (1.8%) respectively, p = 0.08]. Cumulative CYC dose (HR for 10-g increments 1.24, p < 0.001) was associated with hemorrhagic cystitis. CONCLUSION: Cumulative dose was the only risk factor for hemorrhagic cystitis in patients treated with CYC. No proof was obtained for the uroprotective effect of mesna in our cohort.

Plasma thiopurine S-methyltransferase levels and azathioprine-related adverse events in patients with Behçet's disease.

OBJECTIVES: Thiopurine S-methyltransferase (TPMT) is the key enzyme inactivating azathioprine (AZA), an immunosuppressive agent commonly used for treating inflammatory diseases including Behçet's disease (BD), systemic lupus erythematosus (SLE) and systemic vasculitis. Low TPMT levels facilitate occurrence of AZA-related adverse effects. We investigated TPMT levels in patients with BD, compared to healthy controls and patients with SLE or systemic vasculitis. METHODS: This cross-sectional study included 101 BD (77 using AZA), 74 SLE (35 using AZA), and 44 vasculitis (18 using AZA) patients and 101 healthy controls. Plasma TPMT levels were measured using ELISA. Student's t- and Kruskal-Wallis tests were used to compare TPMT levels according to possible risk factors. Receiver operating characteristic (ROC) analysis was used to determine whether a cut-off TPMT level could be found to predict AZA-related adverse effects. RESULTS: Plasma TPMT levels (mean± SD ng/mL) in BD (22.80±13.81) were comparable with healthy controls (22.71±13.49), but significantly lower than in SLE group (29.37±11.39) (p<0.001). TPMT levels in 130 patients receiving AZA were similar to the rest of the group. AZA-related adverse effects were identified in only 8 patients (5 with BD and 3 with SLE). TPMT levels were significantly lower in those 8 patients (14.08±9.49 vs. 25.62±12.68) (p=0.013), besides a cut-off value for predicting adverse effects was determined for the BD group with ROC analysis (area under the curve: 0.813). CONCLUSIONS: This is the first study to evaluate TPMT activity in a Turkish adult population. Although low plasma TPMT level is not the only factor determining AZA toxicity, a TPMT cut-off value may help to predict AZA-related adverse effects in BD.

A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients.

OBJECTIVES: Behçet's disease (BD) is a chronic, multisystemic disorder characterised by recurrent oral aphtous ulcers, genital ulcers and ocular inflammation. Vasculitis and thrombotic events are the most important causes of mortality. Vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms are the other less common vascular manifestations of BD. Cardiac involvement in BD is a rare and life-threatening complication. The aim of this study was to assess the clinical characteristics and outcome of patients with BD who have intracardiac thrombus. METHODS: The hospital files of BD patients followed by rheumatology clinics of four medical centres (Ankara University, Ege University, Hacettepe University and Eskisehir Osmangazi University Hospitals) were retrospectively evaluated. Data included patients' demographic and clinical features, laboratory findings and outcome. All patients fulfilled three or more of the International Study Group Criteria for BD. RESULTS: Twenty-two patients with intracardiac thrombus were evaluated. The mean age of patients with intracardiac thrombosis was 29.1 yrs (22-44) and there was a male predominance with a ratio of 20:2. Cardiac involvement was the first clinical manifestation of BD in 9 of the 22 patients. Initial symptoms of the patients were fever (n=18, 81%), dyspnea (n=9, 40%) chest pain (n=9, 40%) and haemoptysis (n=7, 31.8%). Sixteen patients (72%) had pulmonary arteritis and 10 (45%) patients had venous system lesions included deep vein, inferior vena cava and hepatic vein. Intra-cardiac thrombus were found only in the right cavities in 17 patients (77%), only in the left cavities in 2 patients (9%), and in both left and right cavities of the heart in 3 patients (13.6%). Once the cardiac lesion was diagnosed as a complication of BD, high dose (1mg/kg/d) prednisone (n=22, 100%) plus cyclophosphamide (n=18, 81%) or azathioprine (n=3, 13.6%) and warfarin (n=8, 36.3% (after the elimination of pulmonary aneurysm) therapy for anticoagulation was initiated. Four patients (18%) had high dose prednisone plus cyclophosphamide plus interferon-α (IFN-α) combination treatment and two patients (n=2, 9%) had high dose prednisone plus cyclophosphamide or plus azathioprine combination treatment. After treatment, the intra-cardiac thrombus disappeared in 13 cases and the size of the thrombus reduced in 7 cases. One patient died because of recurrent intra-cardiac thrombus and massive pulmonary arteritis in the emergency department. CONCLUSIONS: Intracardiac thrombus in BD is more common in young men. The right side of the heart is usually involved and cardiac involvement is often accompanied by pulmonary artery occlusion possibly due to pulmonary arteritis. Early and aggressive immunosuppressive and/or anticoagulation therapy are life-saving.