Analysis of clinical, immunological characteristics, damage, and survival in 300 Turkish systemic lupus erythematosus patients.

OBJECTIVE: This retrospective study aimed to conduct a comprehensive analysis of Turkish Systemic Lupus Erythematosus (SLE) patients of Caucasian ethnicity, focusing on their clinical, immunological, and therapeutic characteristics, damage accural and mortality. PATIENTS AND METHODS: We carried out a retrospective assessment of 300 SLE patients diagnosed between 2001 and 2017 at Kocaeli University Rheumatology Clinic. Demographic data, clinical manifestations, immunological profiles, treatment approaches, disease-related damage, and survival information were collected. RESULTS: The study population had a significant female predominance (89%) with a mean age of disease onset of 35.4 (SD:13.3) years. Hematological (72.6%) and mucocutaneous (72%) manifestations were the most common clinical findings, followed by arthritis (66.3%). Females had higher frequency of photosensitivity (p = 0.019), malar rash (p < 0.001), and alopecia (p = 0.014). Anti-dsDNA antibodies were detected in 61.6% of patients, while 57% of patients had hypocomplementemia. Secondary antiphospholipid syndrome was observed in 15.3% of patients; the most common manifestations included deep venous thrombosis (32.6%) and cerebrovascular accidents (30.4%). Lupus Nephritis (LN) affected 40.3% of the cohort. The most common pathologic finding was Class IV LN (30.5%). Eventually, 13 (4.3%) patients developed chronic kidney disease (CKD) and 4 had renal replacement therapies. Patients with LN had higher usage of pulse steroids, azathioprin, mycophenolate mofetil, cyclophosphamide, and rituximab (p < 0.001 for each).  In the juvenile-onset group (n = 31, 10.3%), an increased occurrence of malar rash (p = 0.009), nephritis (p = 0.034), hypocomplementemia (p = 0.001), positive anti-dsDNA (p = 0.007), anti-Sm (p = 0.046), anti-rib-P (p = 0.014) antibodies were observed. At least one damage parameter was observed in 32.6% patients with musculoskeletal manifestations being the most common. Thirteen patients were diagnosed with various malignancies, with cervical cancer being the most common (4 cases). The total 5 and 10-year survival rates were 92.5% and 86.7%, respectively. However, patients with CKD, had lower survival rates; 75% at 3 years and 60% at 15 years. Regression analysis demonstrated an association of CKD and  history of infections with decreased survival (p = 0.02, each). CONCLUSION: Ethnicity and geography influence the clinical diversity of SLE. Recognizing these disparities is crucial for tailoring patient care. Future inception cohort studies in Turkish SLE patients are necessary to address the limitations of retrospective research.

Gynecological symptoms in primary and secondary Sjögren's syndrome and the effect of the disease on sexuality.

BACKGROUND: Sjögren's syndrome (SS) is an autoimmune systemic disease affecting many organs and systems, such as genital system. AIM: This study aimed to present the gynecological symptoms of patients who were followed up in an outpatient clinic because of primary Sjögren's syndrome (pSS) and secondary Sjögren's syndrome (sSS) and to show how the disease affected sexuality. METHODS: This study is a cross-sectional study conducted between 2019 and 2020. The study sample consisted of 60 pSS patients, 42 sSS patients, and 52 healthy control subjects. OUTCOMES: All the participants were questioned about sexuality, and completed the 36-item Short Form Survey, Hospital Anxiety and Depression Scale, Health Assessment Questionnaire, and Modified Hill questionnaire. RESULTS: The patients had a mean age of 55.6 ± 11.85 years in pSS, 59.39 ± 11.18 years in sSS, and 56.1 ± 10.46 years in healthy control subjects. Vaginal and vulvar dryness and dyspareunia were present at a significantly higher rate in SS, especially in pSS, compared with the control subjects. The Health Assessment Questionnaire score was significantly lower in the pSS group than in the sSS group. Arthralgia, myalgia, and fatigue were prominent in all SS patients. CLINICAL IMPLICATIONS: Gynecological symptoms, sexual ability, and the effects of the disease on sexuality should be questioned in all SS patients. STRENGTHS AND LIMITATIONS: It is very important that we evaluate the gynecological symptoms of both pSS and sSS patients and the effect of the disease on these symptoms. The small number of patients and healthy control subjects is a limitation. CONCLUSION: The gynecological and musculoskeletal symptoms negatively affected sexuality in patients with pSS and sSS, and the negative effect of the disease on sexuality was more pronounced in the pSS group.

Treatment of systemic sclerosis-associated digital ulcers: recommendations of the Turkish Society for Rheumatology.

OBJECTIVES: Digital ulcers (DUs) are associated with a significant burden in systemic sclerosis (SSc) by leading to severe pain, physical disability, and reduced quality of life. This effort aimed to develop recommendations of the Turkish Society for Rheumatology (TRD) on the management of DUs associated with SSc. METHODS: In the first meeting held in December 2020 with the participation of a task force consisting of 23 rheumatologists the scope of the recommendations and research questions were determined. A systematic literature review was conducted by 5 fellows and results were presented to the task force during the second meeting. The Oxford system was used to determine the level of evidence. The preliminary recommendations were discussed, modified, and voted by the task force and then by members of TRD via e-mail invitation allowing personalised access to a web-based questionnaire [SurveyMonkey®]. RESULTS: A total of 23 recommendations under 7 main headings were formulated covering non-pharmacological measures for the prevention of DUs and pharmacological treatments including vasodilators, anti-aggregants, antibiotics, wound care, pain control, and interventions including sympathectomy, botulinum toxin, and surgery. Risk factors, poor prognostic factors, prevention of DU and adverse effects of medical treatments were reported as 4 overarching principles. CONCLUSIONS: These evidence-based recommendations for the management of SSc-associated DUs were developed to provide a useful guide to all physicians who are involved in the care of patients with SSc, as well as to point out unmet needs in this field.

Does decision tree analysis predict oral ulcer activity-related factors in patients with Behçet's syndrome?

OBJECTIVES: The study aimed to identify the interactions among treatment protocols and oral ulcer activity related factors in patients with Behçet's syndrome (BS) using the Classification and Regression Tree (CART) algorithm. METHODS: In this cross-sectional study, 979 patients with BS were included from16 centres in Turkey, Jordan, Brazil and the United Kingdom. In the CART algorithm, activities of oral ulcer (active vs. inactive), genital ulcer (active vs. inactive), cutaneous involvement (active vs. inactive), musculoskeletal involvement (active vs. inactive), gender (male vs. female), disease severity (mucocutaneous and musculoskeletal involvement vs. major organ involvement), smoking habits (current smoker vs. non-smoker), tooth brushing habits (irregular vs. regular), were input variables. The treatment protocols regarding immunosuppressive (IS) or non-IS medications were the target variable used to split from parent nodes to purer child nodes in the study. RESULTS: In mucocutaneous and musculoskeletal involvement (n=538), the ratio of IS use was higher in patients with irregular toothbrushing (ITB) habits (27.1%) than in patients with regular toothbrushing (RTB) habits (14.2%) in oral ulcer activity. In major organ involvement (n=441), male patients with ITB habits were more likely treated with IS medications compared to those with RTB habits (91.6% vs. 77.6%, respectively). CONCLUSIONS: Male BS patients on IS who have major organ involvement and oral ulcer activity with mucocutaneous and musculoskeletal involvement have irregular toothbrushing habits. Improved oral hygiene practices should be considered to be an integral part for implementing patient empowerment strategies for BS.

Proteomic analysis of thyroid tissue reveals enhanced catabolic activity in Graves' disease compared to toxic multinodular goitre.

Graves' disease (GD) and toxic multinodular goitre (TMNG) are the most common thyroid diseases which mainly lead to thyrotoxicosis, however, the underlying mechanism of distinct clinical presentations remains unclear. Protein extracts from the thyroid tissue specimens of the patients with GD and TMNG were subjected to Difference Gel Electrophoresis (DIGE). Differentially regulated protein spots were determined by image analysis, and the spots displaying statistically significant differences were identified by Matrix-Assisted Laser Desorption Ionization Time of Flight Mass Spectrometer (MALDI-TOF) followed by MASCOT search. Western blot analysis was used to verify changes occurring at the protein levels. The identified proteins were classified based on their functions in metabolic pathways using bioinformatics algorithms. Fifteen proteins showed significant alterations in abundance between the two disease groups. Bioinformatic analysis revealed the differentially regulated proteins were particularly related to catabolism, oxidative stress and especially energy utilization pathways, including glycolysis, proteolysis, ketone body catabolism and other energy metabolism-related pathways. SIGNIFICANCE OF THE STUDY: Previously, GD has been the subject of many studies that performed the proteomics approaches in the orbital tissue samples or tear. This is one of the very few studies that investigate the changes in the proteome of thyroid tissue in GD. We demonstrated mainly the upregulation of catabolic activity-related proteins in patients with GD compared to TMNG. Although it remains to be elucidated, some of these proteins can be used as markers for GD or have a role in the pathogenesis of the disease. Our study contributes the increasing data over time by providing new biomarker candidates for GD.

Are there any clinical differences between ankylosing spondylitis patients and familial Mediterranean fever patients with ankylosing spondylitis?

OBJECTIVE: Familial Mediterranean Fever (FMF) is an autoinflammatory disease that is commonly present with recurrent episodes of fever, peritonitis, pleuritis or arthritis. Enthesitis and sacroilitis can also be seen in FMF. Spondylitis is a less common manifestation of joint involvement in FMF and there are controversial publications about whether this involvement is FMF-related or coincidentally. The aim of this study was to provide a comparison between ankylosing spondylitis (AS) patients and FMF patients with AS. METHODS: A total of 404 patients who 360 of them was AS and 44 was FMF patients with AS (in accordance with Tel Hashomer) patients with AS (in accordance with modified New York criteria) were included in this study. All cases were evaluated retrospectively and patient's demographic and clinical data were recorded. RESULTS: The mean age was 34.5 ± 8.6 years and 61.4% of patients were female in FMF group. In AS group, the mean age was 41.2 ± 10.8 years and 67.8% of patients were male. In AS group, 92% of patients had inflammatory back pain, 51% had hip pain, 30% had heel pain, 14% had peripheral arthritis and 11% had uveitis. In FMF group, 98% of patients had inflammatory back pain, 59% had hip pain, 48% had heel pain, 43% had peripheral arthritis and 4.5% had uveitis. Syndesmophyte and enthesitis on X-ray were seen in 18% and 22% of AS patients, and 7% and 41% of FMF patients with AS, respectively. There were significant differences between AS patients and FMF patients with AS in terms of heel pain (P: .017), peripheral arthritis (P: .000) and enthesitis (P: .006). CONCLUSION: Peripheral arthritis and enthesitis were more frequent, and uveitis and syndesmophyte were less frequent in FMF patients with spondylitis than AS patients. When we look at gender differences, clinical and genetic features, it seems to be different condition from AS.

Software-based quantitative analysis of lung parenchyma in patients with systemic sclerosis may provide new generation data for pulmonary fibrosis.

OBJECTIVES: To investigate lung volume and density in patients with SSc and changes in these parameters because of PF, using a software-aided image quantification method, and compare this with a matched healthy control group. METHODS: Thoracic high-resolution computed tomography (HRCT) images of patients and controls were analysed using Myrian XP Lung 3D software. Right, and left lung densities and volumes were calculated separately by a blinded operator. Results were analysed between subgroups to investigate associations with the clinical features. RESULTS: A total of 135 patients with SSc and 38 healthy controls (HC) were included. Characteristics of the SSc patients were 94 (69.6%) without PF, 85.4% female, mean age 49.8 (15.4) years; 41 (30.4%) with PF, 88.3% female, mean age 50.2 (11.5) years, and HC group were 89.5% Female, mean age 52.2 (5.8) years. The right and left lung densities were significantly higher, and right and left lung volumes were significantly lower in the SSc patients with signs of fibrosis than those without and HC (P < .001 and P < .001; P = .006 and P = .002, respectively). After excluding patients with PF, right and left lung densities and volumes differed significantly between diffuse cutaneous SSc, limited cutaneous SSc, and HC (P = .002 and P < .001; P = .045 and P = .044, respectively). Patients who developed PF during follow-up had significantly lower baseline right and left lung densities than those who did not (P = .018; P = .014, respectively). Forced vital capacity and carbon monoxide diffusing capacity showed a weak correlation with lung densities and volumes in patients without PF and moderate to high correlation in PF patients. CONCLUSION: Lung density and volume in SSc patients changed significantly in those with PF and those without. Quantitative information extracted by soft-ware aided methods may contribute more to the detection, screening, and risk prediction in SSc-related PF.

Risk factors related to the size and volume of femoral artery pseudoaneurysm after catheter angiography: a single-center study

Background/aim: Evaluate the risk factors associated with pseudoaneurysms' development after the percutaneous interventional procedures performed by cardiology, interventional radiology (IR), and the other clinics. Materials and methods: We retrospectively analyzed the ultrasound scans in the hospital database and picture archiving system (PACS) and enrolled a total of 132 patients during the period from October 2015 and December 2019. We evaluated the maximum diameter and volume of the pseudoaneurysm with the patient and procedure-related factors with univariate analysis. Results: We found that the patients with hypertension and without peripheric artery disease (PAD) had greater sac diameter (p = 0.010 and p = 0.016) and increased sac volume (p = 0.029 and p = 0.007). However, the sac volume increased in patients with diabetes than those without (p = 0.003). Both the increased maximum diameter and the volume of the pseudoaneurysm sac were in the patients in whom the procedure was applied in the common femoral artery (CFA) and with the venous intervention (p < 0.010 and p < 0.016; p = 0.004 and p = 0.001, respectively). We found that platelet count correlated negatively with the sac's maximum diameter and the volume (r = ­0.383, p < 0.001 and r = ­0.486, p < 0.001, respectively) duration of intervention correlated positively with the sac's maximum diameter and the volume (r = 0.205, p = 0.019 and r = 0.320, p < 0.001). Conclusion: Our study reveals that prolonged procedure duration, simultaneous arterial and venous accesses, peripheral artery disease, thrombocytopenia, and puncture site are the aggressive risk factors of pseudoaneurysms size after angiographic procedures.

Epicardial adipose tissue thickness in systemic sclerosis patients without overt cardiac disease.

Systemic sclerosis is associated with an increased prevalence/incidence of coronary artery disease. The aim of this study was to investigate epicardial adipose tissue (EAT) thickness which may contribute to cardio-metabolic risk in systemic sclerosis (SSc) patients without overt cardiac disease. EAT thickness was measured by transthoracic conventional Doppler echocardiography and compared in SSc patients (n = 47) and age- and sex-matched healthy controls (n = 36). The relationships between EAT thickness and markers of cardio-metabolic risk in SSc were examined. EAT thickness was significantly greater in patients with SSc compared to healthy controls (6 [7-5] vs 5 [6.75-3.25], p = 0.041). Compared to controls, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, neutrophil, B-type natriuretic protein (BNP), fasting plasma insulin and HOMA-IR were elevated (18 [31-10] vs 8.5 [18-4], p < 0.001; 0.4 [0.67-0.18] vs 0.21 [0.48-0.09], p = 0.012; 7510 [8731-5990] vs 6435 [7360-5195], p = 0.002; 4350 [5440-3570] vs 3390 [4168-2903], p < 0.001; 111 [185-74] vs 70 [127-70], p = 0.010; 6.7 [10.5-4.7] vs 4.7 [6.8-4.1], p = 0.008; 1.7 [2.6-1] vs 1.1 [1.7-0.9], p = 0.015, respectively). The total and low-density lipoprotein (LDL)-cholesterol were decreased in SSc patients (197 ± 45 vs 284 ± 36, p = 0.005; 118 [148-84] vs 140 [180-115], p = 0.003, respectively). In patients with SSc, the EAT thickness correlated positively with age, ESR, CRP, insulin, hemoglobin A1c and total and LDL-cholesterol (r = 0.574, p < 0.001; r = 0.352, p = 0.015; r = 0.334, p = 0.022; r = 0.290, p = 0.048; r = 0.317, p = 0.030; r = 0.396, p = 0.006 and r = 0.349, p = 0.016, respectively). Our study confirms that EAT thickness is greater in SSc patients compared to healthy controls using echocardiographic measurements. The results of our study suggest that EAT thickness is a candidate for atherosclerotic risk assessment in SSc.

Radiological progression and predictive factors in psoriatic arthritis: insights from a decade-long retrospective cohort study.

OBJECTIVES: Radiological alterations in psoriatic arthritis (PsA) are an established phenomenon frequently observed throughout the disease course. Our goal was to investigate the changes in the bone structure of PsA patients by conventional radiography. METHODS: This study designed as a retrospective cohort study and cross-sectional evaluation for disease activity. The disease activity and the severity of skin and nail involvement were assessed. The Simplified Psoriatic Arthritis Radiographic Score (SPARS) was used to investigate the radiological progression. Logistic regression analysis was used to determine the predictors of radiological changes. RESULTS: Joint space narrowing and bone proliferation in hands (p = 0.001 and p = 0.001, respectively) and joint space narrowing in feet (p = 0.047) were more common at the final evaluation than at the baseline assessment. Total scores of joint space narrowing and bone proliferation in hands and feet were higher at the last visit than at the initial assessment (p < 0.001). Male gender (p = 0.030, OR 4.32 (95%CI 1.15-16.15)], older age (for joint space narrowing [p = 0.026 OR 1.08 (95%CI 1.01-1.56)] and for proliferation [p = 0.025 OR 1.08 (95%CI 1.01-1.44)]), high Disease Activity index for Psoriatic Arthritis (DAPSA) scores at baseline [p = 0.032 OR 6.21 (95%CI 1.17-32.92)], and symmetrical polyarticular involvement at baseline [p = 0.025 OR 5.3 (95% CI 1.23-22.4)] were found as predictors of structural changes. CONCLUSION: By the end of the decade, joint space narrowing and proliferation were observed to be more common than erosion. Male gender, older age, higher initial DAPSA scores, and initial polyarticular involvement were identified as predictors of radiological damage. Key Points • The radiological changes of Psoriatic arthritis are a well-known entity. However, studies investigating the progression of joint involvement over time are scarce. • This study reveals that joint space narrowing and proliferation are the most prominent radiological alterations in Psoriatic Arthritis patients at the end of the decade. • Male gender, older age, higher baseline DAPSA scores, and initial polyarticular involvement are predictive factors influencing the progression of bone destruction  in Psoriatic Arthritis patients.

Divergent perspectives: exploring the relationships between St. George's Respiratory Questionnaire and outcome measures in systemic sclerosis-associated interstitial lung disease.

INTRODUCTION/OBJECTIVES: Controversy exists regarding the concordance of patient-reported outcome measures (PROMs) with other assessment parameters in systemic sclerosis-associated interstitial lung disease (SSc-ILD). This study aims to explore the association between the St. George's Respiratory Questionnaire (SGRQ) and various outcome measures in patients with SSc-ILD within a real-world cross-sectional setting. METHOD: Patients with SSc-ILD were consecutively recruited from our SSc cohort. Simultaneous administration of SGRQ, scleroderma Health Assessment Questionnaire (sHAQ), respiratory visual analog scale (R-VAS), pulmonary function tests (PFTs), and the 6-min walking test (6-MWT) was conducted. The total extent of lung fibrosis was quantified using high-resolution computed tomography (HRCT) images. Relationships between SGRQ and functional, radiographic, and other patient-reported outcome measures were analyzed. RESULTS: The total SGRQ score demonstrated correlations with forced vital capacity (FVC) and R-VAS (r = - 0.397, p = 0.016 and r = 0.418, p = 0.027, respectively). Symptom score correlated with ILD-extension (r = 0.430, p = 0.005); activity score correlated with FVC and R-VAS (r = - 0.502, p = 0.002 and r = 0.395, p = 0.038, respectively); impact score correlated with R-VAS (r = 0.386, p = 0.043). In patients with fibrosis extent exceeding 20%, total SGRQ score was associated with sHAQ and R-VAS (r = 0.398, p = 0.049; r = 0.524, p = 0.021, respectively), activity score with R-VAS (r = 0.478, p = 0.038), and impact score with 6-MWT-D and R-VAS (r = - 0.489, p = 0.034; r = 0.545, p = 0.016, respectively). The symptom score and activity score demonstrated optimal performance in identifying patients with interstitial lung disease (ILD) extent exceeding 20% and forced vital capacity (FVC) less than 70% (area under the curve [AUC] 0.799, p = 0.002, and AUC 0.792, p = 0.03, respectively). CONCLUSIONS: Our study reveals varying degrees of correlation between SGRQ and distinct outcome measures. Given the incomplete alignment of SGRQ with other outcome measures, an integrative approach utilizing existing criteria as complementary tools is recommended. Key Points • Patient-reported outcome measures (PROMs) derive from patients' subjective evaluations of the impact of the disease on their daily activities, social interactions, and psychological well-being. • PROMs frequently serve as outcome measures in randomized controlled trials, yet conflicting findings have emerged in relation to primary outcomes. • This study aims to assess the appropriateness and interrelation of PROMs with both radiological and functional outcome measures, providing insight into the current state of our patients in a real-life context. The investigation delves into the compatibility of these measures with each other.

Extended autoantibody panel in Turkish patients with early-stage systemic sclerosis: Coexpressions and their influences on clinical phenotypes.

BACKGROUND/AIM: To investigate the frequency and clinical relevance of an extended autoantibody profile in patients with systemic sclerosis (SSc). MATERIALS AND METHODS: In this cross-sectional study, serum from 100 consecutive patients was subjected to indirect immunofluorescence (IIF) (HEp-20-10/primate liver mosaic) and Systemic Sclerosis Profile by EUROIMMUN to evaluate anti-nuclear antibodies (ANA) and autoantibodies against 13 different autoantibodies in patients with SSc less than 3 years. RESULTS: Ninety-three of 100 patients were positive for ANA by IIF. Fifty-three patients showed single positivity, 26 anti-topoisomerase antibodies (anti-Scl70 ab), 16 anticentromere antibodies (ACAs), six anti-RNA polymerase III antibodies (anti-RNAPIII ab), one anti-Ku antibody, one anti-PM/Scl100 antibody, two anti-PM/Scl75 antibodies, one anti-Ro52 antibody, whereas 32 patients had multiple autoantibody positivities. Among classic SSc-specific autoantibodies, anti-Scl70 and anti-RNAPIII abs showed the highest cooccurrence (n = 4). One patient was simultaneously positive for anti-RNAPIII ab and ACA, and one was positive for ACA and anti-Scl70 ab. The clinical features were not statistically different between single and multiple autoantibody-positivity for classic SSc-specific autoantibodies (ACA, anti-Scl70 ab, and anti-RNAPIII ab), except for digital ulcer in the multiantibody positive ACA group (p = .019). CONCLUSION: Based on our results, coexpression of autoantibodies is not uncommon in SSc patients. Although autoantibodies specific to SSc in early disease show generally known clinical features, it remains to be investigated how the coexpression of autoantibodies will affect clinical presentation.

Association of quantitative computed tomography indices with lung function and extent of pulmonary fibrosis in patients with systemic sclerosis.

OBJECTIVES: The aim was to investigate the discriminative value of a wide range of quantitative computed tomography (qCT) parameters in systemic sclerosis (SSc) patients with and without pulmonary fibrosis (PF) and their association with pulmonary function tests (PFTs) and visual fibrosis scores (VFS). METHOD: Thoracic high-resolution computed tomography (HRCT) images of SSc patients with and without PF were analyzed with Vitrea® Advanced Visualization software. The mean lung attenuation (MLA), skewness, kurtosis, and threshold-based volumes [low-density volume (LDV), medium-density volume (MDV), and high-density volume (HDV)] derived from the attenuation histograms of the right and left lungs were evaluated separately. Visual scores were measured semi-quantitatively and the overall extent of pulmonary parenchymal abnormality was calculated. RESULTS: Forty-one SSc patients with PF (85.4% female; mean age 50.4 ± 15.6 years) were compared with 94 without PF (88.3% female; mean age 50 ± 11.5 years). All qCT parameters were significantly different between those with and without PF (p < 0.05). Amongst the qCT measurements, R-MLA, L-MLA, R-MDV, L-MDV, and left total lung volume (L-TLV) correlated with all three of forced vital capacity, carbon monoxide diffusion capacity, and VFS, even after adjustment for sex and age (|r|> 0.300 and p < 0.05). R-MLA, L-MLA, R-HDV/TLV, and L-HDV/TLV exhibited diagnostic accuracy in discriminating patients with PF (AUC value > 0.7). CONCLUSION: QCT parameters differentiated SSc patients with PF from the ones without and showed a good correlation with VFS. With the application of user-friendly and less operator-dependent software, qCT analysis may become an objective tool for analysis of PF in SSc, complementary to PFTs and VFS. Key Points • Quantitative computed tomography parameters can accurately and objectively differentiate between SSc patients with and without PF. • Furthermore, in SSc patients with fibrosis, a moderate to a high correlation was identified between many of the qCT parameters, PFT results, and VFS.

Moderation analysis exploring associations between age and mucocutaneous activity in Behçet's syndrome: A multicenter study from Turkey.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet's syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement.

Validation of Turkish version of the Scleroderma Health Assessment Questionnaire.

OBJECTIVES: The Scleroderma Health Assessment Questionnaire (SHAQ) is a functional scale which consists of five scleroderma-specific items (overall disease severity, Raynaud's phenomenon, digital ulcers, respiratory and intestinal involvement) in addition to Health Assessment Questionnaire Disability Index (HAQ-DI). The objective of this study was to perform an adaptation and validation of a Turkish version of the SHAQ. METHOD: We validated psychometric properties of the scale with 70 consecutive systemic sclerosis (SSc) patients, who fulfilled the 2013 ACR/EULAR classification criteria for SSc. We evaluated test-retest reliability with the intraclass correlation coefficient (ICC), discriminant validity by stratifying patients according to organ involvements and disease subtypes, and convergent validity by testing the correlation between SHAQ and related components of Short Form 36 version 2 (SF-36v2). Internal consistency of the questionnaire was evaluated by Cronbach's alpha coefficient. RESULTS: The SHAQ-global, visual analogue scales (VAS) of pulmonary, digital ulcer, and Raynaud's phenomenon were significantly correlated with the physical component score of the SF-36v2 (r = - 0.274, r = - 0.295, r = - 0.326, r = - 0.308, p < 0.05, respectively) for the convergent validity. The instruments could not discriminate between disease subtypes, except the digital ulcer VAS which was significantly higher in patients with dcSSc (1.00 ± 0.93 vs 0.55 ± 0.88, p = 0.026) for the discriminant validity. The HAQ-DI, SHAQ-global, digital ulcer VAS, and pulmonary VAS showed moderate correlation with an increase in the number of the organs involved (r = 0.319, r = 0.329, r = 0.341, r = 0.278, p < 0.05, respectively). We demonstrated high reproducibility for HAQ-DI (ICC = 0.962, 95% confidence interval = 0.934-0.978) and the other items of SHAQ. The overall internal consistency of the SHAQ was satisfactory (Cronbach's alpha = 0.953). CONCLUSIONS: The Turkish version of the SHAQ met the requirements of validity and reproducibility.