The respiratory manifestations in patients with primary Sjögren's syndrome: is interstitial lung disease related to disease activity?

BACKGROUND: Sjögren's syndrome is a systemic, autoimmune disease and can affect many organs and systems. In this study, we aimed to evaluate the respiratory manifestations, and the association between interstitial lung disease (ILD) and disease activity in primary SS (pSS) patients. METHODS: The study design was retrospective cross-sectional, and 151 patients followed up with a diagnosis of pSS between 2004 and 2019 were included in the study. Demographic and clinical data, laboratory results, chest radiographs and thorax computed tomography (CT) results were obtained from patient files and hospital imaging system. Thorax CT was requested from all patients with respiratory complaints and abnormalities in physical examination and pulmonary function test. Disease activity was calculated with EULAR pSS disease activity index (ESSDAI) and clinical European League Against Rheumatism SS Disease Activity Index (clin-ESSDAI). RESULTS: In our study, 97% of pSS patients were female, and the mean age was 55.9 ± 12 years, disease onset age was 45.5 ± 12.1 years, disease duration was 10.7 (1-38) years. According to CT findings of 120 patients, 35% had nodules, and 13.3% had ILD (62.5% nonspecific interstitial pneumonia, 25% lymphocytic interstitial pneumonia, 12.5% usual interstitial pneumonia). Bronchiectasis, emphysema, sequelae fibrotic changes, and pleural thickening was found in 3.3%, 5.8%, 15.8%, and 1.7% of patients, respectively. It was observed that there was a significant relationship between the presence of ILD and persistent cough, mediastinal LAP, low DLCO, high ESSDAI and clin-ESSDAI scores reflecting disease activity. DISCUSSION: The most common pulmonary manifestation in our patients was ILD. ILD was observed more frequently in patients with moderate and severe disease activity. Some of the ILD patients were diagnosed while they were asymptomatic. Even if they are asymptomatic, it is important to follow up the patients with physical examination, spirometry, DLCO and thorax CT.

TNF-Related Apoptosis-Inducing Ligand Receptor 1 in Patients With Ankylosing Spondylitis.

OBJECTIVES: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) belongs to the tumor necrosis factor (TNF) superfamily and is reported to play a role in autoimmune diseases. In this study, we aimed to measure serum TRAIL receptor 1 (TRAIL-R1) concentration and assess any phenotypic relationship in patients with ankylosing spondylitis (AS). METHODS: Fifty-three patients with AS were recruited from August 2014 to December 2014 cross-sectionally. Fifty-three sex- and age-matched healthy controls were also recruited. Serum TRAIL-R1 concentrations were measured using an enzyme-linked immunosorbent assay. The association between serum TRAIL-R1, TNF-α, disease activity indices, markers of systemic inflammation, and clinical features were evaluated. RESULTS: Serum TRAIL-R1 and TNF-α levels were increased in patients with AS compared with healthy controls (4.5 ± 2.3 vs 3.5 ± 2.3 pg/mL, p = 0.036; 3.8 [1.6-7.7] vs 2.0 [0.21-5.7] pg/mL, p = 0.048, respectively). Serum TRAIL-R1 displayed a medium positive correlation with serum TNF-α concentrations (r = 0.412; p = 0.002). Serum TRAIL-R1 concentration was higher in human leucocyte antigen (HLA)-B27-positive patients compared with non-HLA-B27 patients (5.5 ± 2.2 vs 3.1 ± 1.6 pg/mL, p < 0.001). No relationship was found between serum TRAIL-R1 concentration and disease activity scores. CONCLUSIONS: This study confirms that serum TRAIL-R1 levels are higher in AS patients than healthy controls. The persistence of significantly elevated serum TRAIL-R1 levels, even in patients with low disease activity or after excluding biologic treatment, and the association with HLA-B27 positivity, warrants further investigation due to the unclear role of TRAIL-R1 in the pathophysiology of AS.

Evaluation of left and right ventricle by two-dimensional speckle tracking echocardiography in systemic sclerosis patients without overt cardiac disease.

BACKGROUND/OBJECTIVE: The aim was to evaluate the left and right ventricular functions concurrently by two-dimensional speckle tracking echocardiography (STE) in systemic sclerosis (SSc) patients without overt cardiac disease. METHODS: A total of 47 patients with SSc and 36 age- and sex-matched controls were evaluated cross-sectionally. Two-dimensional STE was used to assess the longitudinal peak systolic strains (PSS) of both ventricles including apical long-axis (APLAX), apical four-chamber (4-CH), apical two-chamber (2-CH), and global longitudinal measurements. Any association of metabolic, cardiac, and inflammatory biomarkers with PSS was investigated. RESULTS: The longitudinal PSS of the left ventricle [APLAX, 4-CH, 2-CH and global] were significantly lower in SSc patients than controls (- 18.2 ± 3.2 vs - 19.8 ± 2.7% p = 0.02; - 17.8 ± 3.5 vs. - 20.3 ± 3.3% p = 0.001; - 18.6 ± 3.1 vs. - 21.8 ± 3% p < 0.001; - 17.5 ± 5.7 vs. - 20.6 ± 2.7% p = 0.003, respectively). No difference was found between the groups for right ventricular strains. The longitudinal PSS-4CH correlated positively with CRP and ESR (r = 0.349, p = 0.016; r = 0.356, p = 0.014, respectively) and negatively with serum Galectin-3 (r = - 0.362, p = 0.012). Global longitudinal PSS-left ventricle (LV) correlated positively with CRP and homocysteine (r = 0.297, p = 0.043; r = 0.313, p = 0.041, respectively) and negatively with serum Galectin-3 (r = -0.314, p = 0.041). After multivariable adjustment, CRP remained the only predictor of longitudinal PSS-4CH (95% CI 0.35, 0.70, p = 0.028) and global longitudinal PSS of left ventricle (95% CI 0.004, 0.22, p = 0.043). CONCLUSIONS: Biventricular evaluation of patients with SSc by two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain, and no diastolic dysfunction. In SSc without overt cardiac disease, global cardiac assessment with 2DSTE is a promising method which seems to contribute to the detection of patients without clinical findings. KEY POINTS: • Two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain in SSc patients without overt cardiac disease. • CRP was the predictor of decreased longitudinal strains. • Cardiac assessment in SSc should be made globally.

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.