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.

Risk factors of avascular necrosis in Takayasu arteritis: a cross sectional study.

Takayasu arteritis (TA) is a large-cell vasculitis, and is not usually associated with avascular necrosis (AVN). The objective of the study was to investigate any association between TA and AVN, including the possible pathogenic effect of glucocorticoid (GCs) use. The study design was retrospective and cross sectional. TA patients were enrolled in the study. Demographic variables, disease activity, treatments, physician global assessment, Indian Takayasu Clinical activity score 2010, and Kerr criteria were recorded. Logistic regression analysis was performed to identify predictors of AVN. A total of 29 patients were assessed. AVN was observed in four (13.8%) patients with TA. Male gender and elevated C-reactive protein (CRP) were found to be significantly associated with AVN (p = 0.001 and p = 0.006, respectively). While type IIb TA was more common in patients with AVN (n = 2, 50%), type V was more likely in the absence of AVN (n = 13, 52%). Descending aorta and thoracic aorta were usually involved in patients with AVN (both, n = 3, 75%). In multivariate logistic regression, increased CRP levels were the only predictor for AVN (OR = 1.183, 95% Cl = 1.025-1.364, p = 0.021). No association was identified between AVN in TA patients and either duration or cumulative dose of GCs. The present study found that higher CRP levels and male gender were associated with AVN in patients with TA.

The role of lactate dehydrogenase-to-albumin ratio in clinical evaluation of adult-onset Still's disease.

OBJECTIVES: The aim of the study is to evaluate the importance of lactate dehydrogenase-to-albumin ratio (LAR) in patients with adult-onset Still's disease (AOSD) and to investigate the relationship between this ratio and clinical laboratory variables. METHODS: The study design was retrospective cross-sectional. The demographic and clinical data, laboratory results and imaging findings were documented. Univariate and multinomial logistic regression analyses were performed to find the predictors, which could be useful to define the ferritin level and organ involvement. Receiver-operating characteristic (ROC) analysis was used to clarify the diagnostic ability of the LAR for ferritin level and organ involvement. RESULTS: Fifty-eight patients with AOSD were evaluated. When patients were divided into two groups according to the serum ferritin level with a cut-off of 1500 ng/dL, lymphocyte count and albumin level were significantly less in patients who had higher ferritin levels (P = .015 and P = .005). In multinominal logistic regression analysis, AST, LDH and LAR were found as predictors for ferritin levels. When we compared LAR between patients with and without organ involvement, higher LAR was found in patients with HM, SM, serositis and MAS. Also, LAR was significantly higher in patients with higher ferritin levels (≥1500 ng/dL) than those without (P < .001). In ROC analysis, the cut-off point of LAR predicting the ferritin level was determined as 80.75 with 83.7% sensitivity and 80.0% specificity (AUC = 0.89, 95% CI 0.79-0.98, P < .001). CONCLUSION: LAR can be a valuable inflammatory marker to determine AOSD patients with organ involvement and higher ferritin level.

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.