Anticentromere antibody levels and isotypes and the development of systemic sclerosis

Objective Little is known on the disease course of very early systemic sclerosis (SSc). Among the information yet to be elucidated is whether anticentromere antibody (ACA) isotype levels can serve as biomarkers for future SSc development and for organ involvement. This study was undertaken to evaluate whether IgG, IgM, and IgA ACA levels in IgG ACA-positive patients are associated with disease severity and/or progression from very early SSc to definite SSc. Methods IgG ACA-positive patients from 5 different cohorts who had very early SSc or SSc fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria were included. A diagnosis of very early SSc was based on the presence of IgG ACAs and Raynaud's phenomenon, and/or puffy fingers and/or abnormal nailfold capillaroscopy, but not fulfilling the ACR/EULAR 2013 criteria for SSc. Multivariable regression analyses were performed to determine the association between baseline ACA isotype levels and progression to definite SSc with organ involvement. Results Six hundred twenty-five IgG ACA-positive patients were included, of whom 138 (22%) fulfilled the criteria for very early SSc and 487 (78%) had definite SSc. Levels of IgG ACAs (odds ratio 2.5 [95% confidence interval 1.8-3.7]) and IgM ACAs (odds ratio 1.8 [95% confidence interval 1.3-2.3]) were significantly higher in patients with definite SSc. Of 115 patients with very early SSc with follow-up, progression to definite SSc occurred within 5 years in 48 (42%). Progression to definite SSc was associated with higher IgG ACA levels at baseline (odds ratio 4.3 [95% confidence interval 1.7-10.7]). Conclusion ACA isotype levels may serve as biomarkers to identify patients with very early SSc who are at risk for disease progression to definite SSc.Pathophysiology and treatment of rheumatic disease

Data for: The preliminary validation of laser Doppler flowmetry in systemic sclerosis in accordance with the OMERACT filter: a systematic review

This dataset contains the results of the literature review on the use of a single probe laser Doppler flowmetry in the assessment of the digital microvasculopathy in systemic sclerosis patients

Data for: The preliminary validation of laser Doppler flowmetry in systemic sclerosis in accordance with the OMERACT filter: a systematic review

This dataset contains the results of the literature review on the use of a single probe laser Doppler flowmetry in the assessment of the digital microvasculopathy in systemic sclerosis patients

Automated assessment of absolute nailfold capillary number on videocapillaroscopic images: Proof of principle and validation in systemic sclerosis

Background: Absolute nailfold capillary number should be a putative biomarker in selected rheumatic diseases but could be time-consuming and not highly repeatable. Objective: To validate an automated software for absolute nailfold capillary number and density evaluation, on NVC images in SSc. Methods: An automated software to count nailfold capillary number (AUTOCAPI) had been constructed, through an exploratory image set. Subsequently, application rules have been created to define the ROI in NVC images, through a training images set. The software reliability was assessed through calculation of the ICC between automatic and manual counting, by four independent observers, on the same NVC images. Results: The following ICC's were obtained per observer, for the patients with SSc (40 images), the healthy (20 images), and the PRP subgroups (20 images), respectively: 0.94, 0.81, and 0.62 (observer 1); 0.94, 0.91, and 0.67 (observer 2); 0.88, 0.56, and 0.64 (observer 3); and 0.88, 0.85, and 0.85 (observer 4). Conclusions: The validation of an automated software for measuring absolute nailfold capillary number and density in SSc was achieved. The integration into the pre-existing imaging software should make the assessment of the capillary number in NVC easier, quicker, and standardized

Is laser speckle contrast analysis (LASCA) the new kid on the block in systemic sclerosis? A systematic literature review and pilot study to evaluate reliability of LASCA to measure peripheral blood perfusion in scleroderma patients.

Objectives:A reliable tool to evaluateflow is paramount in systemic sclerosis (SSc). We describe herein on theone hand a systematic literature review on the reliability of laser speckle contrast analysis (LASCA) to measurethe peripheral blood perfusion (PBP) in SSc and perform an additional pilot study, investigating the intra- andinter-rater reliability of LASCA.Methods:A systematic search was performed in 3 electronic databases, according to the Preferred ReportingItems for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In the pilot study, 30 SSc patients and 30healthy subjects (HS) underwent LASCA assessment. Intra-rater reliability was assessed by having afirst anchorrater performing the measurements at 2 time-points and inter-rater reliability by having the anchor rater and ateam of second raters performing the measurements in 15 SSc and 30 HS. The measurements were repeated witha second anchor rater in the other 15 SSc patients, as external validation.Results:Only 1 of the 14 records of interest identified through the systematic search was included in thefinalanalysis. In the additional pilot study: intra-class correlation coefficient (ICC) for intra-rater reliability of thefirstanchor rater was 0.95 in SSc and 0.93 in HS, the ICC for inter-rater reliability was 0.97 in SSc and 0.93 in HS.Intra- and inter-rater reliability of the second anchor rater was 0.78 and 0.87.Conclusions:The identified literature regarding the reliability of LASCA measurements reports good to excellentinter-rater agreement. This very pilot study could confirm the reliability of LASCA measurements with good toexcellent inter-rater agreement and found additionally good to excellent intra-rater reliability. Furthermore,similar results were found in the external validatio