Fri0322 autoantibodies Profile Influence on Systemic Sclerosis Interstitial Lung Disease. A Tertiary Spanish Hospital Experience

Abstract

BackgroundSystemic Sclerosis (SSc) is a rare and heterogeneous connective tissue disease (CTD) characterized by skin fibrosis, vasculopathy/vascular damage and potential visceral impairment. Interstitial lung disease (ILD) constitutes the leading cause of mortality and requires close periodical assessment and follow-up. Diffuse cutaneous sclerosis and specific autoantibody profile (anti SCL70, anti Th/To, Anti U3 RNP and anti PmScl) are considered ILD development risk factors. In contrast, positivity to anticentromere has been considered as a protective factor to develop a clinical significant ILD.ObjectivesTo assess ILD frequency and severity (extension and functional impairment) in SSc patients, analyzing the association with the different autoantibodies.MethodsRetrospective, descriptive study of patients meeting EULAR/ACR 2013 SSc criteria and had a HRTC performed at a tertiary Spanish hospital from 1975 to 2018. One hundred and eight patients were included. HRCTs were graded by two radiologists according to Goh et al semiquantitative score. Three groups were established according to the presence of different autoantibodies: anticentromere (ACA), antitopoisomerase (ATA), and positivity to other ANAs (nucleolar pattern and other specificities).ResultsClinical and laboratory characteristics are presented in table 1. Thirty-three patients had pulmonary involvement, 6 were ACA+, 18 ATA+ and 9 had other ANA specificities. The probability of not having pulmonary involvement among ACA+ and of having pulmonary involvement among ATA + showed statistic significance (p<0.001 and p<0.001). Usual intestinal Pneumonia (UIP) was the most frequently reported pattern (6 patients), followed by Non-Specific Interstitial Pneumonia (NSIP 11 patients); Six patients did not meet the radiological criteria for neither UIP or NSIP. No statistical significant difference was found among radiological pattern and autoantibody profile. Regarding extension: 15 patients had non-extensive involvement (ACA: 2, ATA: 7, “other ANAs”: 6) performing Goh et al score with the FVC correction. Extensive ILD was found among 18 patients (ACA: 4, ATA: 11, “other ANAs”: 3). Despite the fact that ATA+ group was more likely to have an extensive involvement no statistical significance was met. About pulmonary function tests (PFT) the mean FVC was 98,45% on ACA group, whereas on ATA and other ANAs groups the mean FVC were 88,97% and 81,43% respectively. Statistical differences were found between first and third group, However this difference was not found when only patients with pulmonary involvement were analyzed.ConclusionIn our cohort, ATA and other ANAs groups were more likely to develop pulmonary interstitial disease and to need inmunosuppressant treatment. Although the prevalence of ILD among ACA+ was only 10%, 50%of them required, due to the functional impairment or extension, inmunosuppressant treatment. In spite of the low ILD-ACA+ incidence this complication should be kept in mind and periodical screening and assessment must be carried out as in other...