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Background: Interstitial lung disease (ILD) in systemic sclerosis (SSc) represents a negative impact on their quality of life and in mortality1. Anti-Ro52 positivity in ILD-SSc patients has shown a decrease in FVC and an increase in mortality2. Factors leading to progressive form of ILD and Progressive Pulmonary Fibrosis (PPF) have been described 3. However, the involvement of anti-Ro52 positivity in the development of PPF among SSc-ILD patients remains not elucidated. Objectives: Primary outcome: assesing the relationship between anti-Ro52 and the development of PPF in patients with SSc-ILD. Secondary outcomes: describing SSc and ILD-SSc clinical features in anti-Ro52 positive patients, assessing changes in respiratory function tests during the initial 5 years of disease progression. Methods: Retrospective cohorts. SSc-ILD patients attended at the Germans Trias i Pujol Hospital between 2008 and 2018 were included. Baseline was defined as the date of the diagnosis of ILD-SSc. Information of clinical SSc characteristics, clinical-demographic variables and risk factors ILD was collected. Results: Thirty patients with SSc-ILD were included (22 females and 8 males) with a mean age of 59.6 years (SD 14.6), 46,8% were smokers and 15.6% had epidemiological risk factors for ILD. Median duration of follow-up was 67.3 months (SD 22.3). Seven patients met ATS criteria for PPF (4 UIP, 2 NSIP, 1 indefined). Clinical SSc characteristics are shown in Table 1 and immunological features in Table 2. Six patients were anti-Ro52+. Four patients presented diffuse cutaneous SSc and 2 limited cutaneous SSc. Baseline mean FVC was 75.3% (SD 34.4) and baseline mean DLCO was 61% (SD 8), whereas in the control group was FVC 81.2% (SD 18.8) (p=0.467) and DLCO 55.3% (SD 8) (p=0.399) The radiological patterns found were 2 NSIP (33.3%), 4 UIP (66.6%). When compared to the control group no significant differences were found in the prevalence of NSIP (33.3% vs 41.7%) and UIP (66.7% vs 29.2%) (p=0.199). None of them presented respiratory insufficiency. None of them met ATS criteria for PPF at 5 year follow-up, whereas 7 patients met PPF criteria in the control group (p= 0.290). No differences were found in the relative FVC loss at 5-year follow up (Ro52 + 2.8% vs control group –2.6%, p=0.362). Conclusion: No conclusive evidence was found to support a significant relationship between anti-Ro52 positivity and FFP. While the absence of statistical significance may suggest a lack of a strong association, it is important to note the trends of a lower FVC at baseline and a higher prevalence of UIP in anti-Ro52 that warrant further exploration. REFERENCES: 1 Frantz C, Avouac J, Distler O, Amrouche F, Godard D, Kennedy AT, Connolly K, Varga J, Matucci-Cerinic M, Allanore Y. Impaired quality of life in systemic sclerosis and patient perception of the disease: A large international survey. Semin Arthritis Rheum. 2016 Aug;46(1):115-23. doi: 10.1016/j.semarthrit.2016.02.005. Epub 2016 Feb 26. PMID: 27132536. 2 Sánchez-Montalvá A, Fernández-Luque A, Simeón CP, Fonollosa-Plà V, Marín A, Guillén A, Vilardell M. Anti-SSA/Ro52 autoantibodies in scleroderma: results of an observational, cross-sectional study. Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-177-82. Epub 2014 Nov 5. PMID: 25372801. 3 Hoffmann-Vold AM, Allanore Y, Alves M, Brunborg C, Airó P, Ananieva LP, Czirják L, Guiducci S, Hachulla E, Li M, Mihai C, Riemekasten G, Sfikakis PP, Kowal-Bielecka O, Riccardi A, Distler O; EUSTAR collaborators. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2021 Feb;80(2):219-227. doi: 10.1136/annrheumdis-2020-217455. Epub 2020 Sep 28. PMID: 32988845; PMCID: PMC7815627. Table 1. Clinical SSc characteristics (GI, gastrointestinal). Table 2. Immunological features including frequencies for ANA patterns (Anti-Scl70,Autoantibodies against topoisomerase I; Anti-CENA,Anti-centromere protein A antibodies; Anti-CENB, Anti-centromere protein B antibodies; Anti-RNA pol III, Anti-RNA polymerase III antibodies) Acknowledgements: NIL. Disclosure of Interests: None declared.
Calomarde-Gómez et al. (Sat,) studied this question.