AB0726 Interstitial lung disease in scleroderma patients – a portuguese portrait
Authors: A.C. Duarte, A. Cordeiro, T. Santiago, M.J. Salvador, M.J. Santos
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Systemic sclerosis (SSc) is characterised by inflammation and fibrosis of skin and internal organs. Lung involvement, particularly interstitial lung disease (ILD), is a major cause of morbidity and a leading cause of SSc-related mortality. <h3>Objectives</h3> To characterise SSc patients (pts) with ILD and identify possible predictors for its development. <h3>Methods</h3> A multicenter prospective cohort study using the Rheumatic Diseases Portuguese Register/Scleroderma database was performed. All patients fulfilled ACR/<sup>EULAR 2013</sup> classification criteria for SSc. Demographic and clinical data until 15th January 2018 were collected as well as pulmonary function tests (PFTs), including diffusing capacity of carbon monoxide (DLCO). ILD was defined by fibrosis in chest x-ray and/or high-resolution computed tomography (HRCT) scan. Disease progression was defined by an increase from baseline in the area of involved parenchyma and/or development of honeycombing in pts with previous ground glass imaging and/or reduction of more than 15% in DLCO or 10% in forced vital capacity in pts with stablished ILD. We performed a descriptive analysis of SSc cohort. Logistic regression analysis was used to identify variables independently associated with ILD. <h3>Results</h3> A total of 146 pts were included, 88.4% female, with a mean age of 61.5±14.4 years (yrs) and mean disease duration of 14.3±11.3 years. Limited cutaneous SSc (lSSc) was present in 66.4%, diffuse cutaneous SSc (dSSc) in 24%, overlap syndrome in 6.8% and very early diagnosis SSc in 1.4% (information missing in 2pts). In our cohort, 42 pts (28.8%) presented ILD after 17.5±14.1 years of disease duration, 83.5% of them were female; 45.2% had dSSc and 42.9% lSSc. Anti-Scl70 antibody occurred in 23 pts, anticentromere antibody (ACA) in 8, anti-Pm/Scl in 2, anti-RNA polymerase I in 2, anti-RNA polymerase III in 1 and anti-U3RNP in 1. Ten pts had current/previous smoking habits. Nailfold capillaroscopy (NCP) was available in 23 out of 42 pts and 21 had a SSc pattern (5 early, 6 active and 10 late). Table 1 shows differences between pts with and without ILD. In multivariate analysis, anti-Scl70 (OR:3.7 95% CI 1.3–10.5) and current/previous digital ulcers (OR:2.2 95% CI 1.9–5.3) were associated with ILD development. ACA had a protective effect (OR:0.3 95% CI 0.1–0.8). Mycophenolate mofetil was used in 11pts, with 5 of them having previous treatment with cyclophosphamide (CYC; 4 as induction drug). Four pts received azathioprine, half of them as maintenance therapy after induction with CYC. Two pts received rituximab and 1 nintedanib. During follow-up, disease progression occurred in 17 pts (40.1%), 3 of them under immunosuppression. Death occurred in 7 pts with ILD, 5 of them with direct relation to lung disease, 14.4 years after the diagnosis. Table 1 Differences between patients with and without ILD (total=146) <h3>Conclusions</h3> This was the first multicentre Portuguese study about ILD in SSc. In our cohort, ILD occurred in about 30% of the pts and was associated with a mortality rate of 3.4%. Our results confirm that some variables are associated with pulmonary disease development, and thus its identification may help the clinician to detect pts with a higher risk of early ILD. <h3>Disclosure of Interest</h3> None declared