AB0665 OUTCOME OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH SCLERODERMA, A 5 YEAR PROSPECTIVE COHORT STUDY
Authors: Akash Khune
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Systemic sclerosis (SSc) is a multi-system disorder characterized by a disturbance in fibroblast function, microvascular disease, and immune system activation, resulting in fibrosis of the skin and internal organs. <h3>Objectives</h3> To study changes in lung function in patients with scleroderma with ILD on treatment followed up prospectively over a period of 5 years <h3>Methods</h3> In this study, 73 patients with scleroderma with ILD were prospectively followed up from July 2013 to July 2018. Patients' clinical features, investigations, treatment and complications were recorded at regular interval <h3>Results</h3> Of 73 patients, 68 were females and 5 were males. 4 had only one visit and 11 had incomplete data. Baseline characteristics are shown in table 1. Mean PAH at baseline was 35.95±14.26 mmHg and at end of study was 39.44±14.44 mmHg. Immunosuppressant's used were MMF in 52, cyclophosphamide in 15, azathioprine in 13, and methotrexate in 17 patients. 61 patients received oral prednisolone with mean dose of 6.46±7.10 mg/day during maintenance and mean dose 15±8.95 mg/day during exacerbation of ILD. There was no statistically significant difference in mean FVC of 59 patients at baseline (59.76±16.91) and at end of the study (57.03±16.77) and in subgroup analysis of patients who received MMF (p &gt; 0.05).Mean 6MWD at baseline was 370±58.83 meters and at end of study was 377±88.96 meters. Comorbidities seen were hypertension in 15, DM in 6, osteoporosis in 23 (of which one had vertebral fracture), aVN in 3 patients. 5 patients died because of ILD. 13 patients needed home O2. <h3>Conclusion</h3> This prospective study was carried out with aim to determine progression of ILD in patients with systemic sclerosis with ILD over period of 5 years.In our study, there was no statistically significant change of FVC over period of time. Worsening of lung function with deterioration in FVC &gt;10% was seen in only 13 patients (22%) (P&lt;0.01).Our findings are similar to study done by Le Gouellec etal in which 75 patients with scleroderma and ILD were followed over period of 72 months of which 26% patients showed significant worsening of FVC (≥ 10%)<sup>1.</sup>In our study there statistically significant decline in DLCO and increase in PAH over period of 5 years(p&gt;0.001). In study carried out by Le Goullec et at studying Scleroderma/ILD, there was significant decline in DLCO over period of 72 months of follow up<sup>1.</sup> in study done by Stephen C. Mathai et al, there was progressive increase in PAH when patients with scleroderma and ILD with PAH were followed up for period of 4 years<sup>2</sup>.. <h3>References</h3> [1] Le Gouellec N, Duhamel a, Perez T, et al. Predictors of lung function test severity and outcome in systemic sclerosis-associated interstitial lung disease. PLoS one. 2017;12(8):e0181692. Published2017Aug1. doi:10.1371/journal.pone.0181692 [2] Stephan mathai etal,ARTHRITIS &amp; RHEUMATISM,February2009, pp 569–577DOI10.1002/art.24267 © 2009, american College of Rheumatology <h3>Disclosure of interests</h3> None declared