Survival Of Scleroderma Patients With Interstitial Lung Disease: Observation Data From A Malaysian Tertiary Centre

Background: Interstitial lung disease (ILD) is the leading cause of death in scleroderma (SSc) with decline in FVC as a predictor of mortality in patients with SSc-ILD, especially in the early course of the disease. Objectives: The aim of this study is to determine the survival rate of SSc-ILD in a Malaysian cohort of patients from University Malaya Medical Centre (UMMC). Methods: 61 patients clinically diagnosed with SSc-ILD were identified and prospectively recruited. Baseline demographic data were collected. Kaplan-Meier analysis was used to estimate the survival. Results: Females were predominant (56, 91.8%). 39 (64%) had limited cutaneous SSc. Majority were ethnicity Chinese 30 (49.2%), followed by Malays 20 (32.8%), Indians 7 (11.4%) and others 4 (6.6%). Mean age was 56.25 (SD ± 12.5) years while mean duration of disease (non-Raynaud’s disease onset) was 10.5 years (SD ± 9.2) (range of 1 year to 44 years) years. 29 (47.5%) patients were positive for anti-ScL-70, whereas 6 (9.8%) patients were anti-centromere positive. There were 16 (26.2%) deaths. Median survival was 24 years. Patients had a sharper drop in survival probability for the first 10 years compared to the next 20 years (Figure 1). Median survival in limited subset was 24 years whereas in diffuse subset was 11 years. Patients from the limited subset appeared to have higher chance of surviving for 10 years and above, compared to those in the diffuse subset (Figure 2). Conclusion: The results demonstrate the poor survival in SSc-ILD patients. The survival rate tends to be worse in the first 10 years of SSc disease duration. Survival rate was poorer in patients with diffuse cutaneous subset. References: [1]Parelas A et al. Lancet Resp Med 2020 [2]Goh NS et al. Am J Respir Crit Care Med 2008 Disclosure of Interests: JASMIN RAJA Speakers bureau: For Boehringer Ingelheim for topic on Scleroderma-ILD, Grant/research support from: From Boehringer Ingelheim for scleroderma research, Shantini Muthusamy: None declared., CHOUNG MIN NG: None declared.