TY - JOUR T1 - Systemic Sclerosis and Associated Interstitial Lung Disease in Ontario, Canada: An Examination of Prevalence and Survival Over 10 Years JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.201049 SP - jrheum.201049 AU - Janet E. Pope AU - Kobina Quansah AU - Shazia Hassan AU - Soo Jin Seung AU - Jason Flavin AU - Martin Kolb Y1 - 2021/04/01 UR - http://www.jrheum.org/content/early/2021/03/24/jrheum.201049.abstract N2 - Objective Systemic sclerosis (SSc) is a rare autoimmune disease. Pulmonary complications of SSc are one of the leading causes of morbidity and mortality. The objective of this study was to determine prevalence and survival estimates of SSc and SSc-ILD in a Canadian province (Ontario) using administrative data over 10 years. Methods Using ICD-10-CA codes, adult patients diagnosed with SSc and SSc-ILD between April 1, 2008 and March 31, 2018 were identified from the National Ambulatory Care Reporting System (NACRS) and Discharge Abstract Database (DAD) administrative databases. SSc was identified first and ILD if present occurred after the SSc diagnosis. Prevalence estimates were determined for both SSc and SSc-ILD. For survival, Kaplan Meier survival curves were generated. Results At the start of fiscal year 2017/18 (final year of the cohort), there were 2,114 prevalent SSc cases for a cumulative prevalence of 19.1 per 100,000 persons and 257 prevalent cases of SSc-ILD, generating a prevalence of 2.32 cases per 100,000 persons. Mean age was 57 and 58 years with 84% and 80% females for SSc and SSc- ILD patients, respectively. One, 5 and 10 year survival rates respectively for the SSc group were 85.0%, 64.5% and 44.9%, and 77.1%, 44.4% and 22.0% for the SSc-ILD. Conclusion This study provides the first population-based estimates of SSc and SSc- ILD in Canada for prevalence and survival. Results confirm that the prevalence estimates of SSc-ILD falls within the Canadian threshold of rare disease. It also demonstrates the poor survival in SSc especially when ILD is also present. ER -