Background/Purpose: Systemic sclerosis is characterised by high morbidity and mortality due to disease-related or treatment-related complications.

Methods: Data of patients of systemic sclerosis between 1990 and March 2020 was retrieved from case files and electronic health records (EHR). We determined the number of deaths in our cohort of 789 patients with systemic sclerosis from EHR and teleconsultation, and their causes. Kaplan-Meier curve was used to calculate survival at 1, 2, 5 and 10 years of follow-up duration We evaluated baseline characteristics of patients of systemic sclerosis who experienced mortality and compared them with live controls, after matching for age, sex and duration of follow-up, in a case-control design.

Results: There were a total of 90 deaths. The causes were 32 due to Interstitial lung disease (ILD) related deaths, 7 Gastrointestinal (GI) related, 6 malignancies, 5 Scleroderma renal crisis (SRC), and 3 Pulmonary arterial hypertension (PAH). Most of these patients (19 out of 32, 59.4%) had infection as the cause of death; 5 had pulmonary tuberculosis. Twenty four patients died in a non-hospital setting. Mean age at death was 38 years (Standard error [SE]- 1.24), and median duration of follow-up was 338 days (95% CI- 27-1619). Male- female ration was 8:82. Kaplan-Meier curve showed survival rate at 1, 2, 5 and 10 years of follow-up was 93.7%, 89.5%, 78.9% and 72.1% respectively. On univariable analysis, patients who died had greater number of hospital admissions, higher modified Rodnan skin score (mRSS), a higher frequency of ILD, PAH, cardiac involvement, SRC and history of infections. Using multivariable adjusted analysis, only mRSS, number of admissions and infections were significantly higher compared to live controls.

Conclusion: Interstitial lung disease and related lower respiratory tract infections were the most common cause of mortality in systemic sclerosis. Infections accounted for 32.22% of the deaths. Higher skin score, infections and number of admissions were identified as significant risk factors for mortality in multivariable adjusted analysis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/factors-predicting-mortality-in-an-indian-cohort-of-systemic-sclerosis/