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Published on: June 16, 2020
Older Age, Steroid Exposure, and Digital Pitting Scars Predict Poor Outcomes in Scleroderma Renal Crisis: A 30-Year
Abhishek Gollarahalli Patel1,2, Sakir Ahmed1,3, Narayan Prasad4
1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Background:
Scleroderma renal crisis (SRC) is a rare but severe complication of systemic sclerosis (SSc), often associated with high morbidity and mortality. Data from Indian population remains limited.
Objectives:
To describe the clinical features, predictors, and outcomes of SRC in a large, single-centre Indian SSc cohort over three decades.
Methods:
This retrospective cohort study included 880 SSc patients diagnosed between 1990 and 2019, classified by ARA 1980 or ACR/EULAR 2013 criteria. SRC was defined by new-onset hypertension and/or rapidly progressive renal failure, with supportive hematologic and urinary findings. Demographic, clinical, and serologic variables were analysed. Predictors of SRC were identified using multivariate logistic regression, and survival was assessed using Kaplan-Meier and Cox regression analyses.
Results:
SRC occurred in 27 patients (3.0%), with 85.2% developing SRC within one year of diagnosis. Steroid use preceded SRC in 66.6% of cases. SRC was associated with older age (OR 1.03, 95% CI 1.001-1.072), digital pitting scars (OR 6.16, 95% CI 1.60-23.65), and reduced by immunosuppressive therapy (OR 0.39, 95% CI 0.16-0.98). SRC patients had a significantly higher mortality risk (HR 3.66, 95% CI 1.94-6.89) and shorter survival (mean 7.2 vs. 23.8 years). Dialysis was required in 51.8% of SRC cases.
Conclusions:
SRC affected 3% of SSc patients and was associated with high mortality and dialysis dependence. Older age, steroid exposure, and digital pitting scars were key risk factors, while immunosuppression appeared protective. These findings highlight the importance of early identification and careful therapeutic strategies in high-risk patients.