Multisystem Complications and Therapeutic Trends in Scleroderma Renal Crisis: Insight from a Five-Year Retrospective Cohort Study

Background: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc), often presenting with acute kidney injury (AKI) and severe hypertension. Despite advancements in management data on recent trends in demographics and outcomes remain limited. Methods: We utilized the TrinetX database, aggregating EHR from 144 global healthcare organizations. 5,582 adult patients (≥18 years) diagnosed with SRC using diagnosis date as an index event were examined for demographics and outcomes within 5 years. Outcomes of interest included mortality, cardiovascular, renal complications and medications (Angiotensin converting enzyme inhibitor (ACEi), Angiotensin receptor blocker (ARB), steroids and biologics). Results: The study included 5,582 patients with mean age of 62.4±15.4 years and had predominantly female (80.5%) and White (66.02%) patients. Major complications: Mortality in 16.0%. Renal: Acute renal failure in 12.0% ESRD in 2.8%, renal transplant in 0.7%, Hemodilaysis in 2.1%, Cardiovascular: NSTEMI in 2.8%, STEMI in 0.8%, Neurological: Stroke in 2.3%, Pulmonary: Edema in 2.4%, Pulmonary Hypertension in 16.8%, Pulmonary fibrosis in 7.3%, Treatment: Antihypertensives: ACEi (Captopril in 2.4%, enalapril in 1.2%, and ARBs: Losartan in 5.6%, valsartan in 2.4%). Immune suppressants: Hydroxychloroquine (HCQ) in 7.9%, methotrexate (MTX) in 3.7%, mycophenolate in 12.7%, rituximab in 4.1%, prednisone in 18.4%, methylprednisone in 18.3% of the cohort. Conclusion: Our retrospective cohort study highlights the significant mortality and multisystem complications despite high immunosuppression use. Low utilization of ACEi and ARBs indicates potential therapeutic gaps. Further research to refine treatment strategies is essential to improve mortality and patient outcomes.