Keywords
Systemic scleroderma
Diffuse scleroderma
Kidney diseases
How to Cite
Abstract
Introduction: Renal involvement due to systemic sclerosis (SS) occurs in 5-15% of patients with diffuse skin involvement, characterized by severe deterioration of renal function, generated by vasculopathy, inflammation and fibrosis.
Purpose: Provide relevant information to improve early recognition of scleroderma renal crisis through the analysis of 2 clinical cases.
Case presentation: Two clinical cases are presented that share characteristics of SS with rapidly progressive skin involvement and kidney disease compatible with scleroderma renal crisis (SRC), manifested as hypertensive crisis and acute kidney injury (AKI), with hemodialysis requirement. Given the suspected diagnosis of SSc, treatment with enalapril was started immediately, achieving control of blood pressure and improvement of intravascular hemolysis. Given the rapid progression of the skin involvement, cyclophosphamide was indicated, presenting improvement in sclerosis in one of the two cases.
Discussion and conclusion: Due to its low frequency and aggressive behavior, SRC continues to be a diagnostic and therapeutic challenge, where prompt treatment represents the difference between achieving clinical results between achieving clinical results, which is why it should be part of the diagnostic approach in patients with AKI and thrombotic microangiopathy, mainly in those who suffer from hypertensive crisis.
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