Kidney involvement in systemic sclerosis occurs in about 20% of cases, with scleroderma renal crisis as a significant complication. However, cases of glomerular disease with massive proteinuria are rare. We present a unique case of systemic sclerosis with the development of nephrotic syndrome. The report provides clinical details and podocyte pathological findings. A 40-year-old male with prior skin sclerosis was diagnosed with systemic sclerosis. Treatment with oral prednisone led to gradual improvement, but a year later, he experienced a systemic sclerosis renal crisis. Using the angiotensin converting enzyme (ACE) inhibitors improved kidney function. However, 3 months later, nephrotic syndrome was diagnosed. Despite an increased prednisolone dose, proteinuria persisted. A kidney biopsy revealed glomerular sclerosis and characteristic vascular changes. Immunofluorescent studies showed no deposits. Electron microscopy confirmed podocyte abnormalities.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10750524PMC
http://dx.doi.org/10.1177/2050313X231220801DOI Listing

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