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Case Report of Systemic Sclerosis: Uncommon Manifestation of Nephrotic Syndrome Post-Renal Crisis.

Ai Fujii, Takaya Kondo, Yasutaka Katsura,Sho Kumano, Serina Kita,Kazutoshi Nomura, Tomohisa Yabe,Norifumi Hayashi,Keiji Fujimoto,Hitoshi Yokoyama,Kengo Furuichi

SAGE open medical case reports(2023)

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Abstract
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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Key words
Systemic sclerosis,nephrotic syndrome,vasculopathy
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