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The nephrotic syndrome is an extremely rare occurrence in systemic sclerosis (SSc). Here we describe a 39-year-old woman with SSc who developed the nephrotic syndrome along with the expression of antiribosomal P antibody but not of anti-double-stranded DNA antibody in her serum. Although a renal biopsy specimen showed minimal changes on light microscopy, immunofluorescence studies showed granular deposition of C3 and IgM in the glomeruli. The patient recovered from the nephrotic syndrome with the decrease in serum antiribosomal P antibody after the daily administration of 60 mg of prednisolone. It is strongly suggested that antiribosomal P antibody might have been involved in the development of the nephrotic syndrome in our patient.

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