A case of membranous nephropathy preceding diagnosis of mixed connective tissue disease

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  • Watanabe Kotaro
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
  • Udagawa Tomohiro
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
  • Okutsu Mika
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
  • Shimbo Asami
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
  • Akutsu Yuko
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University
  • Yamazaki Susumu
    Department of Lifetime Clinical Immunology Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Mori Masaaki
    Department of Lifetime Clinical Immunology Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Tanaka Eriko
    Department of Pediatrics, JA Toride Medical Center/Department of Pediatrics Kyorin University School of Medicine
  • Tomii Shohei
    Division of Surgical Pathology, Tokyo Medical and Dental University
  • Miura Kenichiro
    Department of Pediatric Nephrology, Tokyo Women’s Medical University
  • Hattori Motoshi
    Department of Pediatric Nephrology, Tokyo Women’s Medical University
  • Morio Tomohiro
    Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University

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Other Title
  • 混合性結合組織病の診断に先行した膜性腎症の1例

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Abstract

<p>Mixed connective tissue disease (MCTD) is characterized by positivity to anti-U1-ribonucleoprotein (RNP) antibodies and presents with symptoms of systemic lupus erythematosus, dermatomyositis/polymyositis, and scleroderma. MCTD-associated nephropathy unusually precedes extrarenal involvement, although pediatric-onset MCTD complicates nephropathy more frequently than the adult-onset type. We experienced the case of a 13-year-old girl who was diagnosed with membranous nephropathy (MN) without extrarenal involvement following school urine screening, which was subsequently confirmed as MCTD. Her urine was positive for protein and occult blood; further examination revealed positive antinuclear antibodies and strongly positive anti-U1-RNP antibodies. The proteinuria and hematuria worsened; subsequently, thorough examination including kidney biopsy was performed. Although the criterion for MCTD was not fulfilled at that point, the diagnosis was MN based on renal glomerular histology; thereafter, corticosteroid and immunosuppressive therapy commenced. Raynaud’s phenomenon appeared later; therefore, MCTD was diagnosed. It was a rare disease course where nephropathy preceded extrarenal manifestations, such as Raynaud’s phenomenon.</p>

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