血尿を呈した,成人発症のC1q腎症の1例 Case Report : Adult onset of C1q nephropathy manifesting only as microhematuria

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Abstract

症例は27歳男性。5年前より顕微鏡的血尿を指摘されていた。仕事で海外駐在の予定があり,赴任前に血尿の精査目的にて入院となった。経皮腎生検を行い,光顕所見ではメサンギウム増殖性糸球体腎炎の所見を認めた。蛍光抗体法にてメサンギウム領域に主としてC1qの沈着を認めた。同部位にC3の僅かな沈着も認めたが,IgA,IgG,IgMの有意な沈着は認めなかった。電顕ではメサンギウム領域にdense depositsを認めた。一方,臨床症状または採血結果からは,膠原病を疑わせる所見はなかった。以上の所見よりC1q腎症と診断した。一般的にC1q腎症では蛋白尿が主体の症状であり,場合によってはネフローゼ症候群を呈することもある。1994年4月から2010年12月までの当科での腎生検施行全3614例中,C1q腎症と診断される患者は他に6例あったが,本症例のみが血尿単独を呈していた。本症例はC1q腎症の発症メカニズムを考えるうえで示唆に富む症例と考えられたので,若干の考察を加えて報告する。

We report a case of C1q nephropathy that manifested only as microhematuria in a 27-year-old man. The patient had presented with microhematuria for five years. A light microscopic study of a renal biopsy specimen showed predominant mesangial C1q deposition. Staining for C3 was positive in the same mesangial area. Stainings for IgA, IgG and IgM were slightly positive, however, these stainings were not on mesangial lesion, suggesting as non-specific pattern. Electron microscopy indicated high electron-dense deposits in the mesangial area. Histopathological studies were compatible with C1q nephrology. In addition, laboratory data showed no serological evidence of collagen disease. Usually proteinuria, occasionally nephrotic syndrome were reported in C1q nephropathy and C1q nephropathy often cause severe damage in the glomerulocapillary area from an early stage. From April 1994 to the end of 2010, we experienced 6 patients with possibility of C1q nephropathy among the all 3614 patients in whom renal biopsy was studied, and the prevalence of C1q nephropathy was 0.17%. All of 6 patients showed continuous microhematuria and proteinuria, and 2 of 6 cases had nephrotic syndrome. Current patient showed only hematuria without proteinuria. Hematuria alone is not a major manifestation of C1q nephropathy. Thus, the clinical findings in our patient may represent a rare phenotype of C1q nephropathy. We report the details of the case with a comparison with previous C1q cases.

Journal

  • Jichi Medical University journal

    Jichi Medical University journal 34(-), 67-74, 2011

    Jichi Medical University

Codes

  • NII Article ID (NAID)
    110008922589
  • NII NACSIS-CAT ID (NCID)
    AA12194963
  • Text Lang
    ENG
  • Article Type
    departmental bulletin paper
  • Journal Type
    大学紀要
  • ISSN
    1881-252X
  • NDL Article ID
    023691374
  • NDL Call No.
    Z19-965
  • Data Source
    NDL  NII-ELS  IR 
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