消化器症状を契機に3D-CTアンギオグラフィにより診断確定した腎梗塞合併古典的結節性多発動脈炎の一男児例  [in Japanese] Gastrointestinal involvement and renal infarction in a boy with classic polyarteritis nodosa diagnosed with 3D-computed tomography angiography  [in Japanese]

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Abstract

  消化器症状で発症した腎梗塞合併古典的結節性多発動脈炎(PN)の一男児例を経験した.症例は14歳,男児.発熱,腹痛,血性下痢で発症し,体重減少も著しく,前医で行った内視鏡検査で回盲部からS状結腸にかけて多発性の深掘型潰瘍の所見を得て腸管ベーチェットが疑われた.しかし,その病理組織所見は非特異的で,口内アフタ性潰瘍や外陰部潰瘍の既往はなく,結節性紅斑,関節炎などもなく,ブドウ膜炎,針反応も認めないため,ベーチェット病の診断には至らなかった.検査所見ではCRPの上昇,赤沈値の亢進,FDP-Eとフィブリンモノマーの軽度上昇を認め血管炎を想定したが確診には至らず,炎症部位を特定するために3D-CTアンギオグラフィを施行した.この結果,両側腎に多発性腎梗塞と腎動脈瘤,肝動脈の蛇行が認められた.また頭部MRアンギオグラフィでは頭蓋内主要動脈全体の蛇行と拡張も認められ,厚生省(現厚生労働省)特定疾患難治性血管炎分科会認定基準を満たし,PNと診断した.シクロホスファミドパルス・パルス療法を導入し,維持療法として経口プレドニゾロン+アザチオプリンによる治療を行ったところ奏功し速やかな症状の改善を得て,1年後の3D-CTアンギオグラフィによる再検索では両側の多発腎梗塞,腎動脈瘤の消失を認めた.PNの症状や血液検査所見は非特異的であることが多く,従来PNの診断には組織所見や血管造影所見を必要としていた.このため早期の診断,治療導入が困難な症例が少なくなかった.本症例は侵襲性の少ない3D-CTアンギオグラフィにより早期に血管炎の所見を確認でき,速やかな治療導入により後遺症なく炎症抑制が可能であった.<br>

  We report a case of classic polyarteritis nodosa complicated with renal infarction. A 14-year-old boy manifested fever, abdominal pain, watery and bloody diarrhea, and weight loss. Laboratory findings indicated anemia, increased levels of C-reactive protein, and erythrocyte sedimentation rate. Lower gastrointestinal endoscopic examination revealed multiple colorectal ulcerations, and histopathological findings were non-specific, suggesting gastrointestinal involvement of Behcet disease. The patient was referred to our hospital, and suspected to have vasculitis syndrome since the abnormal laboratory findings included persistently increased levels of FDP-E/fibrin monomer as well as inflammatory markers, and the extraordinary high excretion of beta 2-microglobulin, which indicated abrupt and massive expression of HLA class I molecule on endothelial cells due to interferon-gammanemia. To examine the site of vasculitis, 3D-CT angiography was applied to demonstrate bilateral renal infarction and renal artery microaneurysms. Together with the clinical, laboratory, and 3D-CT angiographic findings, he was finally diagnosed as having classic polyarteritis nodosa. After 12 month-course of intravenous cyclophosphamide pulses and prednisolone/azatioprine therapy, complete disappearances of inflammatory manifestations, and renal infarction and microaneurysms were documented. The diagnosis of classic polyarteritis nodosa is frequently delayed because both clinical symptoms and signs, and laboratory findings are not disease-specific, but early diagnosis and treatment are necessary to prevent serious organ damage. In addition to the precise estimation of laboratory findings such as inflammatory markers, and FDP-E/D-dimer/fibrin monomer, the newly developed 3D-CT angiography, a less invasive imaging technique, will be helpful to diagnose patients with classic polyarteritis nodosa, and intervene the disease progression with early and active treatment.<br>

Journal

  • Japanese Journal of Clinical Immunology

    Japanese Journal of Clinical Immunology 31(5), 415-421, 2008-10-31

    The Japan Society for Clinical Immunology

References:  17

Cited by:  1

Codes

  • NII Article ID (NAID)
    10024961486
  • NII NACSIS-CAT ID (NCID)
    AN00357971
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    09114300
  • Data Source
    CJP  CJPref  J-STAGE 
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