Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Sch$\ddot{o}$nlein Purpura Nephritis

Henoch-Sch$\ddot{o}$nlein 자반병 신염으로 의심했던 현미경적 다발혈관염 1례

  • Im, Jong Geun (Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine) ;
  • Moon, Kyung Chul (Department of Pathology, Seoul National University College of Medicine) ;
  • Koo, Ja Wook (Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine)
  • 임종근 (인제대학교 상계백병원 소아청소년과) ;
  • 문경철 (서울대학교 의과대학 병리학교실) ;
  • 구자욱 (인제대학교 상계백병원 소아청소년과)
  • Published : 2012.10.31
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Abstract

Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.

Henoch-Sch$\ddot{o}$nlein 자반병 신염과 유사한 증상을 가지는 10세 여자에게 발생한 급격한 신기능 저하를 동반하지 않은 현미경적 다발혈관염 1례를 경험하였기에 보고하는 바이다. 이 혈관염의 경우. 진단 시점 또는 치료 시작할 때의 신장기능 저하가 심할수록 병의 예후가 나쁘므로[20], 사구체 여과율이 떨어지기 전에 빠른 진단과 치료가 필요하다. Henoch-Sch$\ddot{o}$nlein 자반병이 의심될 경우 단백뇨와 혈뇨가 지속되면, 다른 혈관염과의 감별을 위해 ANCA, 신장 조직검사가 도움을 줄 것으로 생각된다.

Keywords

References

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