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Journal of the Korean Society of Pediatric Nephrology 2001;5(2): 213-218.
폐출혈과 급속 진행성 사구체신염을 동반한 현미경적 다발성 동맥염 (Microscopic Polyangiitis) 1례
권해식, 이영목, 김지홍, 김병길, 강혜윤, 홍순원, 정현주
1연세대학교 의과대학 소아과학교실, 신장질환연구소
2연세대학교 의과대학 소아과학교실, 신장질환연구소
3연세대학교 의과대학 소아과학교실, 신장질환연구소
4연세대학교 의과대학 소아과학교실, 신장질환연구소
5연세대학교 의과대학 병리학교실, 신장질환연구소
6연세대학교 의과대학 병리학교실, 신장질환연구소
7연세대학교 의과대학 병리학교실, 신장질환연구소
A Case of Microscopic Polyangiitis with Pulmonary Hemorrhage and Rapidly Progressive Glomerulonephritis
Hae Sik Kwon, Young-Mock Lee, Ji Hong Kim, Pyung-Kil Kim, Hae Youn Kang, Soon Won Hong, Hyeon Joo Jeong
1Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
2Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
3Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
4Departments of Pediatrics, The Institute of Kidney Disease Yonsei University College of Medicine
5Departments of Pathology, The Institute of Kidney Disease Yonsei University College of Medicine
6Departments of Pathology, The Institute of Kidney Disease Yonsei University College of Medicine
7Departments of Pathology, The Institute of Kidney Disease Yonsei University College of Medicine
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ABSTRACT
Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)
Key words: Antineutrophil cytoplasmic autoantibodies | Systemic vasculitis | Renal biopsy | Alveolar hemorrhage | Gastrointestinal bleeding.
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