Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis

Oh, Jin-Won; Kim, Pyung-Kil; Lee, Jae-Seung; Jeong, Hyeon-Joo

doi:2008.12.1.99

J Korean Soc Pediatr Nephrol > Volume 12(1); 2008 > Article

J Korean Soc Pediatr Nephrol 2008;12(1): 99-104. doi: https://doi.org/10.3339/jkspn.2008.12.1.99

급속 진행성 사구체신염으로 시작된 현미경적 다발성 동맥염(Microscopic Polyangiitis) 환자의 7년간의 장기 추적관찰 1례

오진원, 김병길, 이재승, 정현주

¹관동대학교 의과대학 소아과학교실
²관동대학교 의과대학 소아과학교실
³연세대학교 의과대학 소아과학교실
⁴연세대학교 의과대학 병리학교실

Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis

Jin-Won Oh, Pyung-Kil Kim, Jae-Seung Lee, Hyeon-Joo Jeong

¹Department of Pediatrics, Kwandong University College of Medicine
²Department of Pediatrics, Kwandong University College of Medicine
³Departments of Pediatrics, Yonsei University College of Medicine
⁴Departments of Pathology, Yonsei University College of Medicine

ABSTRACT

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

Key words: Microscopic polyangiitis | Immunosuppressant