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Microscopic polyangiitis complicated by intracerebral hemorrhage and pulmonary hemorrhage in a pediatric patient

Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Jackson Tan, Asrar Hussain, Rajendra Daiwajna, Kian Chai Lim, Edwin Lim, Aye Han

Am J Case Rep 2013; 14:276-279

DOI: 10.12659/AJCR.889064

Available online:

Published: 2013-07-25

Background: MPO ANCA-associated vasculitis is very rare in children. Renal disease is almost universally present but lung and central nervous system involvement are not commonly reported.
Case Report: We present a pediatric case of microscopic polyangiitis with the unusual presentation of pauci-immune glomerulonephritis, intracerebral hemorrhage and pulmonary hemorrhage. The neurological and pulmonary symptoms settled after treatment with cyclophosphamide and plasmapheresis. However, there was no renal recovery and the patient was rendered dialysis-dependent.
Conclusions: We believe that this is the first reported case of pediatric microscopic polyangiitis with central nervous system involvement. The disease may have a subclinical presentation resulting in chronic damage to different organs. Prompt treatment of acute disease with immunosuppression and plasmapheresis can halt disease progression in these patients.

Keywords: Cerebral Hemorrhage, Microscopic polyangiitis, Microscopic polyangiitis, Stroke, pulmonary haemorrhage, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis