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Indian J Pediatr. 2017 Oct;84(10):799-801. doi: 10.1007/s12098-017-2379-0. Epub 2017 Jun 2.

ANCA-associated Vasculitis Presenting as Severe Pulmonary Hypertension and Right Heart Failure.

Author information

1
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
2
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India. dr.joseph.l.mathew@gmail.com.
3
Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Abstract

Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor.

KEYWORDS:

Microscopic polyangiitis; Pulmonary hypertension; Vasculitis

Comment in

PMID:
28577200
DOI:
10.1007/s12098-017-2379-0
[Indexed for MEDLINE]

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