ANCA-associated Vasculitis Presenting as Severe Pulmonary Hypertension and Right Heart Failure
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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.
KeywordsMicroscopic polyangiitis Pulmonary hypertension Vasculitis
RKP, JLM and MS cared for the patient, reviewed the literature and wrote the manuscript. RKP, SRD, JLM, SS and MS conceptualised the manuscript, reviewed the literature and contributed to the writing. KS evaluated the patient’s radiologic findings. JLM will act as guarantor for the paper.
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