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

  • Rakesh Kumar Pilania
  • Sumeet R. Dhawan
  • Joseph L. Mathew
  • Surjit Singh
  • K. S. Sodhi
  • Meenu Singh
Clinical Brief

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 

References

  1. 1.
    Bosch X, Font J, Mirapeix E, Revert L, Ingelmo M, Urbano-Marquez A. Antimyeloperoxidase autoantibody-associated necrotizing alveolar capillaritis. Am Rev Respir Dis. 1992;146:1326–9.CrossRefPubMedGoogle Scholar
  2. 2.
    Yu F, Zhao MH, Huang JP, et al. Clinical and pathological features of anti-neutrophil cytoplasm antibody associated systemic vasculitis in children. Zhonghua Er Ke Za Zhi. 2003;41:831–4.PubMedGoogle Scholar
  3. 3.
    Villiger PM, Guillevin L. Microscopic polyangiitis: clinical presentation. Autoimmun Rev. 2010;9:812–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Lauque D, Cadranel J, Lazor R, et al. Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM"O"P). Medicine (Baltimore). 2000;79:222–33.CrossRefGoogle Scholar
  5. 5.
    Jin JJ, Shi JH, Lu WX, Zhu YJ. Clinical features of pulmonary involvement in patients with microscopic polyangiitis. Zhonghua Jie He He Hu Xi Za Zhi. 2011;34:339–43.PubMedGoogle Scholar
  6. 6.
    Homma S, Suzuki A, Sato K. Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. Clin Exp Nephrol. 2013;17:667–71.CrossRefPubMedGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 2017

Authors and Affiliations

  • Rakesh Kumar Pilania
    • 1
  • Sumeet R. Dhawan
    • 1
  • Joseph L. Mathew
    • 1
  • Surjit Singh
    • 1
  • K. S. Sodhi
    • 2
  • Meenu Singh
    • 1
  1. 1.Department of PediatricsPostgraduate Institute of Medical Education and ResearchChandigarhIndia
  2. 2.Department of Radiodiagnosis and ImagingPostgraduate Institute of Medical Education and ResearchChandigarhIndia

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