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Anti-neutrophil cytoplasmic auto-antibodies-associated vasculitis with pulmonary and renal involvement

  • Immunology/Allergology
  • Published:
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Abstract

We present a 13-year-old boy with a rapidly progressive glomerulonephritis and pulmonary haemorrhage with perinuclear anti-neutrophil cytoplasmic autoantibodies (pANCA) corresponding to anti-myeloperoxidase antibodies. The diagnosis of microscopic polyarteritis was made on the basis of the clinical features, the positivity of pANCA, and the histological finding of a pauci-immune crescentic glomerulonephritis. He responded excellently to corticosteroids and cyclophosphamide therapy and complete clinical remission persists 1 year after withdrawal of treatment. We emphasize the usefulness of ANCA antibody assays to establish a prompt diagnosis and adequate treatment in systemic vasculitis in children.

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Abbreviations

ANCA:

anti-neutrophil cytoplasm auto-antibodies

cANCA:

cytoplasmic ANCA pattern

pANCA:

perinuclear ANCA pattern

anti-GBM:

anti-glomerular basement membrane

WG:

Wegener granulomatosis

MPAN:

microscopic polyarteritis nodosa

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Authors and Affiliations

  1. Department of Paediatrics, University Hospital “Germans Trias i Pujol”, E-08916, Badalona, Spain

    G. Pintos-Morell, A. Roca-Comas, M. A. Naranjo, G. Javier & J. Prats

  2. Department of Internal Medicine, University Hospital “Germans Trias i Pujol”, E-08916, Badalona, Spain

    C. Tural

  3. Clinical Laboratory, University Hospital “Germans Trias i Pujol”, E-08916, Badalona, Spain

    E. Abad

Authors
  1. G. Pintos-Morell
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  2. A. Roca-Comas
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  3. M. A. Naranjo
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  4. C. Tural
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  5. E. Abad
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  6. G. Javier
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  7. J. Prats
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Pintos-Morell, G., Roca-Comas, A., Naranjo, M.A. et al. Anti-neutrophil cytoplasmic auto-antibodies-associated vasculitis with pulmonary and renal involvement. Eur J Pediatr 152, 473–475 (1993). https://doi.org/10.1007/BF01955052

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  • DOI: https://doi.org/10.1007/BF01955052

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