Pauci-Immune necrotisingglomerulonephritis associatedwith ANCA in two siblings

  1. Manuel Heras 1
  2. María José Fernández-Reyes
  3. Rosa Sánchez
  4. Ana Saiz
  5. Alvaro Molina
  6. Carmen Mon
  7. Elena Ciruelo
  8. Eva Tomero
  9. Fernando Alvarez-Ude
  1. 1 Universidad de Salamanca
    info

    Universidad de Salamanca

    Salamanca, España

    ROR https://ror.org/02f40zc51

Zeitschrift:
Portuguese Journal of Nephrology and Hypertension

ISSN: 2183-1289

Datum der Publikation: 2008

Ausgabe: 22

Nummer: 2

Seiten: 197-200

Art: Artikel

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Andere Publikationen in: Portuguese Journal of Nephrology and Hypertension

Zusammenfassung

Pauci -immune necrotising glomerulonephritiswith extracapillary proliferation is a common renalhistological manifestation of systemic vasculitis.Although its aetiopathogenesis is not well known,it is possible that environmental factors may havea bearing on genetically predisposed subjects,since there are many familial cases of systemicvasculitis. The frequent association between systemic vasculitis and ANCAs suggests autoimmunemechanisms play a part in its pathogenesis. Wereport two cases of necrotising extracapillary proliferative glomerulonephritis in two siblings sharingthe same environment. We do not know if the firstcase had ANCAs, meaning the role of the autoimmune mechanism is not clear (diagnosis was madein 1988 when it was not possible to measureANCAs in this hospital). Since the second case hadthe same kind of illness associated to positiveANCAs and autoimmune hypothyroidism, we presume that both siblings presented the samepathogenesis.These two cases of familial vasculitis appear tosupport the hypothesis that environmental, geneticand autoimmune factors influence the pathogenesisof systemic vasculitis.