Brazilian Journal of Nephrology (Mar 2019)

Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study

  • Sara Fernandes,
  • Catarina Teixeira,
  • Luis Pedro Falcão,
  • Ana Cortesão Costa,
  • Mário Raimundo,
  • Sónia Silva,
  • João Cardoso,
  • Edgar De Almeida

DOI
https://doi.org/10.1590/2175-8239-jbn-2019-0003
Journal volume & issue
no. 0

Abstract

Read online

Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.

Keywords