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

• Main Author: Fernandes,Sara
• Publication Date: 2020
• Other Authors: Teixeira,Catarina, Falcão,Luis Pedro, Costa,Ana Cortesão, Raimundo,Mário, Silva,Sónia, Cardoso,João, Almeida,Edgar De
• Format: Report
• Language: eng
• Source: Jornal Brasileiro de Nefrologia
• Download full: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000200245
• topic: Glomerulonephritis; Mixed Connective Tissue Disease.

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.