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

• Main Authors: Sara Fernandes, Catarina Teixeira, Luis Pedro Falcão, Ana Cortesão Costa, Mário Raimundo, Sónia Silva, João Cardoso, Edgar De Almeida
• Format: Article
• Language: English
• Published: Sociedade Brasileira de Nefrologia 2019-03-01
• Series: Brazilian Journal of Nephrology
• Subjects: Glomerulonefrite; Doença Mista do Tecido Conjuntivo.
• Online Access: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002019005012104&lng=en&tlng=en

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.