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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 About the authors

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:
Glomerulonephritis; Mixed Connective Tissue Disease.

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