Successful treatment with plasma exchange for ANCA-negative pauci-immune crescentic glomerulonephritis with D-negative hemolytic uremic syndrome.

Research paper by Taisuke T Irifuku, Takayuki T Naito, Takahiko T Ogawa, Takao T Masaki

Indexed on: 31 Jul '14Published on: 31 Jul '14Published in: Clinical nephrology


Co-existence of antineutrophil cytoplasmic antibody (ANCA)-negative pauci-immune crescentic glomerulonephritis (CGN) and thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTPHUS) is extremely rare and has a poor renal prognosis. We report a 76-year-old female that had both ANCA and anti-human lysosomal membrane protein 2 (LAMP-2) antibody-negative pauci-immune CGN with D-negative HUS. She was admitted with proteinuria and worsening renal failure with massive crescent formation on renal biopsy specimens. We initiated intravenous methylprednisolone pulse therapy followed by oral prednisolone, but she still developed D-negative HUS. We then initiated plasma exchange, which achieved remission of D-negative HUS and improved renal function. To our knowledge, this is the first report of recovery from renal failure in ANCA-negative pauci-immune CGN with TTP-HUS.