title: Successful treatment with plasma exchange for ANCA-negative pauci-immune crescentic glomerulonephritis with D-negative hemolytic uremic syndrome.
abstract: 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.
authors: Taisuke
authors: T
authors: Irifuku
authors: Takayuki
authors: T
authors: Naito
authors: Takahiko
authors: T
authors: Ogawa
authors: Takao
authors: T
authors: Masaki
mesh_terms: D061605:Administration, Intravenous; D000284:Administration, Oral; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006463:Hemolytic-Uremic Syndrome / Q000628:therapy*; D006801:Humans; D052119:Lysosomal-Associated Membrane Protein 2 / Q000276:immunology; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D010951:Plasma Exchange / Q000379:methods*; D011239:Prednisolone / Q000008:administration & dosage; D011507:Proteinuria / Q000652:urine; D020551:Pulse Therapy, Drug; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; C497811:LAMP2 protein, human; D052119:Lysosomal-Associated Membrane Protein 2; D011239:Prednisolone; D008775:Methylprednisolone