title: [Rapidly progressive ANCA-negative glomerulonephritis in the course of pauci immune microscopic vasculitis with hemolytic anemia probable in the course of Wilson's disease].
abstract: Pauci-immune glomerulonephritis, i.e., with no evidence of immune deposits in the blood vessel, is the most prevalent form of rapidly progressive glomerulonephritis (RPGN). In the pathogenesis of pauci-immune renal disease inflammation of blood vessels in the presence of circulating anti-neutrophil cytoplasm antibodies (ANCA) takes place. However the lack of ANCA (about 5-30% of patients) does not exclude pauci-immune vasculitis. The patients without circulating ANCA might have fewer extrarenal symptoms than those who are ANCA-positive. We describe a case of a 40-year old women with ANCA-negative renal limited pauci-immune small-vessel vasculitis with rapidly decreasing kidney function. She was ineffectively treated with plasmapheresis combined with a puls of cyclophosphamide (i.v.) and 3 pulses of methyloprednisolone (i.v.). The patient progressed to end-stage renal disease and should be treated with renal replacement therapy. In differential diagnosis we excluded other causes of pauci-immune vasculitis (Churg-Strauss syndrome, Wegener's granulomatosis), vasculitis with immune complexes deposition (systemic lupus erythematosus, Schoenlein-Henoch purpura, post-infection RPGN), Goodpasture disease, haemolytic-uremic syndrome (HUS), disseminated intravascular coagulation (DIC) and Wilson's disease.
authors.affiliation: Katedra i Klinika Nefrologii, Uniwersytet Jagielloński, Collegium Medicum w Krakowie.
authors.forename: Ewa
authors.initials: E
authors.lastname: Wieczorek-Surdacka
authors.forename: Ireneusz
authors.initials: I
authors.lastname: Kaczmarczyk
authors.forename: Piotr
authors.initials: P
authors.lastname: Jasik
authors.forename: Bernadetta
authors.initials: B
authors.lastname: Przepiórkowska-Hoyer
authors.forename: Władysław
authors.initials: W
authors.lastname: Sułowicz
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications* / Q000175:diagnosis; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000276:immunology*; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D011379:Prognosis
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic