Rapidly progressive ANCA-negative glomerulonephritis in the course of pauci immune microscopic vasculitis with hemolytic anemia probable in the course of Wilson's disease

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...

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Published inPrzeglad lekarski Vol. 68; no. 2; p. 123
Main Authors Wieczorek-Surdacka, Ewa, Kaczmarczyk, Ireneusz, Jasik, Piotr, Przepiórkowska-Hoyer, Bernadetta, Sułowicz, Władysław
Format Journal Article
LanguagePolish
Published Poland 2011
Subjects
Adult
Anemia, Hemolytic - complications
Anemia, Hemolytic - diagnosis
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - complications
Antibodies, Antineutrophil Cytoplasmic - immunology
Diagnosis, Differential
Disease Progression
Female
Glomerulonephritis - diagnosis
Glomerulonephritis - immunology
Hepatolenticular Degeneration - complications
Hepatolenticular Degeneration - diagnosis
Humans
Kidney Failure, Chronic - etiology
Prognosis
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Summary: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.
ISSN:0033-2240