ANCA-Positive pauci-immune crescentic glomerulonephritis in a patient with systemic lupus erythematosus

The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury sym...

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Detalles Bibliográficos
Autores: Herrera Añazco, Percy, Velásquez-Castillo, Percy, Pacheco Mendoza, Josmel, Valenzuela-Rodríguez, Germán, Asato-Higa, Carmen
Tipo de recurso: artículo
Fecha de publicación:2017
País:Perú
Institución:Universidad San Ignacio de Loyola
Repositorio:USIL-Institucional
Idioma:español
OAI Identifier:oai:repositorio.usil.edu.pe:20.500.14005/8562
Acceso en línea:https://hdl.handle.net/20.500.14005/8562
http://dx.doi.org/10.5935/0101-2800.20170079
http://bjn.org.br/details/1994/en-US/anca-positive-pauci-immune-crescentic-glomerulonephritis-in-a-patient-with-systemic-lupus-erythematosus
Access Level:acceso abierto
Palabra clave:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Glomerulonephritis
Lupus Erythematosus, Systemic
Descripción
Sumario:The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule. 67% of the glomeruli had fibroepithelial crescents. Moreover, the interstitial space had a moderate lymphomononuclear infiltration and mild fibrosis. In the arterioles, there were walls thickened by subintimal sclerosis. Direct immunofluorescence detected limited IgM and C3 deposits in capillary loops and negative mensangium for IgG, IgA and C1q. A therapy using corticosteroids and intravenous cyclophosphamide was initiated with stable evolution. PICGN associated with SLE is a rare pathology with clinical presentation, varied evolution and without a standard medical treatment.