RESUMO
Basement membranes form an anatomic barrier that contains connective tissue. They are composed of type IV collagen, laminin and proteoglycans. Anti-basement membrane antibodies bind to the non-collagen site of the α3 chain of type IV collagen. A group of renal diseases, pulmonary diseases and perhaps others affecting different organs have long been associated with the presence of antibodies directed against glomerular basement membrane (GBM), alveolar basement membrane and tubular basement membrane. Goodpasture disease has a frequency of 0.5 to 1 case by million/year, and is responsible for up to 20% of crescentic glomerulonephritis in renal biopsy. It has been associated with genetic and immune abnormalities and there are usually environmental triggers preceding clinical onset. Renal disease can occur isolated or in association with pulmonary hemorrhage. In general, renal disease has a rapid progression that determines severe compromise, with rare spontaneous resolution. The diagnosis of Goodpasture disease requires the presence of the anti-GBM antibody, either in circulation or in renal tissue. The prognosis of non-treated patients is poor. The standard of care is plasma exchange combined with prednisone and cyclophosphamide. Anti-GBM antibody levels must be monitored frequently until their disappearance, and then every 6 months to confirm sustained remission in the absence of clinical signs of recurrence. Prognosis of the disease is strongly associated with its initial presentation. Survival rates are related to the degree of renal compromise at onset of the disease. Recurrence of the disease post-transplantation is low.
Assuntos
Doença Antimembrana Basal Glomerular , Autoanticorpos/sangue , Membrana Basal Glomerular , Imunossupressores/uso terapêutico , Troca Plasmática/métodos , Doença Antimembrana Basal Glomerular/diagnóstico , Doença Antimembrana Basal Glomerular/imunologia , Doença Antimembrana Basal Glomerular/fisiopatologia , Doença Antimembrana Basal Glomerular/terapia , Ciclofosfamida/uso terapêutico , Progressão da Doença , Membrana Basal Glomerular/imunologia , Membrana Basal Glomerular/patologia , Humanos , Testes de Função Renal , Monitorização Imunológica/métodos , Prednisona/uso terapêutico , PrognósticoRESUMO
El síndrome de Goodpasture es más frecuente en varones, adultos jóvenes. Se presento una paciente de sexo femenino de doce años de edad con historia de palidez, astenia y adinamia, expectoración hemoptoica y dificultad respiratoria rápidamente progresiva, anemia microcítico y falla renal aguda que evolucionó con insuficiencia respiratoria grave e irreversible secundario a hemorragia pulmonar masiva. Los exámenes anatomopatológicos mostraron hemosiderosis pulmonar y glomerulonefritis crecéntica, compatibles con síndrome de Goodpasture
Assuntos
Humanos , Feminino , Doença Antimembrana Basal Glomerular/diagnóstico , Antibacterianos/uso terapêutico , Causas de Morte , Diagnóstico Diferencial , Glomerulonefrite , Hemossiderose , Ferro/uso terapêutico , Doença Antimembrana Basal Glomerular/fisiopatologia , Doença Antimembrana Basal Glomerular/tratamento farmacológicoRESUMO
El síndrome pulmón riñón se caracteriza por hemorragia alveolar severa y glomerulonefritis. Obedece a variadas etiologías, siendo la vasculitis la causa más frecuente. Recientemente se ha establecido que aquellas vasculitis que comprometen a los capilares, alveolares y glomerulares, cursan con síndrome pulmón riñón. Histológicamente se expresan con una inflamación necrotizante de los capilares alveolares y con glomerulonefritis necrotizante y crescéntica en el riñón. El descubrimiento de los ANCA (Antineutrophil Cytoplasmic Antibodies) a permitido mejorar nuestra comprensión del síndrome pulmón riñón. En este artículo se revisa a la luz de los conocimientos actuales, el diagnóstico etiológico de este síndrome. Considerando que las alteraciones histológicas a nivel pulmón son inespecíficas, el diagnóstico etiológico debe realizarse en base a las manifestaciones clinicopatológicas del compromiso extrapulmonar y/o de las alteraciones serológicas características