Enfermedad antimembrana basal glomerular con hemorragia alveolar aislada
2020
espanolLa enfermedad antimembrana basal glomerular, tambien llamada enfermedad de Goodpasture, es una enfermedad rara autoinmune. Se trata de un subtipo de vasculitis sistemica de pequeno vaso cuya patogenia se caracteriza por la presencia de autoanticuerpos dirigidos principalmente a la membrana basal glomerular y alveolar. El epitopo especifico de dichos autoanticuerpos se encuentra en el dominio no colagenoso-1 de la cadena alfa-3 del colageno tipo IV (α3(IV)NC1). Posee una distribucion por edad bimodal, con un pico de incidencia en la tercera y septima decada, y su desarrollo se ha relacionado con diferentes factores geneticos y ambientales. La mayoria de pacientes presentan una glomerulonefritis rapidamente progresiva y el 50 % de casos una hemorragia alveolar concomitante. El diagnostico requiere demostrar la presencia de autoanticuerpos anti-MBG en la circulacion sanguinea, o depositos lineales de inmunoglobulinas mediante inmunofluorescencia directa sobre una biopsia renal. El tratamiento consiste en la combinacion de plasmaferesis, corticoterapia e inmunosupresores. Presentamos el caso de una paciente de 17 anos con hemorragia alveolar y presencia de autoanticuerpos anti-membrana basal glomerular circulantes, pero sin afectacion renal. Incluir esta patologia dentro del diagnostico diferencial de las hemorragias alveolares permite un diagnostico y tratamiento previo a la aparicion de lesiones renales irreversibles y, por tanto, un mejor pronostico. EnglishAnti–glomerular basement membrane disease, also called Goodpasture disease, is a rare autoinmune disease. It is a subtype of small vessel systemic vasculitis characterized by the presence of autoantibodies, which are directed mainly against the glomerular or alveolar basement membranes. The specific epitope of these autoantibodies is the non-collagenous domain-1 of the alpha-3 chain of type IV collagen (α3(IV)NC1). It has a bimodal age distribution, with one peak in the third decade and another in the seventh decade. In addition, its development has been related to different genetic and environmental factors. The majority of patients usually present rapidly progressive glomerulonephritis and about 50% of cases have concomitant alveolar hemorrhage. The diagnosis requires demonstration of circulating anti-glomerular basement membrane autoantibodies, or a linear deposition of immunoglobulins through direct inmunofluorescence on renal biopsy. The treatment consists of the combination of plasmapheresis, immunosuppressive therapy and corticosteroids. We analyzed the case of a 17-year-old patient with alveolar hemorrhage and circulating anti-glomerular basement membrane autoantibodies, but without renal involvement. Including this pathology in the differential diagnosis of alveolar hemorrhages, allows early diagnosis and treatment, so we avoid the appearance of irreversible renal lesions, and improve the renal prognosis.
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