¨A síndrome de Goodpasture, também conhecida como síndrome de anticorpo antimebrana basal, é uma patologia mediada pelo sistema imunológico, na qual auto-anticorpos contra a cadeia a 3 (IV) do colágeno tipo IV se ligam à membrana basal, alveolar e glomerular, causando glomerulonefrite progressiva e hemorragia pulmonar. Na prática clínica a síndrome consiste em uma hemorragia alveolar idiopática recorrente e glomerulonefrite rapidamente progressiva. Ela é mediada por anticorpos contra a membrana basal glomerular. Ocorre principalmente em homens na faixa etária dos 30 e 40 anos. O mecanismo de injúria renal e pulmonar é complexo. Grande parte dos pacientes apresenta combinação de glomerulonefrite rapidamente progressiva e hemorragia alveolar, podendo em 30% a 40% dos casos exibir acometimento renal isolado caracterizado por hematúria, proteinúria leve a moderada, ou mesmo insuficiência renal aguda. O acometimento pulmonar é mais comum em homens jovens, manifesta-se clinicamente por dispnéia e tosse, com ou sem hemoptise. Segundo alguns autores a hemoptise costuma ser a primeira manifestação, mas a hemorragia pulmonar pode estar oculta. O diagnóstico baseia-se em depósitos lineares de IgG característicos nos glomérulos ou alvéolos por imunofluorescência, e na presença de anticorpo contra a membrana basal glomerular no soro. A presença de infiltrado alveolar ao raio-X simples de tórax pode acompanhar o caso, porém é pouco específico. Anticorpos anticitoplasma de neutrófilo (Anca) estão presentes em 30% dos pacientes com doença antimembrana basal. A doença não tratada geralmente apresenta um prognóstico ruim. A introdução do tratamento combinado de plasmaferese (remoção de anticorpos patogênicos), agentes alquilantes tais como a ciclofosfamida (prevenção de síntese de novos anticorpos) e corticoterapia (ação antinflamatória) revolucionaram a evolução da doença. O prognóstico na apresentação do quadro clinico é pior se há oligúria, fibrose renal avançada ou mais que 50% de crescentes na biópsia renal. Nível elevado de creatinina (creatinina pré-tratamento > 6,6 mg/dl) ou necessidade de diálise também se associam com pior evolução. A sobrevida em um ano é cerca de 75% a 90%. O quadro pulmonar é, freqüentemente, responsivo a plasmaferese. A presença de hemorragia alveolar é indicação formal de tratamento intensivo, a despeito da gravidade do acometimento renal.¨
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