AULA 3 - Glomerulopatias Primárias

GLOMERULOPATIAS PRIMÁRIAS NEFROPATIAS 

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GLOMERULOPATIAS: PRIMÁRIAS SECUNDÁRIAS

PRIMÁRIAS - AFETA O GLOMÉRULO PRIMARIAMENTE, PODE SER INFLAMATÓRIA OU DEGENERATIVA SECUNDÁRIAS - TEM UMA DOENÇA SISTÊMICA E

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CONCEITO: DOENÇA DOS GLOMERULOS GLOMERULOPATIAS: PRIMÁRIAS SECUNDÁRIAS

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• • • • • • •

Glomerulonefrite Glomerulonefrite difusa aguda (GNDA) Glomerulonefrite membranosa (GNM) Glomerulonefrite Glomerulonefrite membranoproliferativa(GNM membranoproliferativa(GNMP) P) Glomerulonefrite Glomerulonefrite proliferativa focal (GPF) Glomerulonefrite Glomerulonefrite IgA (GNIgA) Glomerulopatia com lesões mínimas (GLM) Glomeruloesclerose Glomeruloesclerose focal segmentar (GFS) Glomerulonefrite Glomerulonefrite rapidamente progressiva (GNRP)

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Glomerulonefrite pós-estreptocócica Glomerulonefrite (GNDA= Glomerulonefrit Glomerulonefritee difusa aguda ) DOENÇA DIFUSA GLOBAL, CAUSADA PELA DEPOSIÇÃO DE COMPLEXOS IMUNES NOS GLOMÉRULOS, ESTIMULADA POR UMA INFECÇÃO PRECEDENTE (ESTREPTOCÓCICA)

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Glomerulonefrite pós-estreptocócica Glomerulonefrite (GNDA) Idade: pré-escolar  Sexo masculino Infecção de pele ou orofaringe Streptococus – grupo A É uma doença imunológica: há período latente entre infecção e aparecimento da doença QC: assintomático até IRA 

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PATOGÊNESE INFECÇÃO POR STREPTOCOCOS BETA-HEMOLÍTICO DO GRUPO A (CEPAS “NEFRITOGÊNIC “NEFRITOGÊNICAS”=12, AS”=12, 4, 1, 25 e 49) 1-2 Semanas após DEPÓSITO DOS

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CELULARIDADE (M.O.) PROLIFERAÇÃO DAS CÉLULAS ENDOTELIAIS (M.E.) COMPLEXOS IMUNES NO LADO EPITELIAL DA MBG (M.E.) DEPOSIÇÃO GRANULAR DE IgG e C3 NA MBG E MESÂNGIO( IF)

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Glomérulos com GNDA



Glomérulos normais



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GNDA

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IgG e C3

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GNDA

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Glomerulonefrite pós-estreptocócica Glomerulonefrite

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Glomerulonefrite Membranoproliferati Glomerulonefrite Membranoproliferativa/ va/ GN Mesangiocapilar ME e IMF: permite IMF: permite distinguir 3 tipos de GNMP: • Tipo I: 2/3 dos casos ME: depósitos eletrodensos subendoteliais podendo aparecer depósitos no mesângio. IMF:: depósitos granulares de C3 IMF depósitos de IgG são menores e diminuem com a 

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Glomerulonefrite Membranoproliferati Glomerulonefrite Membranoproliferativa/ va/ GN Mesangiocapilar • • •

Tipo II: ME depósitos acentuadamente densos intramembranosos intramembranosos IMF: depósitos de C3 de aspecto granular e linear, não relacionados aos depósitos densos e sem complexos imunes ATIVAÇÃO DA VIA ALTERNADA DO COMPLEMENTO + EM CRIANÇAS E ADOLESCENTES

Tipo III: mais rara

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Glomerulonefrite Membranoproliferati Glomerulonefrite Membranoproliferativa/ va/ GN Mesangiocapilar •

Aspectos clínicos: hematúria

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Glomerulonefrite Proliferativa Focal e Segmentar

PROLIFERAÇÃO CELULAR COMPROMETENDO APENAS

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IMUNO-HISTOQUÍMICA = DETERMINA A DEPOSIÇÃO DOS COMPLEXOS IMUNES DOS DIFERENTES TIPOS

PRÍMÁRIAS: DOENÇA MESANGIAL POR IgA

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GPF



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GNRP

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Glomerulonefrite Crescêntica/GNRP Glomerulonefrite

Quadro clínico:

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Início abrupto Hematúria micro ou macroscópica Edema discreto Hipertensão leve

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Glomerulonefrite Crescêntica/GNRP Glomerulonefrite Laboratório:

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EAS c/ hematúria, cilindros hemáticos, proteinúria Proteinúria 24 horas = 1 a 2 g; 30% > 3g Creatinina sérica elevada Complemento sérico normal ou diminuído

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Glomerulonefrite Crescêntica/GNRP Glomerulonefrite Evolução

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Após 3 anos = 80 a 90% c/ IRC Recidiva em Tx = pequena

Prognóstico:

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Depende quantidade glomérulos acometidos por  crescentes (epiteliais/fibrosados)

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Glomerulonefrite Crescêntica/GNRP Glomerulonefrite Tratamento:

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Sintomático Específico: Plasmaferese Pulsoterapia com corticóide

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Síndrome de Goodpasture Good pasture

GLOMERULONEFRITE RARA

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Síndrome de Goodpasture Goodpasture

•

IMF: depósitos linear de IgG e C3 ao longo das paredes

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