*1 ,',23È7,&$ 3UHVHQoD GH $F ,J* DQWL 3/$ 5sonerj.org.br/wp-content/uploads/2017/05/... ·...
Transcript of *1 ,',23È7,&$ 3UHVHQoD GH $F ,J* DQWL 3/$ 5sonerj.org.br/wp-content/uploads/2017/05/... ·...
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Glomerulonefrite Membranosa
Alvimar Gonçalves Delgado
Serviço e Disciplina de Nefrologia - HUCFFFaculdade de Medicina
UNIVERSIDADE FEDERAL DO RIO DE JANEIRO
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Glomerulonefrite membranosa
1. Uma das principais causas de síndrome nefrótica em adultos
2. 80% dos casos são do tipo idiopático (?) e cerca de 20% são secundários a várias doenças, dentre elas:a. Lupus e Sarcoidoseb. Hepatite B e Cc. Esquistossomose (no Brasil)d. Neoplasias, Tiroiditee. Drogas (AINES, ouro coloidal, captopril,
penicilamina)
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GN IDIOPÁTICA: Presença de Ac IgG4 (70-80%) anti-PLA2R
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Kriz, W and Elger, M
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Anti-PLA2R50-80%
Glomerulonefrite membranosa primária
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Anti-THSD7A3-9%
Anti-PLA2R50-80%
Glomerulonefrite membranosa primária
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Depósitos granulares de IgGem alças capilares.
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J Am Soc Nephrol 2016
MO IgG C3
C1q PLRA2 ME
De Vriese AS, Glassock RJ, Nath KA, Sethi S and Fervenza FC.
GNM Anti-PLA2R
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IgG C3 PLRA2
IgG C3 PLRA2
A, B e C: GNM Anti-PLA2R positivoD, E e F: GNM Anti-PLA2R negativo
J Am Soc Nephrol 2016
De Vriese AS, Glassock RJ, Nath KA, Sethi S and Fervenza FC.
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J Am Soc Nephrol 2016De Vriese AS, Glassock RJ, Nath KA, Sethi S and Fervenza FC.
GNM Secundária
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Apresentação clínica:
1. Início insidioso2. Proteinúria maciça em mais de 80%dos casos 2. Maioria apresenta síndrome nefrótica
Proteinúria > 3,5 g/24 hHipoalbuminemiaHipercolesterolemiaEdema generalizado
3. Alguns têm proteinúria assintomática 4. Muitos pacientes têm hematúria microscópica5. Evolução: regra dos 3 terços:
1/3 evolui para IRCT em poucos anos1/3 tem proteinúria persistente sem IRCT1/3 regride (às vezes espontaneamente)
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Catran DC, JASN 2005
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Catran, DC and Brenchley, PE. Kidney Int. 2016
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História natural da GNMi
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Clorambucil e ciclofosfamida sãoigualmente eficazes
RC = 47%RP = 19%
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Rituximab in Membranous Nephropathy
CP1125840-1
Proteinuria (g/24 hr)
Proteinuria (g/24 hr)
Serum creatinine (mg/dL)
Serum creatinine (mg/dL)
* P<0.01 vs months -6 and 0Ruggenenti et al: JASN 14:851, 2003
* P<0.01 vs months -6 and 0Ruggenenti et al: JASN 14:851, 2003
-6 0 3 6 9 12
MonthsMonths
**
**** **
****
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CP1313718-3
Relative Changes of Plasma Albumin Concentration and Urinary Proteins Excretion Induced by Short-Term ACTH Treatment in
14 Nephrotic Patients with Idiopathic Membranous Nephropathy%
of
init
ial l
evel
% o
f in
itia
l lev
el
DaysDaysBerg et al: KI 56:1534, 1999Berg et al: KI 56:1534, 1999
ACTH injectionsACTH injections
AlbuminU-1MAlbuminuriaIgG
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Catran, DC and Brenchley, PE. Kidney Int. 2016