急进性肾小球肾炎英文课件
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Department of Nephrology, Peking University First Hospital
–80年代 20例; 90年代 100例
2.Type of immunopathogenesis
Three Types Classification
Type I: Antiglomerular basement membrane Type II: Immune complex Type III: Pauci immune, 50-80% vasculitis
Renal failure over days/weeks（肾功能数天或数 周内恶化）
Early oliguria or anuria(早期出现少尿或无尿)
一、Etiology and Pathogenesis
1.morbidity
USA：1/1,000,000 China: unknown,
3.Classification
Primary glomerulopathy
– Primary Crescentic glomerulonephritis – Crescentic glomerulonephritis, on the basis of the
other primary glomerular dise来自百度文库ses (Mesangial capillary glomerulonephritis) Infection-associated (post bacterial endocarditis) Multi-system disease（LN） Drug-related (PTU)
Usually symptoms are confined to the preceding few weeks or months, but very rapid progression or much slower progression may occur.
Background of Goodpasture’s disease — Milestone
Type III: no or pauci immune deposits
crescentic glomerulonephritis Cellular crescent and pink fibrin deposition
Masson x 260
crescentic glomerulonephritis large scale fibrocellular crescent
新月体性肾炎(毛细血管外增生性肾小球肾炎)
(crescentic glomerulonephritis)
Diagnostic criteria:
– glomerulus crescent formation >50%
– Affected area of glomerular capsule >50%
急进性肾小球肾炎
rapidly progressive glomerulonephritis
急进性肾小球肾炎
(rapidly progressive glomerulonephritis)
Nephritic syndrome(急性肾炎综合征) Proteinuria: usually<3g/day Hematuria: red cell casts Blood pressure often normal，sometimes increase
Five Types Classification
Type I: Antiglomerular basement membrane Type II: Immune complex Type III: Pauci immune+ANCA(+) Type IV: I+ANCA(+) Type V: III+ANCA(-)
Type of Crescent
cellulous cellularfibrous
fibrous
Type I: Immunofluorescence microscopy, IgG and C3, linear deposition, along capillary wall
Type II: IgG and C3, granular deposition, mesangium and capillary wall; proliferation of endothelial and mesangial cells;
二、Pathology
Crescentic GN: the proliferative cellular response outside the glomerular tuft but within Bowman’s space that is known as a crescent because of its shape on histologic cross-section.
PASx260
三、Clinical manifestation
1.Type I: The clinical manifestation of Goodpasture’s disease (抗GBM病) arise from lung hemorrhage and/or rapidly progressive glomerulonephritis. Between 50% and 75% of patients present with acute symptoms of lung hemorrhage and are found to be in a state of advanced renal failure.
1919年由Goodpasture首先报道 – 1例18岁男性病人，发烧、咯血、急 性肾衰竭
1967年Lerner等发现抗GBM抗体
1984年Wieslander等发现(IV)NC1为主 要靶抗原
1995年Kalluri等发现3(IV)NC1为主要 靶抗原
–80年代 20例; 90年代 100例
2.Type of immunopathogenesis
Three Types Classification
Type I: Antiglomerular basement membrane Type II: Immune complex Type III: Pauci immune, 50-80% vasculitis
Renal failure over days/weeks（肾功能数天或数 周内恶化）
Early oliguria or anuria(早期出现少尿或无尿)
一、Etiology and Pathogenesis
1.morbidity
USA：1/1,000,000 China: unknown,
3.Classification
Primary glomerulopathy
– Primary Crescentic glomerulonephritis – Crescentic glomerulonephritis, on the basis of the
other primary glomerular dise来自百度文库ses (Mesangial capillary glomerulonephritis) Infection-associated (post bacterial endocarditis) Multi-system disease（LN） Drug-related (PTU)
Usually symptoms are confined to the preceding few weeks or months, but very rapid progression or much slower progression may occur.
Background of Goodpasture’s disease — Milestone
Type III: no or pauci immune deposits
crescentic glomerulonephritis Cellular crescent and pink fibrin deposition
Masson x 260
crescentic glomerulonephritis large scale fibrocellular crescent
新月体性肾炎(毛细血管外增生性肾小球肾炎)
(crescentic glomerulonephritis)
Diagnostic criteria:
– glomerulus crescent formation >50%
– Affected area of glomerular capsule >50%
急进性肾小球肾炎
rapidly progressive glomerulonephritis
急进性肾小球肾炎
(rapidly progressive glomerulonephritis)
Nephritic syndrome(急性肾炎综合征) Proteinuria: usually<3g/day Hematuria: red cell casts Blood pressure often normal，sometimes increase
Five Types Classification
Type I: Antiglomerular basement membrane Type II: Immune complex Type III: Pauci immune+ANCA(+) Type IV: I+ANCA(+) Type V: III+ANCA(-)
Type of Crescent
cellulous cellularfibrous
fibrous
Type I: Immunofluorescence microscopy, IgG and C3, linear deposition, along capillary wall
Type II: IgG and C3, granular deposition, mesangium and capillary wall; proliferation of endothelial and mesangial cells;
二、Pathology
Crescentic GN: the proliferative cellular response outside the glomerular tuft but within Bowman’s space that is known as a crescent because of its shape on histologic cross-section.
PASx260
三、Clinical manifestation
1.Type I: The clinical manifestation of Goodpasture’s disease (抗GBM病) arise from lung hemorrhage and/or rapidly progressive glomerulonephritis. Between 50% and 75% of patients present with acute symptoms of lung hemorrhage and are found to be in a state of advanced renal failure.
1919年由Goodpasture首先报道 – 1例18岁男性病人，发烧、咯血、急 性肾衰竭
1967年Lerner等发现抗GBM抗体
1984年Wieslander等发现(IV)NC1为主 要靶抗原
1995年Kalluri等发现3(IV)NC1为主要 靶抗原