Term
| What are the four components to nephritic syndrome? |
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Definition
1) hematuria
2) azotemia
3) oliguria-Anuria
4) hypertension |
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Term
| Define: nephritic syndrome |
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Definition
- constellation of symptoms seen with glomerular inflammation including, hematuria, hypertension, decreased ability to produce urine, and a variable degree of kidney failure
*proteinuria is NOT a main feature* |
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Term
| What is the result of a urine sediment for a patient with nephritic syndrome? |
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Definition
| 1) "active" - inflammatory cells and blood, active glomerular inflammation |
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Term
| What ist he underlying mechanism in all nephritic conditions? |
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Definition
1) injury to glomerular endothelium with complement activation and influx of inflammatory cells
2) most cases endothelial injury is related to immune phenomena |
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Term
| What are the major immune phenomena observed that result in nephritic syndrome? |
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Definition
1) circulating immune complexes formed to systemic infection/active autoimmune disease; circulating antigen that is deposited and reacted to
2) circulating antibodies against glomerular basement membrane
3) circulating antibodies against neutrophils |
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Term
| What are common primary glomerular disorders presenting as nephritis? |
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Definition
1) acute post-streptococcal glomerulonephritis (post-infectious)
2) membranoproliferative nephritis (MPGN)
3) crescentic glomerulonephritis (RPGN): anti-GBM disease, RPGN due to immune complex deposition, pauci-immune glomerulonephritis (ANCA associated) |
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Term
| Describe acute post-streptococcal glomerulonephritis |
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Definition
1) immune complex depositions in the glomerulus (A-beta hemolytic streptococcus)
2) high ASO (antistreptolysis O) titers
3) inflammatory cells
4) hematuria
5) low USA incidence
6) can follow other bacterial, viral and parasitic infections (HepB) |
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Term
| Describe the mechanism of the glomerular disease in Acute post-streptococcal glomerulonephritis |
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Definition
1) strep antigen circulates and is deposited
2) IgG reacts w/ antigen
3) complement activation
4) inflammatory cell influx
5) low complement levels; hematuria |
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Term
| What are major types of post-infectious glomerulonephritis? |
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Definition
1) A-beta hemolytic streptococcus
2) Hep B
3) syphilis
4) infectious endocarditis
5) osteomyelitis
6) chronic pneumonia |
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Term
| What are epidemiologic characteristics of membranoproliferative glomerulonephritis? |
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Definition
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Term
| What are morphologic characteristics of membranoproliferative glomerulonephritis? |
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Definition
1) basement membrane abnormalities
2) influx of inflammatory cells |
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Term
| What are characteristics of the pathogenesis of membranoproliferative glomerulonephritis |
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Definition
1) circulating antigen-antibody complexes are trapped
2) caomplement activation
3) antigenemia is prolonged (unlike post-infectious GN)
4) complexes slowly built and removed |
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Term
| What are the most common causes of the antigens seen in membranoproliferative glomerulonephritis |
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Definition
1) life-long chronic infections: Hep C, Hep B, syphilis, HIV, malaria, other parasitic d/o
2) autoimmune chronic conditions: SLE, CVDs
2) malignancy: plasma cell neoplasms, lymphoma/leukemia |
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Term
| What is the difference between Primary MPGN Type I and Type II? |
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Definition
1) Type I : diganosis of exclusion
2) Type II : does not reveal the subendothelial electrone dense deposits seen in MPGN-I, chronic immune complex deposition d/o w/ abnormal activation of complement |
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Term
| Describe the prognosis of membranoproliferative glomerulonephritis |
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Definition
1) slow and progressive
2) 50% develop kidney failure w/in 10 years
3) recurs in transplanted kidneys
4) tx of underlying condition slows progression |
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Term
| Describe crescentic glomerulonephritis |
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Definition
1) rapidly progressive glomerulonephritis (RPGN) - clinical diagnosis
2) glomerular crescents
3) anti-glomerular basement membrane disease; crescentic glomerulonephritis of immune complex deposition; crescentic glomerulonephritis of pauci-immune type |
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Term
| Describe anti-gloerular basememnt membrane disease (RPGN subtype) |
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Definition
1) cellular glomerular crescents
2) no electron deposits
3) autoimmune d/o HLA-DR B1 w/ IgG-Col-IV moieties
4) Goodpasture's disease when pulmonary hemorrhage in addition to RPGN
5) rapid desctruction of glomeruli
6) Tx: plasmapheresis; cytotoxic agents |
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Term
| Describe crescentic glomerulonephritis of immune complex deposition (RPGN subtype) |
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Definition
1) granular immune complexes in gapillary walls
2) large electron dense deposits
3) any type of immune complex GN: post-infectious GN, SLE, IgA nephropathy
3) vigorous active disease; poor prognosis
4) Tx: underlying disease |
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Term
| Describe crescentic glomerulonephritis of Pauci-Immune Type |
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Definition
1) no immune complexes or basement membrane antibodies
2) no deposits
3) most common form of GN
4) ANCAs (c-ANCA; p-ANCA)
5) systemic disease
6) very aggressive
7) Tx: immunosuppressive Tx w/ cytotoxic medication |
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Term
| What does c-ANCA refer to? p-ANCA? |
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Definition
1) anti-proteinase 3
2) anti-myeloperoxidase |
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Term
| What are the major systemic disorders presenting with nephritis? |
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Definition
1) IgA nephropathy
2) Henoch-Schonlein Purpura
3) SLE |
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Term
| Describe IgA Nephropathy/HSP? What is the general difference between the two? |
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Definition
1) characterized by IgA deposition
2) IgA-N: deposition in only the glomeruli; HSP: deposition in the systemic blood vessels |
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Term
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Definition
- deposition initially mesangial; mesangial cellularity
- thickening of capillary walls w/ membrane splitting
- cellular crescents
- segmental or global scarring
- IgA, C3, IgG deposits
- electron dense deposits
- unknown cause
- most common world-wide cause of glomerular disease: male, Asia
- slowly progressing
- Tx: NO TX |
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Term
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Definition
- systemic deposition of IgA in small vessels
- young children
- single episode of purpura is common
- persistent kidney disease is infrequent |
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Term
| What are the major mechanisms that play a role in the pathogenesis of IgA nephropathy and HSP |
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Definition
1) abnormal mucosal immune response secodary to antigenic stimulation
2) genetic predisposition
3) abnormal IgA molecules w/ altered structure
4) defective clearance of IgA molecules from serum by the liver |
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Term
| Describe the deposits seen in Lupus nephritis |
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Definition
| - large granular depositions of all classes of Ig's and complement: IgG, IgM, IgA, C3, C4, C1q = "full house" = characteristic of lupus nephritis |
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Term
| Describe class I lupus nephritis |
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Definition
1) no kidney involvement or minimal mesangial lupus nephritis
- unremarkable glomeruli
- normal kidney fxn |
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Term
| describe class II lupus nephritis |
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Definition
- mesangial proliferative lupus nephritis
- hematuria and proteinuria
- mildly reduced kidney function
- glomerular mesangioproliferative ghanges
- full house deposits |
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Term
| describe class III (focal) and IV (diffuse) lupus nephritis |
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Definition
- proliferative nephritis
- presence of active lesions
- full house immune complex deposition
- dense deposits |
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Term
| What are examples of active lesions seen in classIII and IV lupus nephritis? |
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Definition
- endocapillary proliferation
- necrosis
- neutrophilic infiltrates
- wire loops
- membrane breaks
- crescents
- hyaline thrombi
- fibrin deposition |
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Term
| Describe class V lupus nephritis |
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Definition
- membranous lupus nephritis
- resembles MN
- full house immune depositions
- subepithelial depositions
- active SLE
- less aggressive |
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Term
| Describe class VI lupus nephritis |
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Definition
- advanced sclerosing lupus
- kidney failure
- diffuse and global glomerulosclerosis |
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