Term
| What does the presence of crescents on renal biopsy indicate about the prognosis of disease? |
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Definition
Glomerular crescents= RPGD
Rapid diagnosis with renal biopsy and institution of therapy are critical to prevent ESRD |
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Term
| What are the 3 types of RPGN |
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Definition
Remember those crescents!
1) Anti-GBM disease/Goodpasture (linear IF)
- type II or IV
2) Immune complex (membranoproliferative GN, lupus, cryoglobun GN, IgA nephropathy)
- Type III
3) Pauci immune ANCA associated vasculitis (Wegeners, microscopic polyangiitis, pauci immune crescentic GN) |
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Term
| What factors does cellular proliferation in glomerular disease relate to? |
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Definition
1) Immune for complexes or antibodies depolsiting Subendothelial/GBM
2) Complement activation
- Hypocomplementemia (too much activation) means proliferative
**IgA does NOT activate complement, but is still proliferative** |
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Term
| What glomerular diseases are associated with Hypocomplimentemia? |
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Definition
COMPS
1) Cryoglobulinemia (hep C)
2) AtherOemboli
3) Membranoproliferative GN
4) Post infectious GN
5) Systemic LE |
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Term
True or False:
Membranoproliferative GN is proliferative but does not involve complement activation |
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Definition
False!
Remember, COMPS are activate complement.
The ONLY proliferative GN that does not is IgA GN |
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Term
A previously healthy child presents while recovering from a recent sore throat with gross hematuria and microscopic hematuria. You also see RBC casts.
On PE he exhibits peripheral and facial edema, and 2g/24h proteinuria.
How can you confirm your diagnosis and what is the pathogenesis of this condition? |
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Definition
Young healthy child with what seems to be recent streptococcal infection with nephritic urine suggests
Acute Post-Infectious GN (confirm with SO titer)
1) Group A, hemolytic Streptococci creates an initial immune response.
2) Antibodies are depositied and cross-react with intrinsic glomerular epitopes.
3) Inflammation (IL-6 and ICAM mediated) occurs with complement activation (hypocomplimentemia) |
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Term
You collect a pathological specific from a young child and see the following on microscopy:
LM: Glomerular tufts that are hypercellular with leukocytes in the glomerular capillaries
IF: IgG and C3 with "lumpy, bumpy" pattern (coarsely granular)
EM: Subepithelial "hump" deposits , "flame-like deposits."
How do you treat? |
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Definition
These are classic findings of post-streptococcal GN (sub-epithelial "humps" on EM and "lumpy-bumpy" IgG/C3 pattern on IF.
Treatment is supportive
1) Sodium restriction
2) Control BP
Biopsy is not required for diagnosis and most patients get better. |
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Term
A patient that is HIV (+) presents complaining of flank pain and says that "he has seen a lot of blood in his urine after exercising."
He has +2 proteinuria. What do you see on pathology? |
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Definition
This sounds like IgA Nephropathy, which is common in HIV patients and IBD patients (or those with severe liver disease).
**remember, it is proliferative, but without complement activation**
LM: looks normal or with non-specific findings (NO inflammation)
IF: IgA is everywhere, with C3
EM: Mesangial electron dense deposits
No good treatment except maybe fish oil for renal failure. |
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Term
A young child presents with purpuric rash over their buttocks and legs, as well as arthralgia and GI bleeding.
You perform a skin biopsy and discover necrotizing "leukocytoclastic" vasculitis.
What proliferative GN might this condition relate to? |
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Definition
This is Henoch-Schonlein Purpura (HSP), which is IgA mediated. so IgA nephropathy is realted.
50% of patients have variable renal involvement. |
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Term
A patient presents with with BP of 150/90
They have +4 proteinuria and hematuria and you discover the following findings on pathology.
LM: global hypercellularity with accentuation of glomerular lobules.
On silver stain, you notice a double contour in the capillary walls.
EM: You see subendothelial deposits.
What could be going on? |
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Definition
Nephrotic protein levels in HTN setting with hematuria points to MPGN.
Path is consistent with glomerular lobules (Lobular GN) and Tram-Tracking suggests
Type 1- Mesangiocapillary GN
Subendothelial deposits are also consistent with type 1 MPGN |
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Term
| What proliferative GN has an association with hep C? |
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Definition
Cryoglobulinemia, which is a form of Type 1 MPGN.
It involves Ig that precipitates on cooling and solubilizes on heating. |
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Term
| What pathological changes should you look for to diagnose Type II MPGN? How can you distinguish it from Type I? |
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Definition
1) Type II MPGN involves thickened lamina densa with dense deposits of C3 on either side of it.
**This is mediated by C3 nephritic factor autoantibody, which stabilizes the alternative pathway of C3 convertase (C3bBb) and prevents normal degradation by Factor H/I and**
2) Type 1 MPGN involves mesangocapillary GN (such as Cryoglobulinemia with Hep C association).
You should look for "tram tracking" (double contour of capillary wall) on Silver stain with subendotheial deposits on EM. |
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Term
| What type of MPGN involves both subendothelial and subepithelial immune complex deposits? |
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Definition
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Term
| What are the two principal patterns of immune complex deposition see on pathology of SLE? |
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Definition
Remember FULL HOUSE IF pattern and tubuloreticular inclusions (endothelial cells) on EM
1) Mesangial-subendothelial: pre-formed immune complexes that may eventually extend to the subendothelial space (DNA/anti-DNA)
2) Membranous: smaller complexes forming sup-epithelial deposits |
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Term
| How do you treat the most aggressive form of SLE? |
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Definition
| Class IV is treated with steroid and cyclophosphamide (prevent SE with mezna) |
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Term
What condition is each of the following pathological features associated with?
1) IF: mesangial IgA deposits
2) IF: "FUll house" with different histological classes
3) Double contour of BM, tram tracks and subendothelial deposits
4) IF: linear deposits
5) IF: Minimal immune responses
6) IF: Lumpy bumpy with humps and flame-like bumps on EM |
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Definition
1) IgA nephropathy
2) SLE
3) MPGN
4) Goodpasture
5) Type III-Pauci immune
6) Acute post-infectious GN |
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Term
A young man presents with hemoptysis, cough, shortness of breath and pneumonitis.
You order a renal biopsy and see crescentic glomerulonephritis and anti-GBM antibodies.
What do you see on IF microscopy and how do you treat? |
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Definition
This is classic presentation of Goodpasture's Syndrome, which is type-1 RPGN.
It tends to occur in young males and presents with hemoptysis, cough, shortness of breath and pneumonitis.
1) IF microscopy would reveals LINEAR deposits of IgG within the GBM (targeted against collagen IV)
2) Plasmapheresis is recommended to remove anti-GBM antibodes and steroids and cyclohosphamide should also be added. If you don't catch it early (<6 mg/dl Cr), there is poor prognosis. |
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Term
| What are 2 examples of Type II RPGN? |
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Definition
1) SLE GN ("Full house" histology in young women)
2) Cryoglobulinemic GN (hep C association with capillary tram tracking on silver stain) |
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Term
A 55 year old male presents with a 2 week history of intermittent fever, arthralgia, weight loss, dyspnea and middle ear effusion.
A shows moderate proteinuria and active urine sediments with RBC casts.
You order a renal biopsy and discover necrotizing crescentic lesion (LM, but no immunoprotein deposits (IF)
What diseases might this be and how to you tell/treat? |
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Definition
This is classic Pauci-immune proliferative and necrotizing glomerulonephritis, with vasculitis and a lack of immune deposits on IF and EM.
Could be 1) Wegener's granulomatosis (C-ANCA/PR3) or 2) Microscopic Polyangiiitis (P-ANCA/MPO)
Treat acutely with hi-dose steroids and cyclophosphamide
**Chronic treatment for Wegener's may include Bactrim** |
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Term
| What are the classic examples of Type 1, Type 2 and Type 3 RPGN? |
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Definition
1) Goodpasture's (anti-GBM Ab with linear IgG in GBM).
2) SLE and Cryoglobulinemic GN
3) Pauci-immune proliferative and necrotizing GN (Wegener's and Microscopic Polyangitis) |
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Term
A patient with chronic HTN, hematuria and proteinuria (non-nephrotic) receives a renal US and you find small (9 cm) kidney's.
What kind of urine sediment would you expect to see and how would you slow the disease progression? |
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Definition
1) This is Chronic GN and you would see RBC and Broad casts in the urine.
2) CGN could arise because of any glomerular syndrome and can be slowed down by treating HTN (Ace-i), lipidemia (Statin), smoking cessation and treatment of sequele. |
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