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85 Cards in this Set

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This is the most common form of Acute Glomerulonephritis
IgA Nephropathy = Berger Disease
What is the most common age group for IgA Nephropathy?
Young adults = 15-30 yoa
Describe the pathology of IgA Nephropathy
Focal and segmental glomerulopathy caused by deposition of IgA in the Mesangium
What are the clinical manifestations of IgA Nephropathy?
Protean manifestations:
-40% asymtomatic microscopic hematuria
-40% bouts of macro hematuria
-10% nephrotic syndrome
-10% renal failure

**many present with recurrent hematuria (red/cola-colored urine) 1-2 days after a respiratory infection
What is the histology of IgA Nephropathy?
Focal proliferative glomerulonephritis with diffuse mesangial widening
A 15-year old Asian-American boy presents with red-colored urine. After a history, you are informed that he is suffering from a strange flu-like syndrome that began 2 days earlier consisting of vomiting, myalgias, arthralgias, and fever
IgA Nephropathy
-postinfectious disease (usually respiratory infection)
-Nephritic type syndrome
-most commonly seen in young adult males
Synonym for Crescentic Glomerulonephritis
Rapidly Progressive Glomerulonephritis
List 4 causes of Crescentic Glomerulonephritis
1. Anti-GBM nephritis = Goodpasture's Syndrome
2. ANCA granulonephritis = Wegener Granulomatosis, Microscopic Polyarteritis nodosa, Churg-Strauss Syndrome
3. Immune-complex Glomerulonephritis = Henoch-Schonlein purpura, Cryoglobulinemia
4. Non-immune
What is important with Crescentic Glomerulonephritis?
You must diagnose and treat patients ASAP, or they'll lose both kidneys within a couple weeks
What is the cause of Crescentic Glomerulonephritis in 50% of the cases?
Post-streptococcal etiology
When auto-antibodies are more active when the blood is cold
Cryoglobulinemia
List the properties of Anti-Glomerular Basement Membrane Antibody Glomerulonephritis
1. Rapidly Progressive GN mediated by antibody
2. Antibody is to Collage type IV
3. Linear IF
4. Fibrinoid necrosis of GBM
5. Crescentic GN
6. Goodpasture Syndrome
ANCA granulonephritis that does not have granular deposits on Immunofluorescence
Wegener granulomatosis
What does ANCA Glomerulonephritis begin as?
Necrotizing GN --> becomes Crescentic
ANCA is found in serum and binds to what?
PMN myeloperoxidase and Proteinase 3
Besides IgA Nephropathy, what other disease is characterized by a deposition of IgA in the Glomeruli?
Henoch-Schonlein Purpura
This disease has no immune deposits in the glomeruli, responds to immunosuppression, and is associated with small vessel polyangiitis
ANCA Glomerulonephritis
- Wegener Granulomatosis
List the features of Nephrotic Syndrome
1. Proteinuria
2. Hypoalbuminemia
3. Generalized Edema
4. Hyperlipidemia = due to increase in lipoprotein synthesis
5. Lipiduria
List the diseases associated with Nephrotic Syndrome
1. Minimal change disease
2. Membranous Glomerulonephritis
3. Focal Segmental Glomerulosclerosis
4. Diabetic Nephropathy
What are 2 other terms for Minimal Change Glomerulonephrpathy?
1. Lipoid nephrosis

2. Nil disease
This is the most common cause of Nephrotic Syndrome in Children
Minimal Change Glomerulopathy
What is the pathology of Minimal Change Glomerulopathy?
Under EM there is fusion of epithelial (podocyte) foot processes

**Light Microscope and Immunofluorescence show normal glomeruli
What is the treatment for Minimal Change Glomerulopathy?
Steroids (prednisone)
A 4-year-old boy presents to the emergency with a 1-week history of generalized edema and fatigue. You history reveals that he suffered from a viral URI 1 week before the visit. Serum and urine studies show massive Proteinuria, hyperlipidemia, hypoalbuminemia. What pathology would you expect to see?
EM showing fusion on epithelial foot processes

= Minimal Change Glomerulopathy
Nephrotic syndrome associated with heroin use, morbid obesity, Sickle Cell anemia, and HIV infection
Focal Segmental Glomerulosclerosis
Nephrotic syndrome that does not respond to Steroid treatment and most patients progress to ESKD in 5-10 years
Focal Segmental Glomerulosclerosis

**if HIV related -> ESKD in 1 year
**if child is affected, may take 20 yrs to develop ESKD
What is the pathology seen in Focal Segmental Glomerulonephritis?
- Sclerosis within Capillary tufts of deep juxtaglomerular glomeruli with Focal distribution and Segmental Distribution
-Hyalinosis = deposition of hyaline masses

*Focal = involves some, but not all, glomeruli
*Segmental = involves part of the glomerulus
Most common cause of Nephrotic Syndrome in Adults
Focal Segmental Glomerulosclerosis
Nephrotic syndrome that has Immune-complex deposition that causes basement membrane thickening
Membranous Nephropathy
What is the cause of Primary Membranous Nephropathy?
Unknown
What is the cause of Secondary Membranous Nephropathy?
1. SLE
2. HBV, HCV, & syphillis
3. Drugs = Penicillamine
4. Malignancy = tumor antigens

**Immune-complexes deposit in kidney --> Subepithelial
What is the pathology seen under light microscope in Mebranous Nephropathy?
1. Diffuse membrane-like thickening of the capillary walls

2. Basement membrane "spike-and-dome" appearance is seen in Silver Methenamine stain
A 40-year-old woman with a history of SLE presents to your office with a CC of increased swelling in her legs. She had been referred by her primary care physician who suspected a secondary illness to her lupus. Recent lab studies show proteinuria, hypoalbuminemia, hyperlipidemia, and hypercholesterolemia. You suspect that a renal biopsy would demonstrate immune-complex deposition on electron microscopy as well as "spike and dome" appearance on silver methenamine stain
Membranous Nephropathy
What pathology is seen on EM in Membranous Nephropathy?
Immune-comple deposition in Subepithelial locations
What is the treatment for Membranous Nephropathy?
No treatment -> is a slowly progressive disorder
What pathologies does Diabetic Glomerulosclerosis cause?
1. Diffuse global thickening of the basement membrane
2. Nodular sclerosis (Kimmelstiel-Wilson nodules)
3. Arteriosclerosis --> HTN
4. Trapping of serum proteins
Kimmelstiel-Wilson nodules
Diabetic Glomerulosclerosis

= nodular accumulations of mesangial matrix material
What is the clinical manifestation of Diabetic Nephropathy?
Proteinuria = major manifestation

**Nephrotic syndrome
- severe proteinuria
- Hyperlipidemia
- Lipiduria
Leading cause of chronic renal failure in the US
Diabetic Glomerulosclerosis
List the 4 diabetic kidney diseases
1. Glomerulosclerosis

2. Arteriosclerosis = thickened hyaline walls with narrow lumens --> HTN

3. Pyelonephritis = high concentrations of sugar in the interstitium promotes bacterial growth

4. Papillary necrosis = when the pyramid sloughs off and goes down into the renal pelvis
What is Primary Amyloidosis?
Deposition of AL amyloid from Multiple Myeloma

**AL = amyloid light chain derived from Bence-jones proteins
What is Secondary Amyloidosis?
deposition of AA amyloid in chronic suppurative conditions, such as purulent osteomyelitis or bronchiectasis
Where are Amyloid deposits found in the kidney?
1. Glomeruli (mesangium, GBM)

2. Arterioles

3. Tubular basement membranes
What are the clinical manifestations of Amyloidosis?
1. Nonselective proteinuria (100% of cases)

2. Nephrotic syndrome (60% of cases

3. Renal failure develops over 2-5 yrs

4. Kidneys infiltrated with Amyloid become large!!!
List 5 causes of Nephrotic Syndrome
1. Minimal Change disease
2. Focal and Segmental Glomerulosclerosis
3. Membranous Nephropathy
4. Diabetes Mellitus
5. Amyloidosis
IgA Nephropathy

Focal proliferative glomerulonephritis with diffuse mesangial widening
-Focal & Segmental mesangial widening

-Hematuria
What Nephritic Syndrome is this?

How do you know?

What would be the usual presentation with this?
IgA Nephropathy
-IgA is deposited mainly within the Mesangium
-Focal Proliferative Glomerulonephritis with diffuse mesangial widening
What Nephritic Syndrome would this be?
IgA Nephropathy (Berger Disease)
-MESANGIAL deposits of IgA and C3
What Nephritic Syndrome is this?
IgA Nephropathy = proliferative mesangial disease
-there are 2 mesangial cells
-normally there is 1 mesangial cell per 3 capillary loops
What is this schematic showing?
Rapidly Progressive Glomerulonephritis = Crescentic Glomerulonephritis
-crescent-shaped proliferation of parietal epithelial cells encroaching on the glomerulus
What is this Nephritic Syndrome?
Goodpastures's Syndrome

Type II = Cytotoxic = Auto-Ab's directed toward GBM
What is this typical of?

What type of Hypersensitivity Reaction?
Goodpasture Syndrome
-Anti-GBM Ab's cause focal fibrinoid necrosis where rupture occurs and fibrin leaks out thru the BM
Explain this slide
Male dominant Rapidly Progressive Glomerulonephritis Nephritic Syndrome with a LINEAR IF
Goodpasture Syndrome
-fibrinoid necrosis of GBM
Goodpasture Syndrome
-hypercellularity
-crescents
-fibrin
What disease?
Goodpasture Syndrome
-glomerulus is hyalinized due to Anti-GBM Ab's = Hyaline leaks out from the capillaries
What disease?
-Goodpasture Syndrome

- Capillary loops are being compressed by the Crescent

-GFR will decrease to ZERO in a couple weeks = Rapid Progressive Glomerulonephritis
What disease?
What process is happening?
What does this cause?
Normal Kidney
-one mesangial cells per 3 capillary loops
What is this?
Minimal Change Nephropathy
-fused foot processes of the epithelial cells
Pathology seen on the right
Minimal Change Disease (Lipoid Nephrosis = Nil Disease)
-Fused foot processes
What Nephrotic Syndrome is this?
Focal Segmental Glomerulosclerosis
-Sclerosis within capillary tufts of deep JG glomeruli

HIV, AIDS, Obesity, Sickle Cell
What Nephrotic Syndrome is this?

What is it associated with?
Membranous Nephropathy
Nephrotic Syndrome associated with these 2 pathologies
Membranous Nephropathy
-diffuse capillary wall thickening and basemement membrane thickening
-there is NO hypercellularity
What Nephrotic Syndrome is this?
Membranous Nephropathy
-capillary wall and basement membrane thickening
-NO hypercellularity
What Nephrotic Syndrome is this?
Membranous Nephropathy
-immune-complex deposition in SUBEPITHELIAL locations
What Nephrotic Syndrome is this?
IgG and C3 deposits
This is Membranous Glomerulonephropathy. What is deposited in the Subepithelium?
What is deposited in the Sclerotic lesions in Focal Segmental Glomerulosclerosis?
IgM & C3

**vs. IgG/C3 for Membranous Nephropathy
Membranous Glomerulonephritis
-Spike-and-Dome appearance on Silver Methenamine stain
What Nephrotic Syndrome is this?
Diabetic Glomerulosclerosis

Nodular Sclerosis = Kimmelstiel-Wilson nodules
-nodular accumulation of mesangial matrix material
What Nephrotic Syndrome is this?

What is the pathology?
Diabetic Nephropathy
-Diffuse Glomerulosclerosis = diffusely distributed increase in mesangial matrix
-Hyalinization of Efferent and Afferent Arterioles
What Nephrotic Syndrome is this?
Hyalinized Arterioles due to Diabetic Nephropathy

Hypertension
What is seen here?
What is the consequence?
1. Necrotic Renal Papilla

2. Diabetic nephropathy

3. Drops off and falls into Calyx -> RENAL COLIC = contraction of the ureter b/c it contains foreign material
What is this?
What is the cause?
How might it manifest clinically?
Amyloidosis

Congo Red stain which when polarized under the light microscope results in an APPLE GREEN BIREFRINGENCE pattern
What is the cause of this?
What stain?
Accumulations of cells composed of proliferating Parietal Epithelial Cells and infiltrating Leukocytes
Crescents
What has proliferated to form Crescents in Rapidly Progressive GN?
Parietal epithelial cells
Type of Glomerulonephritis defined by the lack of anti-GBM Ab's or Immune-complexes by immunofluorescence and EM. Most patients with this type of Rapidly Progressive GN have Antineutrophil Cytoplasmic Antibodies (ANCA), which play a role in some vasculitides
Pauci-immune = ANCA granulonephritis
-Wegener Granulomatosis (c-ANCA)
Nephritic that is usually progressive over a matter of weeks and culminates in severe oliguria
Crescentic GN = Rapidly Progressive GN
List the diseases associated with Nephritic Syndrome
1. Post-streptococcal GN
2. Rapidly Progressive GN
3. Membranoproliferative GN
4. IgA Nephropathy
Unlike Nephritic Syndrome, what 3 things does Nephrotic Syndrome not have?
1. Hematuria
2. Oliguria
3. Hypertension
Unlike Nephrotic Syndrome, what 2 things does Nephritic Syndrome not have?
1. Hyperlipidemia

2. Lipiduria
What is the initial event leading to Nephrotic Syndrome?
derangement in Glomerular Capillary Walls resulting in INCREASED PERMEABILITY TO PLASMA PROTEINS
-low-weight proteins, such as albumin
What is the initial event leading to Nephritic Syndrome?
inflammatory RUPTURE of glomerular capillaries with resultant bleeding into the urinary space
Sclerosis of some, but not all, glomeruli & only a portion of the capillary tuft is involved
Focal Segmental Glomerulosclerosis
Membranous Glomerulonephropathy
-diffuse thickening of capillary wall
-NO cellular proliferation
What Nephrotic Syndrome is this?
Diabetes causes Hyalinizing Arteriolar Sclerosis and increases the susceptibility to the development of these 2 Kidney lesions
1. Pyelonephritis

2. Papillary Necrosis
Focal proliferative Glomerulonephritis with Diffuse Mesangial widening
IgA Nephropathy = Berger Disease