Lecture 21.a - Nephritic Syndrome

Question 1

Nephritic and Nephrotic syndromes are both diseases of the ______.

Answer 1

Glomerulus

Question 2

There is a direct relationship between ____ ____ and total glomerular number.

Answer 2

Birth weight

Question 3

What is the normal number of mesangial cells per mesangial area and what happens to the lumenal size of the capillaries with mesangial expansion?

Answer 3

Normal is 3. With mesangial expansion (more than 3 per mesangial area), the lumenal space of the adjacent capillaries becomes narrowed –> impaired kidney function.

Question 4

The Glomerular basement membrane (GBM) is composed of type ____ collagen. ____ syndrome is an X-linked recessive disease that affects the alpha 5 chain of this collagen. A less common disease that affects the GBM is Thin Basement Membrane disease (which is autosomal ______), caused by a mutation in the Alpha ___ or ___ chains.

Answer 4

Type IV collagen

Alport’s syndrome

Autosomal Dominant

Alpha 3 or 4

Question 5

Podocyte effacement (flattening out of the cell with increased space between them/their interdigitations) is a hallmark of ______ (nephritic or nephrotic?) syndrome.

Answer 5

Nephrotic

Question 6

Rapidly progressive glomerulonephritis (RPGN) is classified as a _____% decline in GFR within ____ months with ____% crescents (layers of proliferating cells in Bowman’s capsule that compress the glomerulus) on renal biopsy.

Answer 6

50%

3 months

50%

Question 7

Glomerulonephritis is considered Focal if ____% or less of the glomeruli are affected. If it’s more than that, it’s considered Diffuse.

Answer 7

50% or less –> focal

Question 8

What does Segmental vs Global refer to with respect to glomerulonephritis?

Answer 8

Segmental –> only part of any one glomerulus is affected, while the other part remains functional.

Global –> all of a single glomerulus is affected.

Question 9

Which is characterized by inflammation, Nephritic or Nephrotic syndrome?

Answer 9

Nephritic

Question 10

The most common cause of Nephritic syndrome world wide is _____ nephropathy.

Answer 10

IgA Nephropathy

Question 11

When there is blood in the urine WITHOUT protein (at least at first), think ____ syndrome or ____ ____ ____ nephropathy. These can be distinguished from Nephritic syndrome bc with Nephritic, you WILL see proteinuria from the beginning.

Answer 11

Alport’s syndrome

Thin Basememnt Membrane Nephropathy

Question 12

Alport’s Syndrome results in thickening of ______ in more than 50% of glomerular loops. Also look for enlarged _____ and _____ expansion.

Answer 12

GBM

Podocytes

Mesangial expansion

Question 13

There are also Autosomal recessive (AR) and autosomal dominant (AD) forms of Alport’s Syndrome.

In the AR form, patients tend to reach ESRD in their _____ decade of life, about a decade sooner than the X-linked form. AD form –> slower progression.

Answer 13

3rd decade

Question 14

The most common non-kidney findings in patients with Alport’s syndrome include _____ and _____ changes/loss.

Answer 14

Vision and Hearing changes/loss. Keep in mind visual changes are not common in the AD form.

Question 15

To distinguish between Alport’s and TBMD look to ____ biopsy, which will show lack of staining of the basement membrane in 80% of Alport’s cases.

Also remember how the GBM will differ between Alport’s and TBMD…

Answer 15

Skin

In Alport’s –> GBM will be THICK

Question 16

Anti-GBM forms of Glomerulonephritis include Anti-GBM disease and Goodpasture’s disease. How can you distinguish between the two?

Answer 16

Goodpasture’s will have LUNG involvement, while Anti-GBM disease is Renal limited.

Question 17

Anti-GBM disease and Goodpasture’s are both categorized as Type 1 RPGNs.

How will their Immunofluorescent staining appear?

How does this compare to Type 2 (immune complex) RPGNs and Type 3 (Pauci Immune, - ANCA associated vasculitis) RPGNs?

Answer 17

Immunofluorescence of Type 1 –> linear outline of capillaries

Type 2 –> granular staining

Type 3 –> no staining

Question 18

Presence of _______ RBCs has 98% Specificity for Glomerulonephritis. However, it only has 52% Sensitivity (negative test not reliable). Just keep in mind that only 10% of cases of Hematuria WITHOUT Proteinuria are glomerular in origin –> must rule out _____.

Answer 18

Dysmorphic

Cancer

Question 19

What is the gold standard for diagnosis of Glomerulonephritis?

Answer 19

Renal Biopsy

Question 20

Goodpasture’s syndrome patients have auto-antibodies against the _____ chain of Type IV collagen, which is mainly found in GBM and Alveolar basement membrane (so again, look for LUNG involvement!)

Answer 20

Alpha 3 chain

Question 21

Post-infectious Glomerulonephritis patients typically experience AKI ____-____ days after Strep infection. Patients will present with ____ colored urine, flank pain, and histology will show _______ humps.

Answer 21

10-14 days

Tea-colored urine

Subepithelial humps (immune complex deposition between GBM and visceral epithelium.)

Question 22

If Staph is the infectious agent in Infection-related Glomerulonephritis, when does AKI typically occur?

Answer 22

Typically DURING the infection, which helps distinguish it from post-strep Glomerulonephritis.

Question 23

Lupus nephritis is most common in which demographics?

Answer 23

Young, female, and African American.

Question 24

Class III and IV Lupus Nephritis are aggressive and proliferative. Which is focal, which is diffuse?

Answer 24

Class III –> focal

Class IV –> Diffuse

Question 25

Membranoproliferative Glomerulonephropathy (MPGN) is a Nephritic disease that can be ______ or _____ mediated. ____ is the most common causative agent for _____-mediated.

Answer 25

Immune or Compliment-mediated

HCV

Immune-mediated

Question 26

For IgA nephropathy, look for patients to present with gross hematuria ___ - ___ hours post-infection or exercise. Biopsy will show ______ deposition of IgA.

Answer 26

24-48 hrs

Mesangial deposition

Question 27

Pauci-immune glomerulonephritis (ANCA-related) is the most common cause of Glomerulonephritis in which demographic?

Answer 27

Older patients, with white male predominance.

Question 28

Remember that anyone with proteinuria should be treated with _____ or _____.

Answer 28

ACE inhibitors or ARBs