Nephritic Syndromes

Question 1

When it involves the glomeruli, what are nephritic syndromes associated with?

Answer 1

Hematuria and RBC casts in the urine (muddy, brown/red urine)

Question 2

Nephritic Syndromes are also associated with…

Answer 2

azotemia, oliguria, hypertension (salt retention) and proteinuria (less than 3.5 g/day)

Question 3

Acute Poststreptococcal Glomerulonephritis is caused by

Answer 3

• Group A Beta-Hemolytic Strep Infection.

- Typically symptoms show up 2-3 weeks after infection clears.

Question 4

LM Findings of Acute Poststreptococcal Glomerulonephritis

Answer 4

Glomeruli enlarged and hyper cellular, neutrophils, and “lumpy-bumpy” appearance.

Question 5

EM Findings of Acute Poststreptococcal Glomerulonephritis

Answer 5

SUBEPITHELIAL IC HUMPS

Question 6

IF Findings of Acute Poststreptococcal Glomerulonephritis

Answer 6

Granular Appearance due to IgG, IgM, and C3 deposition along the GBM and mesangium.

Question 7

Pts with Acute Poststreptococcal Glomerulonephritis present with

Answer 7

Peripheral/Periorbital Edema, dark urine (RBC casts), and hypertension

Question 8

Treatment for Acute Poststreptococcal Glomerulonephritis?

Answer 8

Typically resolves spontaneously.

Question 9

LM and IF Findings of Rapidly Progressive Glomerulonephritis (RPGN)

Answer 9

Crescent-moon shape: Crescents consist of Fibrin and plasma proteins with glomerular parietal cells, monocytes and macrophages.

Question 10

EM Findings of RPGN

Answer 10

Rupture of GBM

Question 11

Type I RPGN

Answer 11

Goodpasture’s Syndrome (Type II Hypersensitivity)

Question 12

Type III RPGN

Answer 12

Wegener’s, Microscopic Polyangiitis and Churg-Strauss

Question 13

Type II RPGN IF and EM Findings

Answer 13

Granular Appearance and possibly SubEPIthelial Deposits

Question 14

Findings of Goodpasture’s Syndrome

Answer 14

Antibodies to GBM (IgG and C3) and Alveolar Basement Membrane lead to linear IF

Question 15

Pt with Goodpasture’s Presents with

Answer 15

Hematuria/Hemoptysis

Question 16

Autoantibodies of Goodpasture’s bind to what?

Answer 16

Noncollagenous chain of alpha-3 Type IV Collagen

Question 17

Wegener’s Pts present with…

Answer 17

• Lung, Kidney, and Nasopharyngeal Problems (Be careful not to confuse with Goodpasture’s!)
• So Hemoptysis/Hematuria/SINUSITIS

Question 18

What is Wegener’s?

Answer 18

Granulomatosis with polyangiitis

Question 19

What do you find with Wegener’s?

Answer 19

c-ANCA

Question 20

What do you find with Microscopic Polyangiitis or Churg-Strauss?

Answer 20

p-ANCA

Question 21

Most common Cause of Death in SLE?

Answer 21

Diffuse Proliferative Glomerulonephritis (DPGN)

Question 22

LM Findings of Diffuse Proliferative Glomerulonephritis (DPGN)?

Answer 22

Wire looping of capillaries

Question 23

EM and IF Findings of Diffuse Proliferative Glomerulonephritis (DPGN)?

Answer 23

• SubENDOthelial and sometimes intramembranous IgG-based ICs often with C3 deposition
• Granular

Question 24

Berger’s Disease Pts present with

Answer 24

Childhood with Upper Respiratory Infection or acute Gastroenteritis

Question 25

Berger’s Disease Associated with

Answer 25

Gluten Enteropathy and Celiac Sprue

Question 26

LM, EM, and IF Findings in Berger’s Disease

Answer 26

• Mesangial Proliferation

- Mesangial IgA IC Granular Deposits

Question 27

Mutation in Alport Syndrome

Answer 27

In Type IV Collagen alpha chains (mostly alpha5) leads to a split basement membrane (basket weave appearance)

Question 28

Alport Syndrome Associated with

Answer 28

Glomerulonephritis, Deafness, and , less commonly, eye problems.