Pathoma Nephritic Syndrome

Question 1

Basic idea of nephritic syndrome

Answer 1

Glomerular inflammation and bleeding

Question 2

Key sign of glomerular bleeding

Answer 2

RBC casts and dysmorphic RBCs in the urine

Question 3

Glomerular inflammation will cause these:

Answer 3

1. Limited proteinuria (<3.5 g/day)
2. Oliguria and Azotemia
3. Salt retention with periorbital edema and HTN

Question 4

Biopsy of nephritic syndrome

Answer 4

Hypercellular inflammed glomeruli

Question 5

What causes the damage in nephritic syndrome

Answer 5

Immune-complex deposition activates complement. C5a attracts neutrophils which mediate the damage

Question 6

PSGN? What is it and what exactly causes it?

Answer 6

Post Strep Gomerular nephritis

Arises after a GAS (Beta hemolytic) infection of skin or pharynx

Question 7

PSGN occurs with nephritogenic strains. What does this mean?

Answer 7

Carry M protein virulence factor.

Question 8

Presentation of PSGN and when it occurs

Answer 8

2-3 weeks after infection

Hematuria
Oliguria
HTN
Periorbital edema

Question 9

Who gets PSGN?

Answer 9

Usually kids but adults can get it

Question 10

Biopsy of PSGN

Answer 10

Hypercellular, inflammed glomerulus on H and E

Granular IF (immune complexes)

Subepithelial humps on EM (still on top of basement membrane though)

Question 11

Treatment for PSGN

Answer 11

Nothing, it’ll pass through the basement membrane and disappear.

Children rarely progress to renal failure and some adults develop RPGN

Question 12

What is RPGN?

Answer 12

Rapidly progressing Glomerular Nephritis

Nephritic syndrome that progresses to renal failure in weeks to months

Question 13

RPGN is characterized how on biopsy?

Answer 13

Crescents in Bowman’s space comprised of fibrin and macrophages

Question 14

IF can lead to three types of findings. What are they?

Answer 14

1. Linear - Antibodies line up right underneath the basement membrane. Indicative of an anti-basement membrane antibody
2. Granular - indicates imune complex deposition
3. Negative (pauci-immune = not really immune)

Question 15

What does a linear IF mean?

Answer 15

Goodpasture Syndrome Where you get antibodies attacking collagen in glomerular and alveolar basement membrane.

Question 16

How does Goodpsture’s present and in whom?

Answer 16

Hematuria and hemoptysis, clasically seen in young male adults

Question 17

Granular IF is indicative of what?

Answer 17

PSGN or diffuse proliferative glomerulonephritis

Question 18

What is diffuse proliferative glomerulonephritis and what causes it? Who gets it?

Answer 18

Due to diffuse antigen-antibody complex deposition, usually sub endothelial. Most comon in SLE

Question 19

Negative IF is indicative of what?

Answer 19

1. Wegners granulomatosis
2. Microscopic Polyangiitis
3. Churg-Strauss

Question 20

Wegner granulomatosis is associated with _____. MP and C-S syndrome is associated with _____.

Answer 20

c-anca

p-anca respectively

Question 21

Distinguish MP and C-S

Answer 21

Granulomatous inflammation, eosinophilia, and asthma distinguish C-S from MP

Question 22

Distinguish between Goodpastures and Wegners

Answer 22

Both have hematuria, hemoptysis, crescent shaped glomeruli spaces and failing glomeruli.

But remember Wegner’s also hits the nasopharynx, so you’ll look for sinus involvement

Question 23

What is IgA nephropathy? How common is it?

Answer 23

IgA immune complex deposition in mesangium of glomeruli. This is the most common nephropathy worldwide

Question 24

How does IgA nephropathy present?

Answer 24

Episodic gross or microscopic hematuria with RBC casts, usually following mucosal infections.

May progress to renal failure

Question 25

Discuss Alport Syndrome and who gets it?

Answer 25

Inherited defect in Type IV collagen, most commonly x-linked Results in thinning and splitting of glomerular basement membrane

Question 26

How does Alport's present?

Answer 26

Presents as isolated hematuria, sensory hearing loss, and ocular disturbances all due to basement membrane issues