Non-inflammatory and Inflammatory Glomerular diseases

Question 1

Nephritic Syndrome

Answer 1

Clinical presentation of inflammatory glomerulonephritis

• Microscopic hematuria
• Proteinuria
• Hypertension
• Edema

Question 2

Nephrotic syndrome

Answer 2

Clinical presentation of non-inflammatory glomerulopathy

• Proteinuria (>3.5g/d)
• Hyperlipidemia
• Edema
• Hypoproteinemia
• Urinalysis is key to diagnosis

Question 3

Complications of nephrotic syndrome

Answer 3

• Hypercoag
• Edema
• infection
• Accelerated atherogenesis

Question 4

Histopath of minimal change disease

Answer 4

Everything is normal (light, silver stain, immunofl) except electron microscopy which shows podocyte effacement

Question 5

Etiology of minimal change disease

Answer 5

Unknown but can be secondary to NSAIDs or neoplasms

Question 6

Treatment of minimal change disease

Answer 6

Steroids, podocytes look normal

Question 7

Light micro: FSGS

Answer 7

• Focal, segmental sclerosis
• Segmental collapse with adhesions to bowman’s cap
• Podocyte injury loss

Question 8

Immunofl: FSGS

Answer 8

-IGM and C3 in collapsed mesangium

Question 9

EM: FSGS

Answer 9

-Podocyte effacement, hyalin accumulation in cap loops–>collapse

Question 10

Causes of FSGS

Answer 10

Podocyte injury leadin got proteinuria and glomerulosclerosis

Question 11

What is the point of no return?

Answer 11

When about 40% of podocytes are lost, get global sclerosis

Question 12

What attenuates podocyte loss and scarring?

Answer 12

RAAS inhibition

Question 13

Why is podocyte injury so bad?

Answer 13

Don’t regenerate, leads to scarring and decreased GFR

Question 14

Membranous glomerulopathy histology: PAS/silver stain

Answer 14

Sclerosis with basement membrane spikes

Question 15

Membranous glomerulopathy histology: Trichrome

Answer 15

Sub epithelial proteinaceous deposits

Question 16

Membranous glomerulopathy histology: Immunofl

Answer 16

IgG and C3 in the granular capillary loop

Question 17

Membranous glomerulopathy histology: EM

Answer 17

• Podocyte effacement
• Subepi electron dense deposits
• New BM surrounding immune complex deposits

Question 18

Most common antibody found in membranous glomerulopathy?

Answer 18

M-type PLA2R

Question 19

Secondary etiology of Membranous glomerulopathy

Answer 19

• Infectious: Hep B, C, malaria etc
• Neoplasms
• Meds (gold salts, penicillamine)
• Lupus

Question 20

What is glomerular inflammation a response to?

Answer 20

Final common response to capillary injury–>scar

Question 21

Steps of programmed response to capillary injury

Answer 21

1. Holes/cap wall injury
2. Proliferation (WBCs enter tufts)
3. Acellular crescent formation (ECM fills bowman’s)
4. Cellular crescent formation (cells in matrix produce scar)
5. Glomerular and interstitial scarring

Question 22

Mechanisms that result in capillary injury?

Answer 22

• Antibody mediated
• Immune complex deposits
• Mechanism X (vasculitis)

Question 23

Good pastures disesase

Answer 23

antibody mediated pulmonary-renal synrome with hemoptysis and RPGN

Question 24

Differential diagnosis for pulmonary-renal syndrome?

Answer 24

Good pastures, lupus, ANCA small v vasculitis

Question 25

Good pastures in light micro

Answer 25

- Hypercellular glomerulus - Firbin - Crescents in bowman's space

Question 26

Good pastures in immunofl

Answer 26

- Linear GBM stain | - Stringy fibrin in bowman's

Question 27

Good pastures in EM

Answer 27

- Compressed glomerular tuft - Fibrin in bowman's - No complexes

Question 28

Treatment of Good pastures

Answer 28

- Plasma exchange - Immunosupp - Rituximab

Question 29

Post-strept GN

Answer 29

Immune complex mediated disease around 21 days post-strept with RPGN

Question 30

Post-strept GN light micro

Answer 30

- Diffuse, proliferative exudative GN - Increased PNMs/glomerular cells - Occasional subepi deposits - Accentuated lobular structure

Question 31

Post-strept GN immunofl

Answer 31

Granular IgG, C3

Question 32

Post-strept GN EM:

Answer 32

- large subepi deposits - no sclerotic rxn - subepi and mesangial deposits

Question 33

Lupus nephritis

Answer 33

Immune complex with RPGN, can also present with nephrotic syndrome

Question 34

Lupus nephritis Immunofl

Answer 34

"Full house pattern" stains for all | -granular cap and mesangial loop

Question 35

Lupus nephritis LM

Answer 35

- Prolif in mesangium or lots of prolif everywhere - WIRE LOOPs=thick cap loops - Subendo deposits or epith

Question 36

Lupus nephritis EM

Answer 36

- Mesangial, subepi and sub endo deposits | - Effaced podocytes

Question 37

IgA nephropathy

Answer 37

Immune complex disease that is slowly progressive with edema and gross hematuria (usually non-systemic)

Question 38

systemic version of IgA nephropathy

Answer 38

Henoch Schoenlein purpura (HSP) - Systemic vasculitis in childhood - RPGN, rash, colitis, arthritis

Question 39

IgA nephropathy light M

Answer 39

- Hypercellularity in mesangium | - Focal crescents

Question 40

IgA nephropathy immunofl

Answer 40

Granular in mesang, igA and C3

Question 41

IgA nephropathy EM

Answer 41

Mesangial electron dense deposits

Question 42

ANCA-Associated SV Vasc

Answer 42

Pauci-immune GN (mech X) that mimics common diseases

Question 43

ANCA-Associated SV Vasc light M

Answer 43

Vasculitis with fibrinoid necrosis necrotizing focal GN with crescents

Question 44

ANCA-Associated SV Vasc siver stain

Answer 44

focal crescents, segmental necrosis (loss of BM)

Question 45

ANCA-Associated SV Vasc Fl and EM?

Answer 45

None