Path 2 Glomerular Disease

Question 1

Normal glomerular measurement

Answer 1

BM and podocytes are same width

Question 2

Inside v outside lining gaps

Answer 2

• Fenestrae inside between Endothelial cells

- Filtration slits outside between Epithelial cells (podocytes)

Question 3

Nephrotic syndrome

Answer 3

Increased BM permeability

• Proteinuria, hypoalbuminemia
• Generalized edema
• Hyperlipidemia and hypercholesterolemia

Question 4

Minimal change disease

Answer 4

Lipoid nephrosis (nephrotic syndrome)

• Age 2-6
• No immune complex deposition
• Tx with steroids

Question 5

Minimal change disease gross appearance

Answer 5

Lipid laden cortices

Question 6

Minimal change disease histology (3)

Answer 6

• Normal glomeruli
• Lipid accumulation in proximal convoluted tubules
• Fusing of foot processes (epithelial)

Question 7

Focal segmental glomerulosclerosis

Answer 7

Sporadic partial glomerular scarring

• Same clinical as MCD
• Older patients, can’t Tx with steroids
• IgM and C3 immune complex deposition

Question 8

Focal segmental glomerulosclerosis

Answer 8

• Primary

- Secondary (HIV, heroin, IgA nephropathy)

Question 9

Membranous GN

Answer 9

Nephrotic syndrome in teenagers and young adults

• IgG, C3 immune complex deposition
• Doesn’t respond to steroids

Question 10

Membranous GN stain

Answer 10

PAS

Question 11

Minimal Change Disease stain

Answer 11

Fat stain

Question 12

Membranous GN histology (3)

Answer 12

• Thickened capillary loops
• Spike and dome appearance
• Granular immunofluorescence

Question 13

Membranous GN EM

Answer 13

Thickened BM with immune complex nodules

Question 14

Membranous GN etiology

Answer 14

• Idiopathic

- SLE, infections, drugs, malignancy

Question 15

Diabetic nephropathy

Answer 15

Nephrotic syndrome

Question 16

Diabetic nephropathy glomerulus histology (4)

Answer 16

Kimmelstiel-Wilson nodules

• Fibrin caps
• Increase in mesangial matrix
• Diffuse glmerulosclerosis

Question 17

Diabetic nephropathy extra-glomerulus histology (2)

Answer 17

• Sclerosed afferent & efferent arterioles

- Armanni-Ebstein lesions (glycogen in tubules) in chronic hyperglycemia

Question 18

Diabetic nephropathy EM (2)

Answer 18

• Markedly thickened BM

- Mesangial matrix accumulation

Question 19

Amyloidosis

Answer 19

Nephrotic syndrome

-Subendothelial and mesangial deposits

Question 20

Amyloidosis EM

Answer 20

Criss-cross fibrillary pattern

Question 21

Amyloidosis histology (2)

Answer 21

• Congo Red stain

- Birefringence under polarized light

Question 22

Lupus Nephropathy Class I

Answer 22

No observable renal involvement

Question 23

Lupus Nephropathy Class II

Answer 23

Mesangial glomerulonephritis

Question 24

Lupus Nephropathy Class III

Answer 24

Focal proliferative glomerulonephritis

-Less than half of glomeruli involved, but individual damage can be extensive

Question 25

Lupus Nephropathy Class IV

Answer 25

Diffuse proliferative glomerulonephritis

Question 26

Lupus Nephropathy Class V

Answer 26

Membranous glomerulonephritis | -Intra and extramembranous immune complex deposition

Question 27

Lupus Nephropathy histology (5)

Answer 27

- Extensive inflammatory glomerular scarring - Subendothelial and mesangial immune complex deposition - Wire loop lesions - Tubular reticular structures - Fingerprint patterns pathognomonic

Question 28

Nephritic syndrome

Answer 28

Inflammatory rupture of glomerular capillaries - oliguria - hematuria (w/RBC casts) - azotemia - HTN

Question 29

Acute Proliferative Poststreptococcal GN

Answer 29

Prototypical nephritis - IgG/C3 Immune complexes between epithelial foot processes -> PMN attack -> hemorrhage - Complete recovery in all children, 60% adults - Analogous to rabbit "serum sickness"

Question 30

PSGN gross appearance

Answer 30

Petechial hemorrhages covering surface of kidney

Question 31

PSGN histology

Answer 31

- Glomeruli enlarged, hypercellular (mesangial, endothelial, PMN), "bloodless" - Granular immunofluorescence

Question 32

PSGN EM

Answer 32

Subepithelial immune complexes

Question 33

PSGN lab levels

Answer 33

- Decreased serum C3 | - Increased ASO titer

Question 34

Rapidly Progressive GN | -mechanism, cause, prognosis

Answer 34

Cresentic GN - Proliferation of parietal epithelial cells of bowmans capsure - Can be complication of PSGN - Renal failure over weeks to months

Question 35

Crescentic GN (RPGN) types

Answer 35

- ANCA positive - ANCA negative - Anti-GBM (Goodpasture) - Immune complex (ASO, SLE, IgA)

Question 36

Goodpasture syndrome

Answer 36

Antiglomerular BM antibodies - attacks glomeruli and alveoli - linear immunofluorescence for IgG

Question 37

Alport syndrome - cause, histology, clinical

Answer 37

Type IV collagen mutation (alpha-5 chain) - Rarefaction and splitting of BM lamina densa - Hereditary nephritis, nerve deafness, ocular disorders (cataracts, lens dislocation)

Question 38

Berger disease

Answer 38

IgA nephropathy - IgA deposits in mesangium - Recurrent hematuria in youth, often benign

Question 39

IgA nephropathy as a component

Answer 39

Henoch-Schonlein purpura - Skin purpura - GI pain & bleeding - Joint arthritis - Kidney IgA nephropathy

Question 40

Membranous GN v Membranoproliferative GN

Answer 40

BM v BM and Mesangium

Question 41

Membranoproliferative GN histology (4)

Answer 41

- Tram-track BM (doubled) - Lobular pattern - Mesangial proliferation (hypercellular) - Collapse of capillaries

Question 42

Type 1 Membranoproliferative GN

Answer 42

Immune complex nephritis - unknown antigen - striking tram-track

Question 43

Type 2 Membranoproliferative GN

Answer 43

Dense Deposit Disease - C3 nephritic factor - Serum C3 markedly reduced

Question 44

Chronic GN

Answer 44

End-stage kidney - Marked renal failure - HTN - Small pale kidneys - Hyaline arteriolosclerosis - Interstitial fibrosis