Renal Pathology - Other

Question 1

IgA nephropathy pathogenesis (4):

Answer 1

• Genetic or acquired abnormality of immune regulation
• Increased synthesis of IgA following a respiratory or GI exposure
• IgA1 deposits in mesangium
• Alternative complement pathway is activated

Question 2

IgA nephropathy - light microscopy:

Answer 2

Mesangial widening due to proliferation and endocapillary proliferation

Question 3

IgA nephropathy - IF (2):

Answer 3

• Mesangial deposition of IgA

- C3, properdin, IgG and IgM often

Question 4

IgA nephropathy - EM:

Answer 4

Electron dense deposits in mesangium

Question 5

Alport syndrome (4):

Answer 5

• Hereditary syndrome
• Hematuria with progression to chronic renal failure
• Nerve deafness
• Eye disorders

Question 6

Alport syndrome - pathogenesis (2):

Answer 6

• Abnormal chains of collagen IV

- Defective assembly of collagen IV

Question 7

Alport syndrome - X-linked:

Answer 7

Mutation of COL4A5

Question 8

Alport syndrome - autosomal forms:

Answer 8

Mutations in COL4A3 and COL4A4

Question 9

Alport syndrome - EM (3):

Answer 9

Early:
- GBM thinning
Fully developed:
- GBM alternating thick and thin
- Splitting and layering of lamina densa

Question 10

Alport syndrome - immunohistochemistry:

Answer 10

Abs to alpha3, alpha4 or alpha5 collagen fail to stain GBM and tubular BM

Question 11

Alport syndrome - clinical features (3):

Answer 11

• Hematuria, gross or microscopic
• Red cell casts
• Proteinuria may develop (nephrotic syndrome rare)

Question 12

Chronic glomerulonephritis - gross morphology (2):

Answer 12

• Kidneys contracted

- Cortex thinned with granular surface

Question 13

Chronic glomerulonephritis - histology (4):

Answer 13

• Early: evidence of primary disease
• Eventual obliteration of glomeruli
• Arterial and arteriolar sclerosis from HTN
• Atrophy of tubules

Question 14

Chronic glomerulonephritis - uremic complications (4):

Answer 14

• Uremic pericarditis
• Uremic gastroenteritis
• Secondary hyperparathyroidism
• LV hypertrophy from HTN

Question 15

Henoch-Schoenlein purpura - light microscopy (2):

Answer 15

• Mesangial proliferation, focal or diffuse

- Endocapillary to crescentic glomerulonephritis

Question 16

Henoch-Schoenlein purpura - IF:

Answer 16

Deposition of IgA, sometimes IgG and C3 in mesangial region

Question 17

Henoch-Schoenlein purpura - skin lesions (3):

Answer 17

• Subepidermal hemorrhages
• Necrotizing vasculitis of dermis small vessels
• IgA deposits in walls of small vessels

Question 18

Diabetic nephropathy - morphology (3):

Answer 18

• Capillary basement membrane thickening
• Diffuse mesangial sclerosis
• Nodular glomerulosclerosis

Question 19

Glomerular lesions associated with amyloidosis (4):

Answer 19

• Deposits of amyloid in the mesangium and glomerular capillary walls
• Eventually the glomerulus is completely obliterated
• Deposits in renal interstitium and vessel walls
• May present with nephrotic syndrome