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M2 Renal/Respiratory > Kidney Pathology 3 (Wolfgram) > Flashcards

Kidney Pathology 3 (Wolfgram) Flashcards

1
Q

What is the leading cause of ESRD (in most western societies)?

A

Diabetic Nephropathy

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2
Q

Describe the pathogenesis of hyperfiltration in diabetic nephropathy

A

Early onset

  • GRF increases due to glucose-dependent dilation of the afferent arterioles (via vasoactive mediators)
  • Hyperfiltration increases colloid osmotic pressure in the post-glomerular capillaries, leading to increase sodium reabsorption in the proximal convoluted tubule
  • Increased angiotensin II causes hypertrophic PT growth

** can be corrected with good glycemic control

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3
Q

Describe the mesangial changes of diabetic nephropathy

A
  • mesangial expansion (increased cell number and size, increased deposition of ECM)
  • Nodular diabetic glomerulosclerosis (Kimmelstiel-Wilson lesion)
  • mediated by glucose and glucose-dervice AGEs
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4
Q

What is a Kimmelstiel-Wilson lesion?

A

A type of acellular nodular diabetic glomerulosclerosis commonly seen in diabetic nephropathy

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5
Q

Describe the pathogenesis of the proteinuria seen in DN.

A
  • Widening of the GBM
    • accumulation of Type-IV collagen decreases the charge density (due negatively charged heparin sulfate) normally responsible for repelling serum proteins
  • Podocyte changes -> decreased coverage of the BM
    • Increased width of foot processes
    • Apoptosis triggered by AngII and TGF-beta
    • Migration suppressed by AngII

Serum proteins ultimately cross the basement membrane due to disruption in its texture, gaps, and holes.

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6
Q

Describe the pathogenesis of the fibrosis seen in DN

A

Early onset -> correlates with prognosis/progression

Triggered by growth factor release (TGF-beta and AngII) and glucose/AGEs

tubular cells change phenotype to fibroblasts

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7
Q

Describe the functional and structural characteristics of the following stages of DN

  1. Pre
  2. Incipient
  3. Overt
A

Functional

  1. Pre: increased GFR
  2. Incipient: microalbuminuria, hypertesion, reduced or normal GFR
  3. Overt: proteinuria, decreased GFR, nephrotic syndrome

Structural

  1. Pre: renal hypertrophy
  2. Incipient: mesangial expansion, GBM thickening, arteriolar hyalinosis
  3. Overt: mesangial nodules (Kimmelstiel-Wilson lesions), tubulointerstitial fibrosis
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8
Q

Name and describe the 5 major stanges of DN

A
  • Stage 1: onset of diabetes
    • increased GFR due to hyperfiltration
    • glomerular hypertrophy and increased renal size
    • reversible, transient albuminuria
  • Stage 2: biopsy-positive, asymptomatic
    • mesangial expansion
    • GBM thickening
  • Stage 3: early nephropathy
    • hypertension
    • persistent microalbuminuria (30-300mg/day)
  • Stage 4: overt proteinuria
    • urinary albumin >300mg/day
    • declining GFR -> ESRD within 7-10 years
    • Retinopathy (90-95% of patients)
  • Stage 5: end-stage renal disease
    • requires renal replacement
    • ~15 years after onset of T1DM with proteinuria
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9
Q

Describe the incidence of macrovascular complications in DN

A

5X more frequent, including stroke, CAD, PVD

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10
Q

What are some sequelae of autonomic polyneuropathy brought on by DN?

A

silent angina

gastroparesis

erectile impotence

detrusor paresis

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11
Q

Who is more likely to develop diabetic retinopathy in DN?

A

Almost all patients with type-1 develop DR

50%-60% of type-2 develop DR

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12
Q

True or false: increased albuminuria is associated with increased mortality in DN patients.

A

True

mortality is more or less proportional to level of proteinuria

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13
Q

Name (5) general treatment strategies for management of DN

A
  • hypertension therapy
  • glucose control
  • reduction of proteinuria
  • lipid-lowering therapy
  • lifestyle modification
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14
Q

What is the recommended blood pressure target for DN patients with hypertension (according to JNC8)?

A

JNC8 goal: 140/90 mmHg

JNC7 goal: 130/80 mmHg

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15
Q

How to ACE inhibitors reduce proteinuria in DN?

A

Blockade effects of AngII, including the hemodynamic and non-hemodynamic effects of AngII.

Long term renoprotective (despite significant decrease in GFR) -> reduces proteinuria

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16
Q

Describe the usual lipid profile of a DN patient

What drug family is used to manage this?

What are the lipid profile goals under this therapy?

A

Low HDL, high TGs, smaller LDL particles

manage with statins

LDL goal: <100mg/dL (or <70mg/dL for patients with CVD)

17
Q

Name two major lifestyle modifications indicated for DN patients

A
  • smoking cessation
  • weight reduction
18
Q

20% of cases of amyloidosis light chains affecting the kidney are caused by what?

A

Multiple myeloma (single clone of B-cells)

19
Q

Deposition of lambda light chains are typically seen in what disease?

A

Amyloidosis

20
Q

Describe the kidney manifestations of amyloidosis

A

enlarged kidney despite absent hypertension

albuminuria without hematuria

tubular defects due to amyloid deposits

Renal tubular acidosis (part of Fanconi syndrome)

Polyuria-polydipsia (defects in urine concentration)

21
Q

What disease will show apple-green birefringence on congo-red stain?

A

amyloidosis

22
Q

Name some other organ systems affected by amyloidosis

A
  • heart (restrictive cardiomyopathy)
  • GI motility disturbances, malabsorption, obstruction, hemorrhage
  • macroglossia
  • splenomegaly
  • peripheral neuropathies
  • skin purpura, papules, nodules, plauques (mostly face and upper trunk)
  • Joint pain and swelling (especially shoulder)
23
Q

kappa light chains are typically seen with what?

This disease is also associated with what?

What are the general clinical features?

A

Light Chain Deposition Disease (LCDD)

Strongly associated with multiple myeloma (50%)

Clinical features: proteinuria, hematuria, chronic renal insufficiency

24
Q

Describe the histological features of LCDD

A
  • LM: nodular glomerulosclerosis
  • IF: positive kappa staining, mostly confined to the mesagium
  • EM: granular light chain deposition in the TBM
25
Q

Describe the general decision flow for differentiating amyloidosis, LHCDD/HCDD, and other immunoglobulin deposition diseases

A
  • Stain with congo red
  • If positive apple-green birefringence -> amyloid
  • If negative, stain IF with anti-kappa/lambda
  • If positive -> LHCDD/HCDD
  • If negative -> other
26
Q

What is the inheritance pattern of Alport Syndrome?

What is the major defect?

A

X-linked recessive (80%)

may also be autosomal recessive

Defect in basement membrane, generally due to a mutation of the COL4A5 gene on Xq22 -> defect in type-IV collagen

27
Q

Describe the major renal and extrarenal manifestations of Alport Syndrome

A
  • Hematuria
  • Gradually-developing proteinuria (all males with XLAS, all patients with ARAS)
  • Hypertension
  • ESRD (90% XLAS males by age 40, less prevalent in female XLAS)
  • Cochlear defects (adherance defect in organ of Corti -> 80% males)
  • Ocular defects (lenticonus, maculopathy, M > F)
  • Leiomyomatosis of the esophagus and tracheobronchial tree
28
Q

If you encounter a male with hearing loss and kidney disease, what genetic disease should be considered in your differential?

A

Alport Syndrome

29
Q

Describe Alport Syndrome histologically

A
  • LM: normal glomeruli, may see sclerosis or fibrosis later in disease
  • IF: negative or non-specific
  • EM: variable thickening/thinning (“Basket Weaving”) and lamellation (splitting) of the GBM
30
Q

What are the treatment options for Alport Syndrome

A

No disease-specific therapies, renal replacement ultimately necessary (all males)

Consider RAAS blockade

2-3% of renal transplants for Alport Syndrom will develop anti-GBM disease (immune system native to collagen Type-IV, will consider foreign)

31
Q

Benign Familial Hematuria is also known by what name?

What is the inheritance pattern?

What are the major clinical features?

A

Thin Basement Membrane Disease (TBMD)

autosomal dominant

  • Continuous or intermittent microhematuria
  • Generally no renal insufficiency
  • Extra-renal involvement is rare
32
Q

What are the morphologic features of TBMD?

A
  • LM: normal glomeruli
  • IF: negative
  • EM: thin GBM (<=200nm), no lamellation (splitting)

Remember: Alport Syndrome shows lamellation and ‘woven’ thinning. TBMD shows no lamellation and the thinning is uniform.

33
Q

What is the treatment/clinical management of TBMD?

A

Reassurance

Follow closely (BMP, BP, and urinalysis every 1-2 years) to monitor for rare progression to ESRD

M2 Renal/Respiratory (50 decks)

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