Crr Week 8: Kidney In Systemic Diseases

Front 1

What are the 2 glomerular manifestations of vasculitis?

Back 1

1. Microscopic polyangiitis (p-ANCA)
2. Wegener's granulomatotis (c-ANCA)

Front 2

What are the 2 glomerular manifestations of infective endocarditis?

Back 2

1. Proliferative GN
2. MPGN

Front 3

What are the 2 glomerular manifestations of coagulopathies?

Back 3

1. TTP-HUS
2. DIC

Front 4

What are the 2 glomerular manifestations of plasma cell dycrasias?

Back 4

1. Light chain deposition disease
2. Amyloidosis

Front 5

Which glomerular disease is associated with neoplasms?

Back 5

Membranous GN

Front 6

Which glomerular disease is associated with a heredo-familial disease?

Back 6

Alport synrome

Front 7

List 5 autoimmune ("collagen vascular") diseases.

Back 7

1. Systemic lupus erythematosus
2. Wegener's granulomatosis
3. Polyarteritis nodosa
4. Progressive systemic sclerosis (Scleroderma)
5. Rheumatoid arthritis

Front 8

What characteristic rash is a common manifestation of SLE?

Back 8

Malar rash ("butterfly" rash)

Front 9

Which drug is known to correlate with onset of SLE?

Back 9

Procainamide

Front 10

List some factors that may play a role in the pathogenesis if SLE.

Back 10

1. Environmental --> infections (EBV), drugs
2. Genetic (HLA antigens)
3. Abnormalities in immune system (autoantibodies)
4. Hormonal factors

Front 11

In lupus nephritis, where do circulating immune complexes deposit within the kidney?

Back 11

Anywhere in the glomerulus
(mesangium, subendothelial, subepithelial)

*May also deposit in vessels, tubular basement membranes, and interstitium.

Front 12

What percentage of patients with lupus develop clinical evidence of renal disease?

Back 12

25%

Front 13

List the terminology that corresponds to each class of Lupus Nephritis

Back 13

I : Minimal mesangial LN
II: Mesangial proliferative LN
III: Focal lupus nephritis
IV: Diffuse lupus nephritis
V: Membranous LN
VI: Advanced sclerosis LN

Front 14

Which class of LN is normal by light microscopy with mesangial deposits seen on IF & EM?

Back 14

Class I (minimal mesangial LN)

Front 15

Which class of LN is associated with msangial hypercellularity and mesangial deposits?

Back 15

Class II (mesangial proliferative LN)

Front 16

Which class of LN is associated with segmental of global proliferation in <50% of glomeruli?

Back 16

Class III (focal lupus nephritis)

Front 17

Which class of LN is associated with segmental or global proliferation in >50% of glomeruli?

Back 17

Class IV (diffuse lupus nephritis)

Front 18

Which class of LN is associated with subepithelial deposits +/- mesangial deposits?

Back 18

Membranous LN

Front 19

Which class of LN is associated with >90% of glomeruli globally sclerotic, with no residual activity?

Back 19

Class VI (advanced sclerosis LN)

Front 20

Class V lupus nephritis can be combined with which other classes?

Back 20

Class III or IV

Front 21

What are "wire loop" lesions?

Back 21

Massive subendothelial immune complex deposits

*Seen in lupus nephritis

Front 22

What is the treatment for Class I and II lupus nephritis?

Back 22

No therapy for renal disease
(may need treatment for extra-renal manifestations)

Front 23

What is the treatment for Class III and IV lupus nephritis?

Back 23

1. Cyclophosphamide
2. Corticosteroids (prednisone)

Front 24

What is the treatment for Class V lupus nephritis?

Back 24

Controversial
1. If at high risk for progression: 4-6 month trial of aggressive immunosuppressive therapy

Front 25

List 4 chronic complications of diabetes

Back 25

1. Retinopathy
2. Cardiovascular disease
3. Nephropathy
4. Neuropathy

Front 26

What is the most common cause of patients starting dialysis therapy?

Back 26

Diabetic nephropathy

Front 27

The onset of overt renal disease typically occurs how many years after the diagnosis of DM?

Back 27

15 - 20 years

Front 28

Is the frequency and rate of progression of diabetic nephropathy different between type 1 and type 2 diabetes?

Back 28

No, the progression appears to be similar.

*Since Type II DM is more common than Type I, the majority of cases of diabetic nephropathy occur in type 2 diabetes

Front 29

How can one decrease the risk of diabetic nephropathy and slow the progression of it once it begins?

Back 29

1. *Properly controlled hyperglycemia
2. Control of blood pressure

Front 30

What is the number 1 cause of end-stage renal disease?

Back 30

Diabetes

Front 31

Once diabetics develop diabetic nephropathy, what sort of control is critical in delaying progression to end-stage renal disease?

Back 31

Control of blood pressure
(Goal < 130/80 mmHg)

Front 32

What are the clinical stages of diabetic nephropathy?

Back 32

1. Early
2. Incipient
3. Overt
4. Late
5. ESRD

Front 33

What is a sign of early diabetic nephropathy?

Back 33

Increased GFR

Front 34

What are the signs of "incipient" diabetic nephropathy?

Back 34

1. Microalbumemia
2. Increased GFR

*usually occurs after a few years)

Front 35

What are the signs of "overt" diabetic nephropathy?

Back 35

1. Albuminuria
2. Declining GFR

Front 36

What are the signs of "late" diabetic nephropathy?

Back 36

1. Albuminuria or nephrotic syndrome
2. Increased BUN and serum creatinine
3. Hypertension

Front 37

In which stage of diabetic nephropathy does microalbuminuria occur?

Back 37

"Incipient" stage

Front 38

In which stage of diabetic nephropathy does hypertention occur?

Back 38

"Late" stage

Front 39

In which stage of diabetic nephropathy do BUN and creatinine increase?

Back 39

"Late" stage

Front 40

Are microalbuminemia and overt proteinuria detectable by a routine urine dipstick?

Back 40

Microalbuminemia is NOT dectectable by a routine dipstick, but over proteinuria is

Front 41

What is the earliest histologic abnormality of diabetic nephropathy?

Back 41

Enlarged size of glomeruli

Front 42

What is the first routinely recognizable abnormality seen in diabetic nephropathy?

Back 42

Diffuse thickening of the glomerular capillary basement membranes

(There is also expansion of the mesangial area by deposition of extracellular mesangial matrix material)

Front 43

List some overt microscopic changes that occur in diabetic nephropathy

Back 43

1. Glomerulosclerosis
2. Tubular atrophy
3. Interstitial fibrosis
4. Vascular changes.

Front 44

List 3 signs of end-stage renal disease due to diabetic nephropathy

Back 44

1. Global glomerulosclerosis
2. Interstitial fibrosis
3. Tubular atrophy

Front 45

What is the most characteristic change of diabetic nephropathy?

Back 45

Nodular diabetic glomerulosclerosis
(Kimmelstiel-Wilson nodules)

Front 46

List 4 characteristic pathologic changes seen in diabetic nephropathy

Back 46

1. Nodular diabetic glomerulosclerosis (Kimmelstiel-Wilson nodules)
2. Hyaline arteriolosclerosis (hyaline deposits in glomeruli --> "capsular drops")
3. Linear GBM and TBM staining for IgG and albumin on IF

Front 47

List 4 diseases which cause renal failure and enlargement of the kidneys

Back 47

1. Diabetic nephropathy
2. Polycystic kidney disease
3. Myeloma kidney
4. Amyloidosis

Front 48

List 2 characteristic EM findings in diabetic nephropathy.

Back 48

1. Marked thickening of the GBM
2. Expansion of the mesangium

Front 49

List 3 vascular lesions seen in diabetic nephropathy

Back 49

1. Hyaline arteriolosclerosis
2. Fibrin cap
3. Capsular drop

*Leakage of plasma protein beneath damaged endothelium of vessels

Front 50

Seeing prominent hyaline deposits in which regions of the kidney is virtually pathognomonic for diabetic nephropathy?

Back 50

BOTH the afferent and efferent arterioles

Front 51

What is a "capsular drop"?

Back 51

Hyaline deposit in Bowman's capsule basement membrane.
*Considered virtually diagnostic for diabetic nephropathy

Front 52

Liniear staining of glomerular and tubular basement membranes is characteristic for what disease?

What are these deposits?

Back 52

Diabetic nephropathy

*Plasma proteins adhered to damaged basement membrane (NOT immune complexes)

Front 53

About 90% of diabetics with nephropathy also have which condition?

Back 53

Retinopathy

*Renal-retinopathy syndrome

Front 54

List 3 complications of diabetic nephropathy

Back 54

1. Nephrosclerosis
2. Pyelonephritis
3. Papillary necrosis

Front 55

What is the probable cause of papillary necrosis in diabetic nephropathy?

Back 55

Ischemia in the medulla

Front 56

List 3 characteristics of diabetic microangiopathy

Back 56

1. Microaneurysm formation
2. Thick basement membranes
3. "Leaky" vessels and basement membranes

Front 57

Describe the basic pathogenesis of diabetic nephropathy

Back 57

1. Hyperglycemia
2. Glycosylation of various proteins (advanced glycation end-products --> oxidative damage)
3. Genetic predisposition
4. Conversion of glucose to sorbitol by aldolase reductase
5. Hemodynamic alterations (caused by HTN, hyaline arteriolosclerosis)

Front 58

List 4 treatments for diabetic nephropathy

Back 58

1. Control of BP (ACEI, ARBs)
2. Control of blood sugar
3. Dietary protein restriction
4. Renal/pancreas allograft transplantation