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Ch 31 Primary GN > MN > Flashcards

MN Flashcards

1
Q

Primary MN is caused by autoantibodies specific for?

A

PLAR2

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2
Q

What are the 4 causes of secondary MN?

A
  1. AUTOIMMUNE DISEASES (SLE, autoimmune thyroiditis)
  2. INFECTIONS (Hep B,C, malaria)
  3. DRUGS (gold, penicillamine)
  4. MALIGNANCIES (colon/lung CA)
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3
Q

What are the 2 causes of secondary MN that are more common in children?

A
  1. SLE
  2. Hep B
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4
Q

What is the most common cause of secondary MN in adults?

A

MALIGNANCY

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5
Q

What is the pathologic sine qua non of MN?

A

SUBEPITHELIAL IMMUNE COMPLEX DEPOSITS

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6
Q

What imaging provides the most definitive diagnosis of MN?

A

EM

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7
Q

How many ultrastructural stages does MN have?

A

4 STAGES (STAGE 1–>4)

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8
Q

What stage of MN is characterized by the presence of scattered or more regularly distributed small immune complex–type, electron-dense deposits in the subepithelial zone between the basement membrane and the podocyte?

A

Stage 1

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9
Q

What stage of MN is characterized by projections of basement membrane material (spikes) around the subepithelial deposits?

A

Stage 2

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10
Q

What stage of MN where the new basement membrane material surrounds the deposits?

A

Stage 3

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11
Q

What stage of MN where deposits are intramembranous?

A

Stage 3

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12
Q

What stage of MN is characterized by loss of the electron density of the deposits, which often results in irregular electron-lucent zones within an irregularly thickened basement membrane

A

Stage 4

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13
Q

Mesangial deposits are rare in which type of MN? (Primary or Secondary)

A

PRIMARY MN

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14
Q

What type of MN is caused by subepithelial in situ immune complex formation with antibodies from the circulation complexing with antigens derived from the podocyte?

A

PRIMARY MN

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15
Q

What type of MN is characterized by presence of mesangial dense deposits?

A

SECONDARY MN

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16
Q

What is the most common Ig seen in IF in MN?

A

IgG

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17
Q

What IgG subclass is most prominent in the capillary wall deposits of primary MN?

A

IgG4

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18
Q

Tubular basement membrane staining for Ig in IF is rare in whicht type of MN? (Primary or Secondary)

A

Primary

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19
Q

What are the findings in LM,IF,EM in primary MN?

A

LM: diffuse global capillary thickening with no glomerular hypercellularity

IF: diffuse global granular capillary wall staining for Ig (*IgG/IgG4) & complement

EM: subepithelial immune complex deposits

20
Q

What special stain is used that reveal the basement membrane changes induced by the subepithelial immune deposits in Primary MN?

A

JSMS (JONES SILVER METHENAMINE STAIN)

21
Q

What special stain accentuate basement membrane material?

A

JSMS (Jones Silver Methenamine Stain)

22
Q

Patients with primary membranous nephropathy may have an autoantibody targeting either of these 2 autoantigens but rarely against both autoantigens:

A
  1. PLA2R
  2. THSD7A
23
Q

What is the predominant type of IgG autoantibody against PLA2R?

A

IgG4

24
Q

PLA2R is expressed on which parts (2) of the podocyte?

A
  1. CELL BODY
  2. FOOT PROCESSES
25
Q

THSD7A is expressed at which part of the podocyte?

A

BASAL SURFACE

26
Q

THSD7A-associated membranous nephropathy affects which gender more?

A

FEMALE

27
Q

What autoantigen in MN is associated with malignancy?

A

THSDA7

28
Q

What are the 2 results of massive nephrosis?

A
  1. BILATERAL RENAL VEIN THROMBOSIS
  2. HYPOVOLEMIA
29
Q

What are the 4 causes that may cause a sudden deterioration of kidney function?

A
  1. CRESCENTIC GN
  2. ACUTE BILATERAL RENAL VEIN THROMBOSIS
  3. HYPOVOLEMIA
  4. DRUG-INDUCED AKI
30
Q

Renal Vein thrombosis is associated with this type of GN?

A

MN

31
Q

What are the 3 clinical manifestations of renal vein thrombosis?

A
  1. MACROSCOPIC HEMATURIA
  2. FLANK PAIN
  3. DETERIORATION IN KIDNEY FUNCTION
32
Q

What is the gold standard in the diagnosis of renal vein Thrombosis?

A

VENOGRAPHY WITH CONTRAST

33
Q

What is the best predictor of spontaneous remission in MN?

A

DECREASE IN PROTEINURIA OF >50% IN THE 1ST YEAR

34
Q

What are the 4 patient risk factors associated with progressive decline in kidney function in MN?

A
  1. MALE
  2. > 50 YEARS
  3. UNCONTROLLED HPN
  4. REDUCED GFR AT PRESENTATION
35
Q

What is one of the strongest indicators of progressive disease in MN?

A

PERSISTENCE OF MODERATE PROTEINURIA

36
Q

What is the hallmark laboratory finding in MN?

A

PROTEINURIA

37
Q

At what albumin level is the risk for venous thromboembolic events higher?

A

< 2.5g/dl

38
Q

What anticoagulant could be given for severe nephrotic syndrome with with albumin <2g/dl in MN to prevent renal venous thrombosis?

A

WARFARIN

39
Q

What are the 9 medications used in the treatment of MN?

A
  1. CORTICOSTEROIDS
  2. CYCLOPHOSPHAMIDE
  3. CHLORAMBUCIL
  4. CNI (Tacrolimus, Cyclosporine)
  5. ACTH
  6. MMF
  7. RITUXIMAB
  8. AZATHIOPRINE
  9. ECULIZUMAB
40
Q

What are the 2 alkylating agents?

A
  1. CYCLOSPORINE
  2. CYCLOPHOSPHAMIDE
41
Q

What do you call the combination of corticosteroids and cytotoxic drugs (cyclophosphamide & chlorambucil) used to treat MN?

A

PONTECELLI PROTOCOL

42
Q

What is the PONTECELLI PROTOCOL?

A

Step 1:
IV Methylprednisolone: 1g/day
1st 3 days of the month

Step 2: (a+b)
a. oral Methylpred (0.4mg/kg/day)
Prednisone (0.5mg/kg/day)
> Given on alternating monthly
schedule
➕️➕️➕️
b. Chlorambucil (0.2mg/kg/day)

43
Q

MN patients with what risk classification is considered for immunosuppressive therapy?

A

MODERATE TO HIGH RISK

44
Q

What risk classification is characterized by persistent proteinuria between 4-6 g protein/day, despite RAAS blockade and normal kidney function?

A

MODERATE RISK

45
Q

What risk classificafion is characterized by persistent proteinuria of > 8 g of protein/day, with or without renal insufficiency

A

HIGH RISK

46
Q

Where is the location of immune deposits in MN?

A

SUBEPITHELIAL

Ch 31 Primary GN (10 decks)

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