MN Flashcards

(46 cards)

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:

23
Q

What is the predominant type of IgG autoantibody against PLA2R?

24
Q

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

A
  1. CELL BODY
  2. FOOT PROCESSES
25
THSD7A is expressed at which part of the podocyte?
BASAL SURFACE
26
THSD7A-associated membranous nephropathy affects which gender more?
FEMALE
27
What autoantigen in MN is associated with malignancy?
THSDA7
28
What are the 2 results of massive nephrosis?
1. BILATERAL RENAL VEIN THROMBOSIS 2. HYPOVOLEMIA
29
What are the 4 causes that may cause a sudden deterioration of kidney function?
1. CRESCENTIC GN 2. ACUTE BILATERAL RENAL VEIN THROMBOSIS 3. HYPOVOLEMIA 4. DRUG-INDUCED AKI
30
Renal Vein thrombosis is associated with this type of GN?
MN
31
What are the 3 clinical manifestations of renal vein thrombosis?
1. MACROSCOPIC HEMATURIA 2. FLANK PAIN 3. DETERIORATION IN KIDNEY FUNCTION
32
What is the gold standard in the diagnosis of renal vein Thrombosis?
VENOGRAPHY WITH CONTRAST
33
What is the best predictor of spontaneous remission in MN?
DECREASE IN PROTEINURIA OF >50% IN THE 1ST YEAR
34
What are the 4 patient risk factors associated with progressive decline in kidney function in MN?
1. MALE 2. >50 YEARS 3. UNCONTROLLED HPN 4. REDUCED GFR AT PRESENTATION
35
What is one of the strongest indicators of progressive disease in MN?
PERSISTENCE OF MODERATE PROTEINURIA
36
What is the hallmark laboratory finding in MN?
PROTEINURIA
37
At what albumin level is the risk for venous thromboembolic events higher?
< 2.5g/dl
38
What anticoagulant could be given for severe nephrotic syndrome with with albumin <2g/dl in MN to prevent renal venous thrombosis?
WARFARIN
39
What are the 9 medications used in the treatment of MN?
1. CORTICOSTEROIDS 2. CYCLOPHOSPHAMIDE 3. CHLORAMBUCIL 4. CNI (Tacrolimus, Cyclosporine) 5. ACTH 6. MMF 7. RITUXIMAB 8. AZATHIOPRINE 9. ECULIZUMAB
40
What are the 2 alkylating agents?
1. CYCLOSPORINE 2. CYCLOPHOSPHAMIDE
41
What do you call the combination of corticosteroids and cytotoxic drugs (cyclophosphamide & chlorambucil) used to treat MN?
PONTECELLI PROTOCOL
42
What is the PONTECELLI PROTOCOL?
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
MN patients with what risk classification is considered for immunosuppressive therapy?
MODERATE TO HIGH RISK
44
What risk classification is characterized by persistent proteinuria between 4-6 g protein/day, despite RAAS blockade and normal kidney function?
MODERATE RISK
45
What risk classificafion is characterized by persistent proteinuria of > 8 g of protein/day, with or without renal insufficiency
HIGH RISK
46
Where is the location of immune deposits in MN?
SUBEPITHELIAL