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Ch 31 Primary GN > MPGN & C3 GLOMERULOPATHY > Flashcards

MPGN & C3 GLOMERULOPATHY Flashcards

1
Q

The new classification of MN is based on what findings on IF?

A

IMMUNE DEPOSIT STAINING

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2
Q

What are the 2 types of MPGN based on the new classification?

A
  1. IMMUNE COMPLEX-MEDIATED
  2. COMPLEMENT-MEDIATED OR
    C3 GLOMERULOPATHY
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3
Q

What type of MPGN is characterized by IF staining for both Ig & complement?

A

IMMUNE COMPLEX-MEDIATED MPGN

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4
Q

What type of MPGN is characterized by IF staining predominantly for C3?

A

COMPLEMENT-MEDIATED OR C3 GLOMERULOPATHY

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5
Q

What are the 3 deposits that would result from activation of the classical complement pathway?

A

C1q, C3, C4

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6
Q

Immune complex glomerular deposits results in the activation of what pathway?

A

CLASSICAL COMPLEMENT PATHWAY

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7
Q

What type of MPGN with histologic features of diffuse global capillary wall thickening, an increased mesangial matrix, and mesangial and endocapillary hypercellularity?

A

TYPE I MPGN

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8
Q

What is the earlier name for Type I MPGN?

A

LOBULAR GLOMERULONEPHRITIS

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9
Q

What type of MPGN that has a distinctive feature of doubling or more complex replication of GBMs?

A

TYPE I MPGN

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10
Q

What are the 2 stains that highlight basement membranes?

A
  1. Jones silver methenamine stain
  2. Periodic acid–Schiff stain
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11
Q

Where is the location of immune complex deposits in Type 1 MPGN?

A

SUBENDOTHELIAL

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12
Q

What are the 2 causes of MPGN which has hyaline thrombi within the capillary lumens?

A
  1. CRYOGLOBINEMIA
  2. LUPUS
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13
Q

Dense deposit disease is previously called as?

A

TYPE 2 MPGN

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14
Q

C3 glomerulopathy is divided into 2 classification which are?

A
  1. DDD
  2. C3 GN
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15
Q

What is the characteristic pattern of IF staining in Type 1 MPGN?

A

PERIPHERAL GRANULAR TO BAND-LIKE STAINING FOR COMPLEMENT (especially C3)

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16
Q

Mesangial interposition into an expanded subendothelial zone that contains electron-dense immune deposits is the ultrastructural hallmark in EM of what type of MPGN?

A

TYPE 1 MPGN

17
Q

Type 1 MPGN is also refered to as?

A

Mesangiocapillary GN

18
Q

What is the difference between the levels of serum C3 in MPGN vs PSGN?

A

MPGN - C3 persistently depressed
PSGN - C3 return to normal within 2 months

19
Q

What are the 4 secondary causes of MPGN?

A
  1. INFECTION
  2. RHEUMATOLOGIC DISORDERS
  3. MALIGNANCY
  4. INHERITED
20
Q

What type of MPGN is characterized by deposits of electron-dense material in the subendothelial zones of glomeruli?

A

TYPE I MPGN

21
Q

What are the 3 features suggestive of poor prognosis in Type 1 MPGN?

A
  1. HYPERTENSION
  2. IMPAIRED GFR
  3. CELLULAR CRESCENTS
22
Q

The treatment of Type 1 MPGN is based on?

A

UNDERLYING DISEASE

23
Q

What are the 5 classes of drugs used in the treatment of Type 1 MPGN?

A
  1. PREDNISONE
  2. ANTIPLATELETS (ASA, DIPYRIDAMOLE)
  3. ANTICOAGULANT (WARFARIN)
  4. ALKYLATING AGENT (CYCLOPHOSPHAMIDE)
  5. CNI (MMF, CYCLOSPORINE)
24
Q

What is the type of MPGN that has a pathognomonic feature of discontinuous electron-dense bands in the GBM?

A

DDD

25
Q

IF finding of Intense capillary wall linear to bandlike staining for C3 is seen in what type of MPGN?

A

DDD

26
Q

What are the 2 types of C3 Glomerulopathies?

A
  1. DDD
  2. C3 GN
27
Q

What pathway is abnormally regulated in C3 glomerulopathy?

A

ALTERNATIVE COMPLEMENT PATHWAY

28
Q

What are the 3 mechanisms that result in overactivation of C3 convertase?

A
  1. DEVELOPMENT OF C3Nef (Autoantibody)
  2. ABSENCE OF CIRCULATING REGULATORS (Factor H)
  3. PRESENCE OF INHIBITOR OF FACTOR H
29
Q

What is the most common of the 3 patthogenic mechanism in DDD?

A

PRESENCE OF AUTOANTIBODY C3NeF

30
Q

What is the antibody that protects C3 convertase (C3bBb) from dissociation by factor H and thus prolongs its half-life by 10-fold?

A

C3NeF

31
Q

What complement is severely depressed in DDD?

A

C3

32
Q

Overactivity of C3Nef results in?

A

Perpetual breakdown of C3 –> severely depressed C3 in DDD

33
Q

Which type of MPGN has a worse prognosis?

A

DDD

34
Q

What are the 4 treatment used in C3 glomerulopathy?

A
  1. MMF
  2. RITUXIMAB
  3. PLASMA INFUSION
  4. ECULIZUMAB

Ch 31 Primary GN (10 decks)

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