FIBRILLARY GN & IMMUNOTACTOID GLOMERULOPATHY

Question 1

What is the atrangement of fibrils in fibrillary GN?

Answer 1

• NONBRANCHING
• RANDOMLY ARRANGED

Question 2

What is the diameter of fibrils in Fibrillary GN?

Answer 2

20 nm

Question 3

What is the arrangement of fibrils in immunotactoid glomerulopathy?

Answer 3

• GREATER ORGANIZATION
• MICROTUBULAR APPEARANCE

Question 4

What is the diameter of fibrils in Immunotactoid glomerulopathy?

Answer 4

30-40 nm

Question 5

FGN can mimic the light microscopic appearance of the ff 3 types of GN:

Answer 5

1. MPGN
2. PROLIFERATIVE GN
3. MN

Question 6

What is the appearance of fibril deposits in fibrillary GN when stained with a Jones silver methenamine stain?

Answer 6

MOTH-EATEN

Question 7

What are the 2 class/complement that stain dominantly in IF staining in Fibrillary GN?

Answer 7

1. IgG
2. C3

Question 8

In IF, the immune deposits in Fibrillary GN stain dominantly for what subclass of IgG?

Answer 8

IgG4

Question 9

What is the distinctive IF staining pattern in Fibrillary GN?

Answer 9

CAPILLARY WALLS - irregular band-like appearance
MESANGIUM - irregular shaggy appearance

Question 10

What are the 2 differences in patterns of deposits in FGN vs Immunotactoid glomerulopathy by EM?

Answer 10

FGN: no tubular structure
Randomly distributed

IMMUNOTACTOID:
Microtubular structure
Align in parallel rays

Question 11

IF staining in Immunotactoid is predominantly?

Answer 11

IgG

Question 12

What is autoimmune disease caused by autoantibodies targeting putative autoantigen DNAJB?

Answer 12

FIBRILLARY GN

Question 13

What type of GN is more likely to have an associated hematopoietic process and poor long-term survival?

Answer 13

IMMUNOTACTOID GN

Question 14

What type of GN usually have underlying lymphoproliferative disease?

Answer 14

IMMUNOTACTOID

Question 15

What are the 2 drugs which has a role in treatment of Fibrillary GN in studies?

Answer 15

1. RITUXIMAB
2. CHLORAMBUCIL