Primary Gn

Question 1

What is the autoimmune disease characterized by circulating IgG autoantibodies against two major podocyte antigens?

Answer 1

PRIMARY MEMBRANOUS NEPHROPATHY (PMN)

Question 2

What are the 2 major podocyte antigens in Primary MN?

Answer 2

1. PLA2R1 (70%)
2. THSDA (5-10%)

Question 3

What are the 2 new classification for MPGN

Answer 3

1. IMMUNE COMPLEX-MEDIATED
2. COMPLEMENT- MEDIATED OR C3 GLOMERULOPATHY

Question 4

New classification of MPGN is based on:

Answer 4

IF

Question 5

What type of MPGN, seen in IF where both immunoglobulin and complement are deposited

Answer 5

Immune complex-mediated MPGN

Question 6

What type of MPGN with predominant C3 staining by IF?

Answer 6

Complement-mediated MPGN or C3 glomerulopathy

Question 7

An autoimmune disease which results from autoantibodies targeting DNAJB9 (a putative autoantigen)

Answer 7

FIBRILLARY GN

Question 8

Patients with immunotactoid glomerulonephritis should be evaluated for the underlying 3 disorders:

Answer 8

1. Monoclonal gammopathy
2. Malignancy
3. Lymphoproliferative disorders

Question 9

Autoantibodies in sporadic anti-GBM disease are directed against NC1 domain of what chain of type IV collagen?

Answer 9

ALPHA-3 CHAIN

Question 10

What antibody in ANCA-associated vasculitis, which when present lung involvement, or upper respiratory tract involvement are more likely to relapse?

Answer 10

ANTI-PROTEINASE-3 (PR3) ANTIBODIES

Question 11

Anti-GBM alloantibodies cause posttransplantation nephritis in X-linked Alport syndrome are directed against the NC1 domain of what chain of the type IV collagen?

Answer 11

ALPHA-5 CHAIN

Question 12

IgA vasculitis was previously called as?

Answer 12

HENOCH-SCHONLEIN PURPURA

Question 13

GPA (Granulomatosis with polyangiitis) was previously called?

Answer 13

WEGENER’S GRANULOMATOSIS

Question 14

EGPA (Eosinophilic granulomatosis with polyangiitis) was previously called as?

Answer 14

CHURG-STRAUSS SYNDROME

Question 15

What are the 4 glomerular disease with Nephrotic Features?

Answer 15

1. MCD
2. MN
3. FSGS
4. FIBRILLARY GN

Question 16

What glomerular disease manifest purely with Nephrotic features?

Answer 16

MCD

Question 17

Loss of charge selectivity is the cause of proteinuria in what type of GN?

Answer 17

MCD

Question 18

Loss of size selectivity is the cause of proteinuria in what type of GN

Answer 18

MN

Question 19

What is the strong evidence for glomerular bleeding?

Answer 19

ACANTHOCYTURIA

Question 20

What are the 4 differential diagnosis of glomerular hematuria without proteinuria, renal insufficiency, or red blood cell casts?

Answer 20

1. IgA NEPHROPATHY
2. TBMN
3. HEREDITARY NEPHRITIS
4. NORMAL GLOMERULI

Question 21

What is the protein excretion rate in Nephrotic syndrome?

Answer 21

> 3.5g/day

Question 22

Refers to the presence of lipid in the tubular epithelial cells and urine

Answer 22

“LIPOID NEPHROSIS”

Question 23

MCD was previously called as?

Answer 23

“LIPOID NEPHROSIS”

Question 24

What the the 6 factors that are increased in Nephrotic syndrome?

Answer 24

2, 5, 7, 8, 10, 13

Question 25

What are the 3 factors that are decreased in Nephrotic syndrome?

Answer 25

9, 11, 12

Question 26

The most consistent tubular lesion of increased protein & lipid resorption droplets in TECs is seen in what type of GN?

Answer 26

MCD

Question 27

What is the most frequent positive IF finding in MCD?

Answer 27

Low-level mesangial staining for IgM & C3

Question 28

What is the pathologic sine qua non of MCD in EM?

Answer 28

EFFACEMENT OF FOOT PROCESSES

Question 29

What are the LM, IF, EM findings in MCD?

Answer 29

LM: no glomerular lesions *focal segmental mesangial prominence (not >3-4cells) IF: no staining *low level staining for IgM,C3 EM: effacement of foot processes

Question 30

In MCD, there is abnormal regulation of this cell subset:

Answer 30

T-CELL SUBSET

Question 31

What NSAID has been associated with MCD?

Answer 31

FENOPROFEN

Question 32

What type of lymphoma is associated with MCD?

Answer 32

CLASSIC HODGKIN LYMPHOMA (NODULAR SCLEROSIS TYPE)

Question 33

What is dose of prednisone for the treatment of MCD?

Answer 33

1mg/kg Not to exceed 80mg/day

Question 34

What is considered a response to therapy in MCD?

Answer 34

No proteinuria by dipstick for at least 3 days

Question 35

In adult patients with MCD, a response to corticosteroid treatment may take up to how many weeks?

Answer 35

15 weeks

Question 36

In MCD, how long should treatment with corticosteroids continued after complete remission of proteinuria?

Answer 36

6 weeks

Question 37

What is the dose of oral cyclophosphamide in the treatment of MCD?

Answer 37

2mg/kg 8-12 weeks

Question 38

What is an alternative to cyclophosphamide therapy for relapsing or steroid-dependent MCD?

Answer 38

CYCLOSPORINE

Question 39

What is the duration of treatment with cyclosporine for relapsing/steroid-dependent MCD?

Answer 39

18-24 months

Question 40

What are the 2 types of CNI?

Answer 40

1. Tacrolimus 2. Cyclosporine

Question 41

B lymphocyte-depleting agent?

Answer 41

RITUXIMAB

Question 42

What is the usual treatment regimen for MCD?

Answer 42

1. Prednisone (induction of remission) Response: 15 weeks Continued after remission: 6 more weeks 2. Cyclophosphamide (maintenance) 8-12 weeks

Question 43

What is a differential diagnosis in steroid-resistant MCD?

Answer 43

FSGS

Question 44

What are the 2 treatment options for steroid-resistant MCD?

Answer 44

1. CYCLOSPORINE 2. RITUXIMAB

Question 45

What are the 10 glomerular disease that cause Nephrotic syndrome & GN?

Answer 45

1. MCD 2. FSGS 3. C1q NEPHROPATHY 4. MN 5. MPGN 6. C3 GLOMERULOPATHY 7. ACUTE PSGN 8. IgAN 9. FIBRILLARY GN 10. IMMUNOTACTOID GN