PAUCI-IMMUNE GN

Question 1

What is the characteristic feature of the glomerular lesion in Pauci-immune crescentic GN?

Answer 1

Focal necrotizing and crescentic glomerulonephritis, with little or no glomerular staining for immunoglobulins by IF microscopy

Question 2

What are the 2 types of Pauci-immune crescentic GN?

Answer 2

1. Renal limited (Primary)
2. Part of systemic small vessel vasculitis

Question 3

GPA is previously known as?

Answer 3

WEGENER’S GRANULOMATOSIS

Question 4

EGPA is previously known as?

Answer 4

CHURG-STRAUSS SYNDROME

Question 5

What is the distinguishing pathologic difference between pauci-immune crescentic glomerulonephritis and anti-GBM and immune complex crescentic glomerulonephritis?

Answer 5

absence or paucity of glomerular staining for immunoglobulins

Question 6

What is the major pathogenic factor in PAUCI-IMMUNE CRESCENTIC GN?

Answer 6

ANCA IgG

Question 7

What are the 2 antibodies which has a pathogenic role in Pauci-immune GN?

Answer 7

1. Anti-MPO
2. PR3

Question 8

What is the most common cause of RPGN in adults?

Answer 8

Renal limited and vasculitis-associated pauci-immune GN

Question 9

What are the 2 patterns caused by ANCA in IF?

Answer 9

1. P-ANCA (Perinuclear)
2. C-ANCA (Cytoplasmic)

Question 10

What are the 2 major antigen specificities for ANCA?

Answer 10

1. MPO
2. PR3

Question 11

What is the PPV of a positive ANCA result in a patient with classic features of RPGN?

Answer 11

95%

Question 12

In patients with hematuria and proteinuria, the PPV of a positive ANCA result is?

Answer 12

84%

Question 13

The treatment of pauciimmune crescentic glomerulonephritis (with or without systemic vasculitis) is still based primarily on these 2 drugs:

Answer 13

1. CORTICOSTEROIDS
2. CYCLOPHOSPHAMIDE

Question 14

What is the dose of methylprednisolone for induction therapy in pauciimune GN?

Answer 14

• 7mg/kg/d
• 3 consecutive days

Question 15

What is the dose of prednisone for the induction therapy in pauciimune GN?

Answer 15

• 1mg/kg/day (1st month)
• alternate day (2nd-3rd month)
• discontinued (4th-5th month)

Question 16

What is the dose of IV Cyclophosphamine for induction therapy in pauciimune GN?

Answer 16

• 0.5mg/kg/day (starting dose)
• then adjusted upward to 1mg/kg/d based on the 2-week leukocyte cpunt nadir

Question 17

What is the dose of oral Cyclophosphamine for induction therapy in pauciimune GN?

Answer 17

• 2mg/kg/day (starting dose)
• then adjusted downward as need to maintain nadir leukocyte >3000

Question 18

What are the 2 drugs used in the induction therapy for pauciimune GN?

Answer 18

1. CORTICOSTEROIDS
2. CYCLOPHOSPHAMIDE

Question 19

What are the 3 drugs used in the maintenance therapy for pauciimune GN?

Answer 19

1. AZATHIOPRINE
2. RITUXIMAB
3. METHOTREXATE

Question 20

What drug should be avoided with allopurinol?

Answer 20

AZATHIOPRINE

Question 21

What drug may lead to HBV reactivation?

Answer 21

RITUXIMAB

Question 22

What are the 3 trials regarding the induction therapy in Pauciimune GN?

Answer 22

1. CYCLOPS
2. MEPEX
3. RITUXVAS

Question 23

What are the 5 trials regarding maintenance therapy for pauciimune GN?

Answer 23

1. CYCAZAREM
2. WEGENT
3. IMPROVE
4. MAINRITSAN
5. REMAIN