ANTI-GBM GN

Question 1

What type of GN is characterized by circulating antibodies to the GBM (anti-GBM) and deposition of IgG along the GBM?

Answer 1

ANTI-GBM GN

Question 2

What are the 2 types of Anti-GBM disease?

Answer 2

1. RENAL-LIMITED DISEASE (Anti-GBM GN)
2. PULMONARY-RENAL VASCULITIC SYNDROME (Goodpasture Syndrome)

Question 3

What are the 2 peaks with respect to age in Anti-GBM disease?

Answer 3

1st peak: 2nd-3rd decade
2nd peak: 6th-7th decade

Question 4

What type of anti-GBM disease which has a higher frequency of pulmonary hemorrhage and usually occurs in the 2nd-3rd decade?

Answer 4

GOODPASTURE SYNDROME

Question 5

What type of anti-GBM disease which is common among women and usually occurs in the 6th-7th decade?

Answer 5

RENAL-LIMITED DISEASE (ANTI-GBM DISEASE)

Question 6

Genetic susceptibility to anti-GBM disease is associated with?

Answer 6

HLA-DR2

Question 7

The pathologic IF finding of linear staining of the GBMs predominantly for IgG by is indicative of?

Answer 7

ANTI-GBM GN

Question 8

What are the 2 special stains that outlines the basement membrane?

Answer 8

1. Jones Methenamine Silver
2. PAS stain

Question 9

Which type of GN has the highest percentage of crescent formation?

Answer 9

ANTI-GBM GN

Question 10

The antigen to which anti-GBM antibodies react is?

Answer 10

α3α4α5(IV) NC1

Question 11

What are the 2 phases of injury in Anti-GBM disease?

Answer 11

1. HETEROLOGOUS PHASE
2. AUTOLOGOUS PHASE

Question 12

Refers to the first 24 hours and is mediated by direct deposition of the heterologous antibodies on the GBM, with subsequent recruitment of neutrophils?

Answer 12

HETEROLOGOUS PHASE

Question 13

What is the phase of acute injury which depends on the host’s immune response to the heterologous immunoglobulin bound to the GBM?

Answer 13

AUTOLOGOUS PHASE

Question 14

What are 3 standard treatment used for anti-GBM disease?

Answer 14

1. PLASMAPHERESIS
2. CORTICOSTEROIDS
3. CYCLOPHOSPHAMIDE

Question 15

What is the GN characterized by an abrupt, acute glomerulonephritis, with severe oliguria or anuria and has high risk of progression to ESKD if appropriate therapy is not instituted immediately?

Answer 15

RENAL ANTI-GBM DISEASE

Question 16

What GN is characterized by the presence of pulmonary hemorrhage concurrent with glomerulonephritis?

Answer 16

GOODPASTURE SYNDROME

Question 17

What is essentially a risk factor for the development of anti-GBM antibodies?

Answer 17

Occupational exposure to petroleum-based mineral oils

Question 18

What is the diagnostic laboratory finding in anti-GBM disease?

Answer 18

circulating antibodies to α3 chain of type IV collagen of the GBM

Question 19

What is the process which consists of removal of 2 to 4 L of plasma and its replacement with a 5% albumin solution?

Answer 19

PLASMAPHERESIS

Question 20

What is the dose and duration of treatment of Prednisone for anti-GBM disease?

Answer 20

1st month: 1 mg/day
2nd-3rd months: alternate day therapy

Question 21

What is the dose and duration of treatment of IV methylprednisolone for anti-GBM disease?

Answer 21

• 7mg/kg/day
• 3 days

Question 22

What is the dose and duration of treatment of Cyclophosphamide for anti-GBM disease?

Answer 22

• 2mg/kg/day
• 8-12 weeks

Question 23

What is the major prognostic marker for the progression to ESKD in anti-GBM dse?

Answer 23

Serum creatinine level at the time of initiation of treatment

Question 24

What is the cut-off creatinine level for which immunosuppressive therapy is withheld?

Answer 24

> 7g/dl

Question 25

In what set of patients where Immunosuppressive therapy should not be withheld, even with serum creatinine levels > 7 mg/dL?

Answer 25

WITH BOTH CIRCULATING ANTI-GBM ANTIBODIES AND ANCA

Question 26

Concomitant presence of what was associated with a more favorable renal outcome in anti-GBM disease?

Answer 26

ANCA