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Ch 32 Secondary GN > GPA (Granulomatosis With Polyangiitis) > Flashcards

GPA (Granulomatosis With Polyangiitis) Flashcards

1
Q

What is the triad of GPA?

A
  1. VASCULITIS
  2. GRANULOMAS (necrotizing, granulomatous inflammation of upper/lower repiratory tracts)
  3. GN
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2
Q

What is the serologic marker of GPA?

A

ANCA

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3
Q

GPA Is more common in what gender?

A

MALE

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4
Q

What is the peak incidence of GPA?

A

4th-6th decade

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5
Q

What is the most common form of crescentic glomerulonephritis in older adults?

A

PAUCI-IMMUNE RPGN
(including GPA and MPA)

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6
Q

What is the classic histopathologic finding in GPA?

A

FOCAL SEGMENTAL NECROTIZING & CRESCENTIC GN

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7
Q

True vasculitis in GPA involves what size of renal arteries?

A

SMALL & MEDIUM-SIZED

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8
Q

What 3 types of vessels is affected in true vasculitis in GPA?

A
  1. RENAL ARTERIES (small &medium-sized)
  2. VEINS
  3. CAPILLARIES
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9
Q

C-ANCA is directed against?

A

PR3

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10
Q

P-ANCA is directed against?

A

MPO

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11
Q

What is the specificity of C-ANCA for GPA?

A

98-99%

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12
Q

What 3 medications have been associated with ANCA, usually anti-MPO, and at very high titers?

A
  1. PTU
  2. HYDRALAZINE
  3. MINOCYCLINE
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13
Q

What drug is associated with unique ANCA anti-MPO and anti-PR3 positivity, often with high titers of ANCA in association with positive ANAs, APL antibody titer, and other serologic tests?

A

LEVIMASOLE

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14
Q

A rise in this titer correlated with relapse in the patients with renal disease but only weakly in those without kidney disease

A

ANCA

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15
Q

a rise in this titer is associated with severe relapses, especially in those with renal disease and pulmonary hemorrhage

A

PR3 ANCA

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16
Q

What renal manifestation is yncommon in GPA?

A

NEPHROTIC SYNDROME

17
Q

What is the treatment regimen for GPA?

A

CYCLOPHOSPHAMIDE + CORTICOSTEROIDS

18
Q

What is the dose of cyclophosphamide int he treatment of GPA?

19
Q

What is the typical regimen for induction therapy for severe GPA or MPA RPGN?

A
  1. IV pulse methylprednisolone (7 mg/kg, to a maximum dose of 500–1000 mg) x 3 consecutive
  2. Then oral prednisone 1 mg/kg/day (to a maximum of 60–80 mg/day) for the 1st month, with subsequent tapering of the dose
    PLUS: IV/oral cyclophosphamide x 6 months
20
Q

What are the 4 severe complications associated with cyclophosphamide?

A
  1. INFECTIONS
  2. INFERTILITY
  3. HEMORRHAGIC CYSTITIS
  4. MALIGNANCY
21
Q

What are the 3 medications that can be used for maintenance therapy for GPA?

A
  1. AZATHIOPRINE
  2. MMF
  3. METGOTREXATE

Ch 32 Secondary GN (35 decks)

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