Pulmonary Renal Syndromes Flashcards Preview

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Flashcards in Pulmonary Renal Syndromes Deck (21)
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1
Q

Pulmonary-Renal Syndrome

A

development of diffuse alveolar hemorrhage and GN

2
Q

2 main categories of Pulmonary-Renal Syndrome

A
  1. ANCA-associated Vasculitis (AAV)

2. Anti-GBM disease (Goodpasture)

3
Q

3 ANCA associated Vasculitides that have pulmonary-renal involvement

A
  1. Granulomatosis with Polyangiitis (GPA aka The nazi)
  2. Eosinophilic Granulomatosis with Polyangiitis (EGPA aka Churg-Strauss)
  3. Microscopic Polyangiitis (MPA)
4
Q

GPA characteristics

A

Necrotizing vasculitis with granuloma formation with NO asthma or eosinophilia
PR3-Ab +
C-ANCA
Cytoplasmic pattern on IF

5
Q

EGPA characteristics

A

Necrotizing vasculitis with granulomas with asthma and eosinophilia
MPO Ab +
P-ANCA
Perinuclear pattern on IF

6
Q

MPA characteristics

A

Necrotizing vasculitis without granulomas.
MPO-Ab +
P-ANCA
Perinuclear pattern on IF

7
Q

Age group for presentation of most AAV

A

50-70 y/o

8
Q

Pathogenesis of an AAV

A

Environmental or infectious trigger primes MPO/pr3 + Neutrophils, MPO/pr3 moves to surface where Ab’s attach and cause inflammatory response

9
Q

Symptoms of AAV

A

Fever
Malaise
Myalgias
Migratory Arthralgias

10
Q

Which AAV is a saddle nose deformity seen in?

A

GPA (pr3)

11
Q

Renal symptoms of AAV

A

Hematuria, proteinuria, and RF

12
Q

Are purpura associated with AAV palpable or not?

A

Palpable.

Non-palpable purpura is seen more in thrombocytopenia

13
Q

Which AAV has a more acute presentation

A

GPA (PR3)

14
Q

What pattern of glomerular damage is seen in AAV?

A

Crescentic pattern RPGN

15
Q

Induction Treatment for AAV

A

High dose glucocorticoids + Rituximab
OR
glucocorticoids + Cyclophosphamide

16
Q

Maintenance Therapy for AAV

A

1: Azathioprine + Rituximab
2: Methotrexate

17
Q

Anti-GBM age group

A

Bimodal: 20’s and 60’s

18
Q

What part of the GBM does Goodpasture affect?

A

Lamina Densa a3 chain of Type IV collagen

19
Q

What type of Glomerular damage is seen in Anti-GBM?

A

RPGN with crescent formation and Nephritic Syndrome

20
Q

What type of pattern is seen on IF in Anti-GBM?

A

Diffuse Linear

21
Q

Treatment for Anti-GBM

A

Plasmapheresis + Glucocorticoids + Cyclophosphamide