Pulmonary Renal Syndromes

Question 1

Pulmonary-Renal Syndrome

Answer 1

development of diffuse alveolar hemorrhage and GN

Question 2

2 main categories of Pulmonary-Renal Syndrome

Answer 2

1. ANCA-associated Vasculitis (AAV)

2. Anti-GBM disease (Goodpasture)

Question 3

3 ANCA associated Vasculitides that have pulmonary-renal involvement

Answer 3

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

Question 4

GPA characteristics

Answer 4

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

Question 5

EGPA characteristics

Answer 5

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

Question 6

MPA characteristics

Answer 6

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

Question 7

Age group for presentation of most AAV

Answer 7

50-70 y/o

Question 8

Pathogenesis of an AAV

Answer 8

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

Question 9

Symptoms of AAV

Answer 9

Fever
Malaise
Myalgias
Migratory Arthralgias

Question 10

Which AAV is a saddle nose deformity seen in?

Answer 10

GPA (pr3)

Question 11

Renal symptoms of AAV

Answer 11

Hematuria, proteinuria, and RF

Question 12

Are purpura associated with AAV palpable or not?

Answer 12

Palpable.

Non-palpable purpura is seen more in thrombocytopenia

Question 13

Which AAV has a more acute presentation

Answer 13

GPA (PR3)

Question 14

What pattern of glomerular damage is seen in AAV?

Answer 14

Crescentic pattern RPGN

Question 15

Induction Treatment for AAV

Answer 15

High dose glucocorticoids + Rituximab
OR
glucocorticoids + Cyclophosphamide

Question 16

Maintenance Therapy for AAV

Answer 16

1: Azathioprine + Rituximab
2: Methotrexate

Question 17

Anti-GBM age group

Answer 17

Bimodal: 20’s and 60’s

Question 18

What part of the GBM does Goodpasture affect?

Answer 18

Lamina Densa a3 chain of Type IV collagen

Question 19

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

Answer 19

RPGN with crescent formation and Nephritic Syndrome

Question 20

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

Answer 20

Diffuse Linear

Question 21

Treatment for Anti-GBM

Answer 21

Plasmapheresis + Glucocorticoids + Cyclophosphamide