Diffuse Alveolar Hemorrhage

Question 1

2 circulations in lung

Answer 1

Bronchial (high pressure, low volume)
Pulmonary (low pressure, high capacitance)

Question 2

Bronchial circulation supplies __

Answer 2

Conducting airways (mainstem bronchi until terminal bronchioles)

Question 3

Bronchial circulation arises from:

Answer 3

Aorta

Question 4

Source of low-grade, chronic and diffuse alveolar hemorrhage

Answer 4

Pulmonary circulation

Question 5

Bleeding in cystic fibrosis is due to:

Answer 5

Bronchiectasis

Question 6

Infection presenting with hemoptysis

Answer 6

S. pneumoniae
S. aureus

Question 7

Timeline of hemosiderin-laden macrophages

Answer 7

3 days: first appear
7-10 days: peak
2 months: resolution

Question 8

Most common ANCA-associated vasculitis presenting with DAH from pulmonary capillaritis

Answer 8

MPA

Question 9

Vessels affected by ANCA vasculitis

Answer 9

Small and medium

Question 10

Necrotizing vasculitis of small- and medium-sized vessels with granulomatous inflammation

Answer 10

GPA

Question 11

Clinical features are chronic rhinitis, sinusitis, OM, nasal cartilage destruction (saddle nose), salivary gland swelling, SG stenosis, tracheobronchial ulceration, parenchymal nodules that may cavitate, DAH

Answer 11

GPA

Question 12

Antibodies in GPA

Answer 12

Anti-PR3 (c-ANCA pattern)

Question 13

Gold standard for diagnosis of GPA

Answer 13

Biopsy: vasculitis and capillaritis with necrotizing granulomata, paucity of immune complexes

Question 14

Antibodies in MPA

Answer 14

Anti-MPO (p-ANCA pattern)

Question 15

Biopsy in MPA

Answer 15

PC with neutrophilic infiltration of small arterioles, venues, and capillaries with fibrinoid necrosis

Question 16

Treatment of ANCA vasculitis

Answer 16

Induction with GC and cyclophosphamide for 3-6 months
Maintenance with prednisone, methotrexate, azathioprine for 1-2 years

Others:
Rituximab, IVIg, plasmapharesis

Question 17

Vasculitis limited to lung and kidney

Answer 17

Anti-GBM disease

Question 18

Diagnosis of anti-GBM

Answer 18

Anti-GBM antibodies in serum and basement membrane of lung and kidney

Question 19

Treatment of anti-GBM

Answer 19

CS
Cyclophosphamide
PLASMAPHERESIS in all cases

Question 20

PC with alveolar hemorrhage without renal or other systemic manifestations

Answer 20

IPC

Question 21

Differentiates IPC from IPH

Answer 21

Lower Hgb, higher ESR in IPC

Question 22

Treatment of IPC

Answer 22

Same as ANCA vasculitis

Question 23

Autosomal dominant pulmonary hemorrhage syndrome

Answer 23

COPA Syndrome

Question 24

Pathophysiology of COPA Syndrome

Answer 24

Dysfunctional autophagy
Th17 skewing and immune dysregulation
Chronic, relapsing and remitting alveolar hemorrhage

Question 25

CT findings of COPA

Answer 25

Cystic disease

Question 26

Biopsy of IPH

Answer 26

Bland alveolar hemorrhage, absence of inflammation

Question 27

Treatment of IPH

Answer 27

CS Steroid-sparing: Hydroxychloroquine Azathioprine Others: 6-mercaptopurine

Question 28

Cytologic finding in DAH

Answer 28

Hemosiderin-laden macrophages

Question 29

Management of massive hemoptysis

Answer 29

High PEEP Double lumen ET Bronchial artery embolization