Vasculitis and Alveolar Hemorrhage

Question 1

Typical Presentation of Takayasu Arteritis for pulmonary Patient?

Answer 1

• Young Females, Pulmonary Artery Aneurysm, Pulmonary artery Stenosis, Pulmonary Infarct

Question 2

Polyarteritis Nodosa Involvement in lung?

Answer 2

Typically does not involve lungs. 3 NO’s (No ANCA, no glomerulonephritis, No Pulmonary capillaritis) . Rare Case reports of Bronchial Artery involvement.

Question 3

What Disease process do you think about with Pulmonary Artery Aneurysm from Vasculitis?

Answer 3

Behcets Disease

Question 4

Induction Agents for SEVERE GPA/MPA?

Answer 4

Rituxan or Cyclophosphamide

Question 5

Maintenance therapies for SEVERE GPA/MPA?

Answer 5

1. Rituxan
2. Methotrexate or Azathiopurine
3. Mycophenolate Mofetil or Leflunomide

Question 6

Induction Agents for NON-severe GPA

Answer 6

Glucocorticoids + Methotrexate (First Line)

2nd Line Options are Glucocorticoids alone or +
- Rituxan
- Cyclophosphamide
- Azathiopurine
- Mycophenolate Mofetil

Question 7

What did the PEXIVAS Trial Show?

Answer 7

No difference in outcomes leading to DEATH or ESRD in treating GPA patients with Plasma exchange or reduced dose Steroids alone regimen.

Question 8

1. What Risk do you have with Plex (Plasmapheresis).
2. When Do you consider PLEX for treatment option of a Vasculitis?

Answer 8

1. Increased Risk of Infection
2. Consider with Patient being ANCA + And Anti-GBM, High-risk ERSD, Salvage/rescue therapy

Question 9

Avacopan is a maintenance drug to prevent remission of Vasculitis, What is its mechanism of action?

Answer 9

C5a receptor Inhibitor

Question 10

Damage to what tissue leads to Diffuse Alveolar Hemorrhage?

Answer 10

Alveolar Epithelium, Extracellular Matrix and Capillary Endothelium

Question 11

> 20% Hemosiderin-Laden macrophages indicates what?

Answer 11

Diffuse Alveolar Macrophages.

Takes 6-8 weeks to go away.