Vascular Disease

Question 1

Induction therapy for GPA

Answer 1

Cyclophosphamide and prednisone (+/- PLEX)

Question 2

Maintenance therapy for GPA

Answer 2

Azathioprine or Rituxin or Cyclophosphamide

Question 3

Genetic mutation associated with pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis

Answer 3

EIF2AK4

Question 4

Genetic mutation associated with Birt-Hogg-Dube disease

Answer 4

Folliculin

Question 5

Genetic mutations (2) associated with HHT

Answer 5

ENG or ACVRL1

Question 6

Screening for pulmonary AVMs in HHT

Answer 6

First echo with bubble study. If positive get CTA.