Pulmonary Vascular

Question 1

C-ANCA Associated with

Answer 1

(Cytoplasmic)

PR-3, and GPA

Question 2

P-ANCA Associated with

Answer 2

(perinuclear)

MPO, MPA and EGPA

Question 3

GPA Treatment: Upfront Limited Disease

vs Upfront Severe Disease

Answer 3

GCs + MTX (MTX noninferior to CYC by NORAM)

GCs+ CYC or rituximab
(RAVE RTX can replaced CYC, no change in remission at 6mos)
-RTX better in young, fertile patients
-if relapsing disease- RTX is superior

-consider PLEX in severe disease

Question 4

GPA Treatment: Remission Maintenance

• limited
• severe

Answer 4

• MTX or AZT (AZT cannot induce remission but equivalent for maintenance by CYCAZAREM)
• MTX or AZT if CYC for induction
• if RTX for induction, unclear if maintenance required

Question 5

Refractory GPA

Answer 5

• RTX if repeat induction (better than CYC by RAVE)

- PLEX if fulminant disease

Question 6

MPA vs GPA therapy

Answer 6

MPA therapy similar except RTX clearly preferred to CYC.

• RTX is clearly first line for severe disease induction and maintenance.
• Limited disease- GCS + MTX followed by MTX or AZT

Question 7

EGPA Treatment:
Induction
-Severe
-limited

Maintenance

Answer 7

• GCS + CYC or RTZ or anti-IL-5
• GCS +/- MTX/AZT

-MTX or (AZT or leflunomide after CYC)

Question 8

Diagnosis of DAH

Answer 8

BAL with progressively bloody return
OR
>20% hemosiderin-laden macrophages on BAL

Question 9

Conversion of Woods units to dynes.sec/cm5

Answer 9

Multiply by 80

Question 10

Positive vasodilator response in workup of PH

-who to treat

Answer 10

• change in mean PAP by 10mmHg to mean less than 40
• evidence for treatment with CCB based on IPAH. Not clear how to interpret in CTD or HIV-associated PAH
• tx with CCB includes NYHA functional class I-IV

Question 11

Common mutations in PAH

Answer 11

1. BMPR2 (10-40% of cases of IPAH); ~70% of heritable cases

2. ALK-1 (activin receptor-like kinase)

Question 12

RHC results to define patient with PAH

Answer 12

PAmean >= 25mmHg

PCWP 3

Question 13

Treatment (initial regimen)
NYHA class I 
II
III
IV

Answer 13

I- could consider single agent therapy vs combo upfront
II- combo oral therapy
III or IV with high risk features- IV therapy and oral PDE-5i, consider addition of ERA within a few weeks-months

• atrial septostomy
• pulmonary transplant

Question 14

High risk (>10% 1 year mortality)

Answer 14

1. Clinical fx of RV failure
2. WHO class IV
3. 6MWT <165m
4. CPET VO2 <11
5. BNP >300 or nt pro BNP >1400
6. RAP >14 (RA area >26cm2)
7. pericardial effusion
8. ScVO2 <60%

Question 15

Clues to suggest PVOD:

Answer 15

1. Imaging suggestive of pulmonary edema with normal PCWP
2. Evidence of pulmonary hemorrhage (hemosiderin laden macrophage on BAL)
3. Increase in pulmonary edema with initiation of PAH therapies

–Gold standard: open lung biopsy

Question 16

Leading Cause of Death in Sickle Cell Disease:

Answer 16

Acute chest syndrome

Question 17

Most common pathogens for pneumonia leading to acute chest syndrome:

Answer 17

Chlamydia pneumoniae, Mycoplasma pneumoniae, respiratory viruses

Question 18

Criteria for Severe Acute Chest:

-Treatment

Answer 18

PaO2 <60, pCO2 >50, SpO2 <85% on RA, segmental/lobar infiltrates in 3 or more lobes, require vent support

-Exchange transfusion to reduce Hb S to <30%

Question 19

HPS treatments:

Answer 19

1. Garlic and pentoxyfilline, modest improvement in PaO2
2. embolization if AVM/dilation is large
3. Liver transplant the only intervention known to improve survival

Question 20

Hereditary mutation for PVOD

Answer 20

EIF2AK4

Question 21

Takayasu can cause PH via:

Answer 21

Stenosis of pulmonary arteries

Question 22

PAN: No…

• can cause pulmonary hemorrhage via
• assoc

Answer 22

Never causes capillaritis (no DAH, no GN), no ANCA

• cause lung hemorrhage by bronchial arteritis
• assoc with Hep B/C