IPF - Connective Tissue

Question 1

What treatment has been shown in prospective trials to improve lung function in patients with scleroderma-associated interstitial lung disease

Answer 1

Cytoxan, MMF (Mycophenolate mofetil)

Question 2

Which connective tissue diseases is rarely associated with ILD

Answer 2

lupus

Question 3

Why do patients with connective tissue disease-associated-ILD have a better survival than those with idiopathic interstitial pneumonia?

Answer 3

Higher incidence of non-specific interstitial pneumonia and to a lesser extent UIP has better prognosis if associated with CTD (RA is the exception)

Question 4

Treatment for idiopathic IPF-associated pulmonary HTN?

Answer 4

O2 (NOT meds)

Pirfenidone will tx fibrosis, but not pulm HTN and pulm HTN meds can make oxygenation worse

Question 5

When screening a scleroderma pt for pulm HTN, what measures do you get

Answer 5

FVC/DLCO and NT-proBNP

Question 6

Pt with scleroderma associated pulm HTN should generally be rx’d with

Answer 6

pulm HTN meds

Question 7

Vasodilator testing during RHC predicts

Answer 7

response to Ca channel blockers, not reliable for predicting (non)response to other pulm htn meds

Question 8

lymphangioleiomyomatosis tx

Answer 8

spirolimus

avoid estrogens

Question 9

pattern of cysts in LAM?

Answer 9

random, uniform

Question 10

cystic lung disease associated with Sjogren’s syndrome

Answer 10

LIP - lymphocytic interstitial pneumonia (peribronchovascular, lower zone)

Question 11

Birt-Hogg-Dube pattern of cysts and tx

Answer 11

medial and lower zone; screen for renal ca