Rheumatologic Pleuropulmonary Disease

Question 0

Scleroderma lung mediators?

Answer 0

Early: IL-8 and TNF

MIF1alpha, RANTES, and endothelin-1

Late: TGF-beta

Question 1

Two forms of systemic sclerosis lung disease?

Answer 1

Interstitial lung disease, and vascular disease

Question 2

Tell me about IL-8

Answer 2

Early responder to scleroderma lung. A neutrophil attractant and activator.

Question 3

Talk about TNF

Answer 3

Early scleroderma lung mediator. Neutrophil attractant and promoter of neutrophil-endothelial binding

Question 4

What is MIF1alpha?

Answer 4

Scleroderma lung mediator. Promoter of neutrophil chemotaxis

Question 5

What is RANTES?

Answer 5

“regulated on activation normal Tcell expressed and secreted”

Scleroderma lung mediator

Question 6

What does endothelin 1 do?

Answer 6

Vasoconstrictor- more important in vascular disease, but still a mediator in scleroderma lung

Question 7

What is TGFbeta’s role in scleroderma lung?

Answer 7

A late mediator- promotes fibroplasia

Question 8

What’s a typical CT of scleroderma lung?

Answer 8

Favors bases, posterior and periphery. Looks like ground glass first and reticular later.

Question 9

How to distinguish NSIP pattern vs UIP pattern in scleroderma lung?

Answer 9

NSIP has temporal homegeneity and responds better to treatment. NSIP has ground glass appearance, while UIP has honeycomb appearance

Question 10

How to treat sclerosis lung disease?

Answer 10

Cyclophosphamide NOT steroids