Pulmonary-Restrictive Disease

Question 1

Ideopathic Pulmonary Fibrosis Patho

Answer 1

-characterized by repeated cell injury with abnormal repair of lung tissue leading to proliferation of fibrous connective tissue in lungs

Question 2

Approved Treatment for IPF

Answer 2

NONE

• many treatments have been tried, none effective
• prednisone is CONTRAINDICATED

Question 3

Potential Therapies for IPF

Answer 3

• N-acetylcysteine’s antioxidant effect to slow progression
• Pirfenione to block fibroblast formation. Not approved in US
• Lung transplant and clinical trials

Question 4

Sarcoidosis Patho

Answer 4

• Cause is unknown

- leads to immune response (lymphocytes and granulomas) affecting lung tissues

Question 5

When to Treat Sarcoidosis

Answer 5

• many pts do not need treatment
• those with progressive lung dx, cardiac or neuro involvement, ocular dx that doesn’t respond to drops, or hypercalcemia need treatment

Question 6

Pharmacological Measures for Sarcoidosis

Answer 6

• PO Prednisone is the mainstay. Taper dose to lowest effective for up to 6 months
• PO or IM Methotrexate is used to spare pt from steroids. SE include bone marrow suppresion (give folate), liver toxicity, and PF

Question 7

Pulmonary Hypertension Patho

Answer 7

• can be idiopathic, familiar, or r/t connective tissue dx, HIV, liver dx, congenital HD, Left Heart dx, hypoxia, or thromboembolism
• Treatment is ONLY indicated for those in the pulmonary arterial HTN (idiopathic, familiar, connective tissue, congenital HD, HIV, or liver dx)
• results from three pathways: endothelin, nictric oxide, and prostacyclin. Medications will act on these pathways

Question 8

Classes for Pulmonary HTN

Answer 8

Prostacyclin analogues
Endothelin Receptor Antagonists
PDE5 Inhibitors
Soluble Guanylate Cyclase Stimulator

Question 9

Prostacyclin Analogues in Pulmonary HTN

Answer 9

• MOA: increases cAMP causing vasodilation and reduced smooth muscle proliferation
• have very short HL, so are administered continuous IV, continuous SQ, inhaled, or PO
• acute withdrawal from continuous IV is LIFE THREATENING
• SE: hypotension, flushing, jaw pain, thrombocytopenia, and site specific (IV sepesis, injection site pain, or cough)
• all of these meds contain the term “prost”

Question 10

Endothelin Receptor Antagonist in Pulmonary HTN

Answer 10

• MOA: blocks the receptor for endothelin 1, which vasodilates and prevents smooth muscle proliferation
• SE: peripheral edema, liver function abnormalities, and anemia
• all of these meds end in “-entan”

Question 11

PDE5 Inhibitors in Pulmonary HTN

Answer 11

-MOA: blocks the action of PDE5, which increases cGMP and leads to vasodilation and prevention of smooth muscle proliferation
-SE: HA, flushing, rhinitis
-CANNOT TAKE with nitrates (nitro and isordil)
Sildenafil and Tadalafil

Question 12

Management of Pulmonary HTN

Answer 12

• Oral ERA or PDE5 inhibitor is first line
• followed by inhaled prostacyclin analogue
• followed by IV or SQ prostacyclin for advanced dx