Drugs for Restrictive Lung Diseases, Cystic Fibrosis, and Pulmonary Artery HTN Flashcards
Portactant Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Surfactant Agent
MOA
Purified animal-derived products rich in surfactant proteins B and C, neutral lipids, surface active phospholipids, and dipalmitoylphosphatidyl-choline (DPPC)
Administration
Preterm infants born at less than 30 wks.
Adverse Effects
Contraindications
Other
Calfactant Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Surfactant Agent
MOA
Purified animal-derived products rich in surfactant proteins B and C, neutral lipids, surface active phospholipids, and dipalmitoylphosphatidyl-choline (DPPC)
Administration
Preterm infants born at less than 30 wks.
Adverse Effects
Contraindications
Other
Beractant Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Surfactant Agent
MOA
Purified animal-derived products rich in surfactant proteins B and C, neutral lipids, surface active phospholipids, and dipalmitoylphosphatidyl-choline (DPPC)
Administration
Preterm infants born at less than 30 wks.
Adverse Effects
Contraindications
Other
Methotrexate (pulm) Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Immunosuppressive
MOA
- DHFR inhibitor preventing pyrimidine synthesis
- Increased Adenosine Related Immunosuppression - AICAR builds builds up and inhibits AMP deaminase and ADA (adenosine) deaminase. AMP and adenosine move outside the cell to simulate A2a/b GPCR that increases cAMP and causes Immunosuppression
Administration
Given to Sarcoidosis patients
Adverse Effects
Dermatologic Reactions, Birth Defects, Maliganant Lymphoma, INTERSITIAL PNEUMONITIS and PULMONARY FIBROSIS
Contraindications
Other
Rituximab (pulm) Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
mAb - immunosuppressive
MOA
Binds CD20 to prevent B-lymphocyte maturation via 3 mechanisms 1) Macrophages and NK cells are recruited to bind Rituximab Fc receptors 2) Rituximab activates completment 3) induction of apoptosis
Metabolism Slowly metabolized (6-9 month depletion with a single 3-dose drug course)
Administration
Given to patients with Wegner’s
Adverse Effects
Hypertension, asthenia, Pruitis, urticarial, rhinitis, arthralgia
Contraindications
Other
Azathioprine (pulm) Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
DNA and RNA synthesis inhibitor
MOA
DNA and RNA synthesis inhibitor that produces immunosuppression possibly by facilitating apoptosis of T cell populations
Administration
Given to Patients with Wegner’s
Adverse Effects
Neoplastic, mutagenic, Leukopenic an THROMBOCYTOPENIC toxicity, and increased risk of infection
Contraindications
Other
Ivacaftor Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
CFTR potentiator
MOA
Treats CF patients with G551D mutation
Metabolism
Extensive Hepatic Metabolism (CYP3A4 and P-gp inhibition)
Adverse Effects
Upper respiratory symptoms, Pain, GI symptoms, and nasal congestion
Contraindications
INEFFECTIVE in patients homozygous for ∆F508
Other
Cyclophosphamide (pulm) Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Alkylating agent
MOA
Alkylates rapidly growing cells to produce B and T cell lymphopenia with selectivity toward suppression of B-lymphocyte activity
Administration
Used in the treatment of Wegner’s Granulomatosis
Adverse Effects
Associated with neutro- and thrombocytopenia, bladder cancer, myeloproliferative, or lymphoproliferative malignancies
Contraindications
Other
Corticosteriods Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Steroid
MOA
Inhibition or inflammatory cytokine synthesis via preventing inhibitor of kB (IkB) from unbining NfkB which woul lead to the production of cytokines like IL-1ß and TNF
Administration
Given to patients with Wegner’s Granulomatosis
Adverse Effects
Contraindications
Other
Epoprostenol (pulm) Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Prostanoid
MOA
Used in Pulmonary Artery HTN to mimic the effects of PGI2 (prostacyclin) which is to inhibit platelet agreggation, Induce vasodilation, and retard smooth muscle growth
Used in Pulmonary Artery HTN
Metabolism
Half-life of only 3-5 minutes
Administration
**Continuous IV infusion (RISK OF INFECTION)
Adverse Effects
Dose limiting Hypotension, flushing, muscle pain, bleeding risk
Contraindications
Other
Iloprost Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Prostanoid
MOA
Used in Pulmonary Artery HTN to mimic the effects of PGI2 (prostacyclin) which is to inhibit platelet agreggation, Induce vasodilation, and retard smooth muscle growth
Used in Pulmonary Artery HTN
Metabolism
Half-life of 25 minutes
Administration
requires 6-9 doses/day each lasting 10 minutes
Adverse Effects
HEMOPTYSIS, cough, flushing, muscle cramps, tongue/back pain
Contraindications
Other
Treprostinil Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Prostanoid
MOA
Used in Pulmonary Artery HTN to mimic the effects of PGI2 (prostacyclin) which is to inhibit platelet agreggation, Induce vasodilation, and retard smooth muscle growth
Used in Pulmonary Artery HTN
Metabolism
Half-life of 4 hours
Administration
Continuous subcutaneous IV infusion=> more stable in solution
Adverse Effects
CYP2C8 drug-drug interactions possible (Gemfibrozil decreases clearance, Rifampin increases clearance) Jaw pain, Injection site irritation, bleeing risk
Contraindications
Other
Bosentan Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Endothelin-1 antagonist
MOA
Given to people with PAH to block smooth muscle proliferation and pulmonary arterial vasoconstriction by binding endothelin-1 type A (smooth muscle) and type B (endothelial) receptors
Used in Pulmonary Artery HTN
Metabolism
5-8 hours; Extensive Hepatic metabolism by CYP2C9 and 3A4. (cyp inducers like Rifampin could cause drug-drug interactions
Administration
Oral Administration
Adverse Effects
Teratogenic effects, and LIVER (elevated LFTs) and BLOOD (anemia) toxicities, nasopharyngitis
Contraindications
Other
**Major advantage of prostacyclins is oral activity.
Lumacaftor/Ivacaftor Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
CFTR potentiator
MOA
Lumacafor added a conformational stabilizer to the drug action of Ivacaftor. This improves CFTR protein delivery to the cell surface
Metabolism
Lumacaftor is a strong CYP INDUCER meaning Ivacaftor stays in the system 3x as long (9-27 hours)
Adverse Effects
Upper respiratory symptoms, Pain, GI symptoms, and nasal congestion
Contraindications
Other
Ambrisentan Drug Class MOA Metabolism Administration Adverse Effects Contraindications Other
Drug Class
Endothelin-1 antagonist
MOA
Given to people with PAH to block smooth muscle proliferation and pulmonary arterial vasoconstriction by binding endothelin-1 type A (smooth muscle) and type B (endothelial) receptors
Used in Pulmonary Artery HTN
Metabolism
Half-life 15 hours; extensive CYP2C9, 3A4, OATP, and P-gp substrate
Administration
Oral Administration
Adverse Effects Teratogenic effects (note lack of liver (LFTs normal) and blood toxicities as seen with bosentan), PERIPHERAL EDEMA
Contraindications
Other
**Major advantage of prostacyclins is oral activity.
