Pirfenidone 267mg Capsules
It is used to treat idiopathic pulmonary fibrosis.
Drug Class
Antifibrotic agent
Pharmacologic Class
Pyridone derivative
Pregnancy Category
Category C
FDA Approved
Oct 2014
DEA Schedule
Not Controlled
Overview
What is this medicine?
Pirfenidone is a medicine used to treat a lung disease called idiopathic pulmonary fibrosis (IPF). IPF causes scarring in the lungs, making it harder to breathe. Pirfenidone helps slow down the scarring process in your lungs.
How to Use This Medicine
Taking Your Medication Correctly
To get the most benefit from your medication, follow your doctor's instructions carefully. Read all the information provided with your prescription and follow the instructions closely. Take your medication with food to help your body absorb it properly. Establish a routine by taking your medication at the same time every day. Continue taking your medication as directed by your doctor or healthcare provider, even if you start to feel better.
Storing and Disposing of Your Medication
To maintain the quality and safety of your medication, store it at room temperature in a dry place, away from the bathroom. Keep the lid tightly closed to protect the medication from moisture and other environmental factors. Store all medications in a secure location, out of the reach of children and pets. When disposing of unused or expired medication, do not flush it down the toilet or pour it down the drain unless instructed to do so by your pharmacist or healthcare provider. Instead, check with your pharmacist for guidance on the best disposal method or participate in a local drug take-back program.
What to Do If You Miss a Dose
If you miss a dose, take it as soon as you remember, with food. However, if it's close to the time for your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses to make up for the missed one. Additionally, do not take more than three doses in a 24-hour period. If you miss 14 or more days of taking your medication, consult your doctor before restarting your treatment to ensure it's safe to do so.
To get the most benefit from your medication, follow your doctor's instructions carefully. Read all the information provided with your prescription and follow the instructions closely. Take your medication with food to help your body absorb it properly. Establish a routine by taking your medication at the same time every day. Continue taking your medication as directed by your doctor or healthcare provider, even if you start to feel better.
Storing and Disposing of Your Medication
To maintain the quality and safety of your medication, store it at room temperature in a dry place, away from the bathroom. Keep the lid tightly closed to protect the medication from moisture and other environmental factors. Store all medications in a secure location, out of the reach of children and pets. When disposing of unused or expired medication, do not flush it down the toilet or pour it down the drain unless instructed to do so by your pharmacist or healthcare provider. Instead, check with your pharmacist for guidance on the best disposal method or participate in a local drug take-back program.
What to Do If You Miss a Dose
If you miss a dose, take it as soon as you remember, with food. However, if it's close to the time for your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses to make up for the missed one. Additionally, do not take more than three doses in a 24-hour period. If you miss 14 or more days of taking your medication, consult your doctor before restarting your treatment to ensure it's safe to do so.
Lifestyle & Tips
- Take pirfenidone with food to help reduce stomach upset and other side effects.
- Protect your skin from the sun. Pirfenidone can make your skin more sensitive to sunlight, leading to severe sunburn or rash. Use sunscreen (SPF 50 or higher), wear protective clothing (hat, long sleeves), and avoid direct sun exposure, including tanning beds and sunlamps.
- Do not smoke while taking pirfenidone, as smoking can reduce the effectiveness of the medication.
Available Forms & Alternatives
Dosing & Administration
Adult Dosing
Standard Dose:
Initial titration: Day 1-7: 267 mg TID (801 mg/day). Day 8-14: 534 mg TID (1602 mg/day). Day 15 onwards: 801 mg TID (2403 mg/day). Take with food.
Dose Range:
801 - 2403 mg
Condition-Specific Dosing:
Idiopathic Pulmonary Fibrosis (IPF):
Titrate to 801 mg orally three times daily (2403 mg/day) with food.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established
Child:
Not established
Adolescent:
Not established
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment required.
Moderate:
No dose adjustment required.
Severe:
Not recommended (use with caution, monitor closely).
Dialysis:
Not recommended (use with caution, monitor closely).
Hepatic Impairment:
Mild:
No dose adjustment required.
Moderate:
Consider dose reduction or interruption if clinically indicated. Monitor LFTs closely.
Severe:
Not recommended.
Pharmacology
Mechanism of Action
Pirfenidone is an antifibrotic agent. Its precise mechanism of action in idiopathic pulmonary fibrosis (IPF) is not fully elucidated, but it is believed to exert its effects through multiple pathways. It inhibits the synthesis of TGF-beta (a pro-fibrotic mediator) and TNF-alpha (a pro-inflammatory cytokine). It also reduces fibroblast proliferation, collagen synthesis, and the production of other mediators involved in fibrosis and inflammation, thereby slowing the progression of fibrosis in the lungs.
Pharmacokinetics
Absorption:
Bioavailability:
Not precisely quantified (relative bioavailability with food is high)
Tmax:
0.5-4 hours (with food)
FoodEffect:
Food significantly increases AUC and Cmax, and reduces Cmax variability, leading to fewer GI side effects. Should be taken with food.
Distribution:
Vd:
Approximately 70 L
ProteinBinding:
Approximately 50-80%
CnssPenetration:
Limited
Elimination:
HalfLife:
Approximately 2-3 hours
Clearance:
Not available
ExcretionRoute:
Primarily renal (approximately 80% as metabolites)
Unchanged:
Less than 1% (in urine)
Pharmacodynamics
OnsetOfAction:
Not acutely defined (chronic therapy)
PeakEffect:
Not acutely defined (chronic therapy)
DurationOfAction:
Not acutely defined (chronic therapy)
Safety & Warnings
Side Effects
Serious Side Effects: Seek Medical Help Right Away
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor immediately or seek medical attention:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Chest pain or pressure
Sunburn
Unexplained bruising or bleeding
Liver problems, which can be rare but potentially deadly. Warning signs may include:
+ Dark urine
+ Tiredness
+ Decreased appetite
+ Upset stomach or stomach pain
+ Light-colored stools
+ Vomiting
+ Yellow skin or eyes
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects, contact your doctor if they bother you or do not go away:
Feeling dizzy, tired, or weak
Signs of a common cold
Sinus pain
Headache
Joint pain
Change in taste
Trouble sleeping
Weight loss
Stomach pain or heartburn
* Diarrhea, vomiting, upset stomach, and decreased appetite are common side effects of this medication. If you experience any of these, talk to your doctor about ways to manage them. Seek medical attention if they are severe, do not improve, or worsen over time.
Remember
This list is not exhaustive, and you may experience other side effects not mentioned here. If you have questions or concerns about side effects, contact your doctor for medical advice.
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor immediately or seek medical attention:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Chest pain or pressure
Sunburn
Unexplained bruising or bleeding
Liver problems, which can be rare but potentially deadly. Warning signs may include:
+ Dark urine
+ Tiredness
+ Decreased appetite
+ Upset stomach or stomach pain
+ Light-colored stools
+ Vomiting
+ Yellow skin or eyes
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects, contact your doctor if they bother you or do not go away:
Feeling dizzy, tired, or weak
Signs of a common cold
Sinus pain
Headache
Joint pain
Change in taste
Trouble sleeping
Weight loss
Stomach pain or heartburn
* Diarrhea, vomiting, upset stomach, and decreased appetite are common side effects of this medication. If you experience any of these, talk to your doctor about ways to manage them. Seek medical attention if they are severe, do not improve, or worsen over time.
Remember
This list is not exhaustive, and you may experience other side effects not mentioned here. If you have questions or concerns about side effects, contact your doctor for medical advice.
Seek Immediate Medical Attention If You Experience:
- Signs of liver problems: yellowing of your skin or the whites of your eyes (jaundice), dark urine, unusual tiredness, pain in the upper right side of your stomach.
- Severe skin reactions or rash, especially if accompanied by fever, blistering, or peeling skin.
- Severe nausea, vomiting, or diarrhea that does not go away.
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Be sure to describe the symptoms you experienced as a result of the allergy.
If you have a history of kidney disease or liver disease, as these conditions may affect how your body processes the medication.
* If you are currently taking any medications that may increase your skin's sensitivity to light, as this can lead to adverse reactions. There are numerous medications that can cause this sensitivity, so it is crucial to consult with your doctor or pharmacist if you are unsure.
Please note that this is not an exhaustive list of all potential interactions between this medication and other substances or health conditions. Therefore, it is vital to discuss all of your medications, including prescription and over-the-counter drugs, natural products, and vitamins, with your doctor and pharmacist. This will help ensure that it is safe for you to take this medication in conjunction with your other treatments and health conditions.
Remember, do not initiate, discontinue, or modify the dosage of any medication without first consulting your doctor to confirm that it is safe to do so.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Be sure to describe the symptoms you experienced as a result of the allergy.
If you have a history of kidney disease or liver disease, as these conditions may affect how your body processes the medication.
* If you are currently taking any medications that may increase your skin's sensitivity to light, as this can lead to adverse reactions. There are numerous medications that can cause this sensitivity, so it is crucial to consult with your doctor or pharmacist if you are unsure.
Please note that this is not an exhaustive list of all potential interactions between this medication and other substances or health conditions. Therefore, it is vital to discuss all of your medications, including prescription and over-the-counter drugs, natural products, and vitamins, with your doctor and pharmacist. This will help ensure that it is safe for you to take this medication in conjunction with your other treatments and health conditions.
Remember, do not initiate, discontinue, or modify the dosage of any medication without first consulting your doctor to confirm that it is safe to do so.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication.
When taking this drug, you may be more susceptible to sunburn. To minimize this risk, avoid exposure to direct sunlight, sunlamps, and tanning beds. Use a broad-spectrum sunscreen with a high sun protection factor (SPF) and wear protective clothing and eyewear that shields you from the sun.
Quitting smoking is highly recommended before initiating this medication, and it is advisable to avoid smoking throughout the treatment period. Smoking may compromise the efficacy of this drug. Consult your doctor to discuss the implications of smoking on your treatment.
Regular blood tests and other laboratory assessments are crucial while taking this medication. Ensure that you undergo these tests as scheduled by your doctor.
If you are pregnant, planning to become pregnant, or are breastfeeding, it is vital to discuss your situation with your doctor. You and your doctor will need to weigh the benefits and risks of this medication to both you and your baby.
When taking this drug, you may be more susceptible to sunburn. To minimize this risk, avoid exposure to direct sunlight, sunlamps, and tanning beds. Use a broad-spectrum sunscreen with a high sun protection factor (SPF) and wear protective clothing and eyewear that shields you from the sun.
Quitting smoking is highly recommended before initiating this medication, and it is advisable to avoid smoking throughout the treatment period. Smoking may compromise the efficacy of this drug. Consult your doctor to discuss the implications of smoking on your treatment.
Regular blood tests and other laboratory assessments are crucial while taking this medication. Ensure that you undergo these tests as scheduled by your doctor.
If you are pregnant, planning to become pregnant, or are breastfeeding, it is vital to discuss your situation with your doctor. You and your doctor will need to weigh the benefits and risks of this medication to both you and your baby.
Overdose Information
Overdose Symptoms:
- Increased severity of known side effects (e.g., severe nausea, vomiting, diarrhea, rash, photosensitivity, liver enzyme elevations).
What to Do:
Seek immediate medical attention or call a poison control center (e.g., 1-800-222-1222). Treatment is supportive and symptomatic.
Drug Interactions
Contraindicated Interactions
- Fluvoxamine (strong CYP1A2 inhibitor)
Major Interactions
- Other strong CYP1A2 inhibitors (e.g., enoxacin, ciprofloxacin, amiodarone, propafenone, mexiletine, fluvoxamine - if not contraindicated)
- Strong CYP1A2 inducers (e.g., rifampin, omeprazole, smoking)
Moderate Interactions
- Moderate CYP1A2 inhibitors (e.g., ciprofloxacin, amiodarone, propafenone, mexiletine - if not contraindicated)
- Moderate CYP1A2 inducers (e.g., smoking cessation, certain herbal products)
Minor Interactions
- Not specifically listed as minor, but caution with any drug affecting CYP1A2
Monitoring
Baseline Monitoring
Liver Function Tests (ALT, AST, bilirubin)
Rationale: To establish baseline liver function and identify pre-existing hepatic impairment, as pirfenidone can cause liver injury.
Timing: Prior to initiation of therapy.
Renal Function (SCr, eGFR)
Rationale: To assess baseline renal function, as pirfenidone is primarily renally eliminated.
Timing: Prior to initiation of therapy.
Routine Monitoring
Liver Function Tests (ALT, AST, bilirubin)
Frequency: Monthly for the first 6 months, then every 3 months thereafter, or as clinically indicated.
Target: Within normal limits or stable from baseline.
Action Threshold: Discontinue pirfenidone if ALT or AST > 5 x ULN, or if ALT/AST > 3 x ULN with concomitant hyperbilirubinemia (> 2 x ULN). Dose reduction or interruption for lesser elevations.
Signs and symptoms of photosensitivity/rash
Frequency: Regularly during treatment.
Target: Absence of severe rash or photosensitivity.
Action Threshold: Dose reduction or interruption for severe reactions. Advise patients to use sun protection.
Gastrointestinal symptoms (nausea, diarrhea, dyspepsia)
Frequency: Regularly during treatment.
Target: Tolerable symptoms.
Action Threshold: Dose reduction or interruption for severe or intolerable symptoms.
Symptom Monitoring
- Nausea
- Diarrhea
- Dyspepsia
- Abdominal pain
- Vomiting
- Fatigue
- Dizziness
- Headache
- Insomnia
- Photosensitivity reaction
- Rash
- Pruritus
- Weight decrease
Special Patient Groups
Pregnancy
Pirfenidone is Pregnancy Category C. There are no adequate and well-controlled studies in pregnant women. Animal studies have shown adverse effects on embryo-fetal development. Use during pregnancy only if the potential benefit justifies the potential risk to the fetus.
Trimester-Specific Risks:
First Trimester:
Potential for developmental toxicity based on animal data.
Second Trimester:
Potential for developmental toxicity based on animal data.
Third Trimester:
Potential for developmental toxicity based on animal data.
Lactation
It is not known whether pirfenidone or its metabolites are excreted in human milk. Pirfenidone and/or its metabolites were excreted in the milk of lactating rats. Because of the potential for serious adverse reactions in breastfed infants, a decision should be made whether to discontinue nursing or to discontinue the drug, taking into account the importance of the drug to the mother.
Infant Risk:
Moderate risk (L3) - potential for serious adverse reactions based on animal data; human data lacking.
Pediatric Use
The safety and effectiveness of pirfenidone in pediatric patients have not been established. It is not recommended for use in this population.
Geriatric Use
No overall differences in safety or effectiveness were observed between elderly and younger patients. No specific dose adjustment is required based on age alone, but monitor for adverse effects as elderly patients may be more sensitive to side effects.
Clinical Information
Clinical Pearls
- Always titrate the dose gradually over 15 days to the target maintenance dose of 801 mg TID to improve tolerability and reduce gastrointestinal side effects.
- Emphasize the importance of taking pirfenidone with food to minimize GI upset.
- Strict sun protection measures are crucial due to the risk of severe photosensitivity reactions.
- Regular liver function monitoring is essential, especially during the first 6 months of therapy.
- Counsel patients on the signs and symptoms of liver injury and photosensitivity, and when to seek medical attention.
- Smoking cessation is strongly advised as smoking can significantly reduce pirfenidone exposure and efficacy.
Alternative Therapies
- Nintedanib (another FDA-approved antifibrotic for IPF)
Cost & Coverage
Average Cost:
Highly variable, typically >$10,000 per month
Generic Available:
Yes
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to high co-pays or co-insurance)
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor promptly. To ensure your safety, never share your medication with others or take someone else's medication. Some medications may come with an additional patient information leaflet, so be sure to check with your pharmacist for more information. If you have any questions or concerns about this medication, don't hesitate to reach out to your doctor, nurse, pharmacist, or other healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek medical attention. When reporting the incident, be prepared to provide details about the medication taken, the amount, and the time it occurred.