Pirfenidone 534mg Tablets
It is used to treat idiopathic pulmonary fibrosis.
Drug Class
Antifibrotic agent
Pharmacologic Class
Pyridone derivative
Pregnancy Category
C
FDA Approved
Oct 2014
DEA Schedule
Not Controlled
Overview
What is this medicine?
Pirfenidone is a medication used to treat a lung disease called Idiopathic Pulmonary Fibrosis (IPF). IPF causes scarring in the lungs, making it hard to breathe. Pirfenidone helps slow down the scarring process in the lungs, which can help preserve lung function and improve quality of life.
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 at the same time every day. Continue taking your medication as directed by your doctor or healthcare provider, even if you start to feel well.
Storing and Disposing of Your Medication
Store your medication at room temperature in a dry place, away from the bathroom. Keep the lid tightly closed and store all medications in a safe location, out of 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. Instead, check with your pharmacist for guidance on the best disposal method. You may also want to inquire about drug take-back programs in your area.
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 dose, skip the missed dose and resume your regular schedule. Do not take two doses at the same time or take extra doses. Be sure not to take more than three doses in a 24-hour period. If you miss 14 or more days of taking your medication, contact your doctor before restarting your treatment.
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 at the same time every day. Continue taking your medication as directed by your doctor or healthcare provider, even if you start to feel well.
Storing and Disposing of Your Medication
Store your medication at room temperature in a dry place, away from the bathroom. Keep the lid tightly closed and store all medications in a safe location, out of 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. Instead, check with your pharmacist for guidance on the best disposal method. You may also want to inquire about drug take-back programs in your area.
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 dose, skip the missed dose and resume your regular schedule. Do not take two doses at the same time or take extra doses. Be sure not to take more than three doses in a 24-hour period. If you miss 14 or more days of taking your medication, contact your doctor before restarting your treatment.
Lifestyle & Tips
- Take pirfenidone with food to reduce stomach upset (nausea, diarrhea).
- Avoid or minimize exposure to sunlight (including sunlamps and tanning beds) and use sunscreen (SPF 50 or higher) and protective clothing (hat, sunglasses) when outdoors, as pirfenidone can cause severe sunburn or rash (photosensitivity).
- 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; Day 8-14: 534 mg TID; Day 15 onwards: 801 mg TID. Maintenance dose: 801 mg (two 267 mg capsules or one 534 mg tablet and one 267 mg capsule) orally three times daily with food, for a total of 2403 mg/day.
Dose Range:
2403 - 2403 mg
Condition-Specific Dosing:
idiopathicPulmonaryFibrosis:
801 mg orally three times daily with food, total 2403 mg/day, after initial 2-week titration.
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 (limited data).
Dialysis:
Not recommended (limited data).
Hepatic Impairment:
Mild:
No dose adjustment required.
Moderate:
Consider dose reduction or interruption if clinically indicated. Monitor closely.
Severe:
Not recommended (increased exposure).
Confidence:
High
Pharmacology
Mechanism of Action
Pirfenidone is an antifibrotic agent. Its precise mechanism of action is not fully elucidated, but it is believed to exert its effects through multiple pathways. It inhibits the synthesis of TGF-beta (transforming growth factor-beta), a potent pro-fibrotic mediator, and TNF-alpha (tumor necrosis factor-alpha), a pro-inflammatory cytokine. It also reduces fibroblast proliferation and the production of collagen and other proteins involved in fibrosis. These actions collectively lead to a reduction in inflammation and fibrosis in the lungs.
Pharmacokinetics
Absorption:
Bioavailability:
Not precisely quantified, but rapidly absorbed.
Tmax:
0.5-4 hours (fasted), 2-7 hours (fed)
FoodEffect:
Food significantly decreases Cmax (by approximately 50%) and increases Tmax, but has a minimal effect on AUC. Taking with food reduces gastrointestinal side effects.
Distribution:
Vd:
Approximately 70 L
ProteinBinding:
Approximately 50-80%
CnssPenetration:
Limited
Elimination:
HalfLife:
Approximately 2-3 hours
Clearance:
Not precisely quantified, but rapid
ExcretionRoute:
Mainly renal (approximately 80% as metabolites, <1% as unchanged drug)
Unchanged:
<1%
Pharmacodynamics
OnsetOfAction:
Not acutely defined for antifibrotic effects; clinical benefits observed over months.
PeakEffect:
Not acutely defined for antifibrotic effects.
DurationOfAction:
Not acutely defined for antifibrotic effects; requires continuous dosing.
Safety & Warnings
Side Effects
Urgent 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 or seek immediate 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. Be aware of signs such as:
+ 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 for guidance:
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
Decreased appetite
These side effects are common with this medication. If you experience any of them, talk to your doctor about ways to manage or reduce their severity. If any side effects bother you, do not improve, or become severe, contact your doctor for advice.
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, don't hesitate to 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 or seek immediate 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. Be aware of signs such as:
+ 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 for guidance:
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
Decreased appetite
These side effects are common with this medication. If you experience any of them, talk to your doctor about ways to manage or reduce their severity. If any side effects bother you, do not improve, or become severe, contact your doctor for advice.
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, don't hesitate to contact your doctor for medical advice.
Seek Immediate Medical Attention If You Experience:
- Signs of liver problems: yellowing of skin or eyes (jaundice), dark urine, severe nausea or vomiting, unusual tiredness, pain in the upper right side of the stomach.
- Severe skin reactions: blistering, peeling, or widespread rash.
- Severe stomach problems: persistent or severe nausea, vomiting, or diarrhea that does not improve.
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.
If you have kidney disease or liver disease, as these conditions may affect how your body processes the medication.
If you are taking any medications that may increase your skin's sensitivity to light. There are numerous drugs that can cause this interaction, so consult 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 drugs or health conditions. To ensure your safety, it is crucial to:
Inform your doctor and pharmacist about all the medications you are taking, including prescription and over-the-counter drugs, natural products, and vitamins.
Discuss all your health problems with your doctor and pharmacist to verify that it is safe to take this medication in conjunction with your other treatments.
Never start, stop, or adjust the dosage of any medication without first consulting your doctor to avoid potential interactions or adverse effects.
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.
If you have kidney disease or liver disease, as these conditions may affect how your body processes the medication.
If you are taking any medications that may increase your skin's sensitivity to light. There are numerous drugs that can cause this interaction, so consult 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 drugs or health conditions. To ensure your safety, it is crucial to:
Inform your doctor and pharmacist about all the medications you are taking, including prescription and over-the-counter drugs, natural products, and vitamins.
Discuss all your health problems with your doctor and pharmacist to verify that it is safe to take this medication in conjunction with your other treatments.
Never start, stop, or adjust the dosage of any medication without first consulting your doctor to avoid potential interactions or adverse effects.
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. Additionally, use a sunscreen with adequate sun protection factor (SPF) and wear protective clothing and eyewear when going outside.
If you smoke, it is recommended that you quit before starting this medication. Continuing to smoke while taking this drug may reduce its effectiveness. Discuss smoking cessation options with your doctor.
Regular blood tests and other laboratory evaluations will be necessary to monitor your condition while taking this medication. Adhere to the schedule outlined by your doctor for these tests.
If you are pregnant, planning to become pregnant, or are breastfeeding, notify your doctor immediately. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision.
When taking this drug, you may be more susceptible to sunburn. To minimize this risk, avoid exposure to direct sunlight, sunlamps, and tanning beds. Additionally, use a sunscreen with adequate sun protection factor (SPF) and wear protective clothing and eyewear when going outside.
If you smoke, it is recommended that you quit before starting this medication. Continuing to smoke while taking this drug may reduce its effectiveness. Discuss smoking cessation options with your doctor.
Regular blood tests and other laboratory evaluations will be necessary to monitor your condition while taking this medication. Adhere to the schedule outlined by your doctor for these tests.
If you are pregnant, planning to become pregnant, or are breastfeeding, notify your doctor immediately. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision.
Overdose Information
Overdose Symptoms:
- Increased severity of known side effects (e.g., nausea, vomiting, diarrhea, rash, fatigue, dizziness, liver enzyme elevations).
What to Do:
There is no specific antidote for pirfenidone overdose. Treatment should be supportive and symptomatic. Contact a poison control center immediately. Call 1-800-222-1222.
Drug Interactions
Contraindicated Interactions
- Fluvoxamine (strong CYP1A2 inhibitor)
Major Interactions
- Strong CYP1A2 inhibitors (e.g., ciprofloxacin, amiodarone, propafenone, mexiletine, fluvoxamine - though fluvoxamine is contraindicated, others require dose adjustment or avoidance)
- Moderate CYP1A2 inhibitors (e.g., enoxacin)
Moderate Interactions
- Other CYP inhibitors (e.g., omeprazole, rifampicin, smoking cessation with bupropion)
Minor Interactions
- Not specifically listed for minor interactions with clinical significance.
Monitoring
Baseline Monitoring
Liver function tests (ALT, AST, bilirubin)
Rationale: Pirfenidone can cause liver injury, including drug-induced liver injury (DILI).
Timing: Prior to initiation of therapy.
Renal function (serum creatinine, eGFR)
Rationale: To assess baseline kidney function, especially given renal excretion of metabolites.
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, and as clinically indicated.
Target: Within normal limits or stable baseline.
Action Threshold: Discontinue if ALT/AST > 5 x ULN or if ALT/AST > 3 x ULN with concomitant hyperbilirubinemia (> 2 x ULN). Consider dose reduction or interruption if ALT/AST > 3 to ⤠5 x ULN.
Signs and symptoms of liver injury
Frequency: Regularly (patient education for self-monitoring).
Target: Absence of symptoms.
Action Threshold: Jaundice, dark urine, right upper quadrant pain, fatigue, nausea, vomiting. Prompt evaluation required.
Signs and symptoms of photosensitivity/rash
Frequency: Regularly (patient education for self-monitoring).
Target: Absence of symptoms.
Action Threshold: Severe rash, blistering, or signs of Stevens-Johnson syndrome/TEN. Discontinue if severe.
Symptom Monitoring
- Nausea
- Diarrhea
- Dyspepsia
- Abdominal pain
- Fatigue
- Headache
- Dizziness
- Insomnia
- Rash
- Photosensitivity reaction
- Anorexia
- Weight decrease
- Jaundice
- Dark urine
- Right upper quadrant pain
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. Due to 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:
Not available; potential for serious adverse reactions.
Pediatric Use
The safety and effectiveness of pirfenidone in pediatric patients have not been established. It is not indicated for use in children.
Geriatric Use
No overall differences in safety or effectiveness were observed between elderly patients (âĨ65 years) and younger patients. No specific dose adjustment is required based on age alone.
Clinical Information
Clinical Pearls
- Always advise patients to take pirfenidone with food to minimize gastrointestinal side effects.
- Emphasize strict sun protection measures due to the high risk of photosensitivity.
- Regular and timely liver function monitoring is crucial, especially during the first 6 months of therapy.
- Educate patients on symptoms of liver injury and severe rash, and when to seek immediate medical attention.
- Smoking cessation is strongly recommended as it can significantly reduce pirfenidone exposure and efficacy.
- Dose titration is essential to improve tolerability and should be followed as prescribed.
Alternative Therapies
- Nintedanib (another antifibrotic agent for IPF)
Cost & Coverage
Average Cost:
$9,000 - $12,000 per 30 tablets (534mg)
Generic Available:
Yes
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to step therapy)
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor for further guidance. 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 your 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 seeking help, be prepared to provide details about the medication taken, the amount, and the time it happened.