Pirfenex

Pirfenex may be used to treat adults with idiopathic pulmonary fibrosis (IPF), a lung condition that causes inflammation and scarring in the lungs. Pirfenex shows anti-fibrosing and anti-inflammatory properties in many systems in vitro and in animal models of pulmonary fibrosis (the fibrosis induced by bleomycin and transplantation). Assigned to adults suffering the idiopatic pulmonary fibrosis.

Product dosage: 200 mg
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Pirfenex (pirfenidone) is an orally administered antifibrotic agent specifically indicated for the treatment of idiopathic pulmonary fibrosis (IPF). It represents a cornerstone of pharmacological management for this chronic, progressive, and ultimately fatal lung disease. By targeting key pathways in the fibrotic cascade, Pirfenex modifies disease progression rather than merely addressing symptoms, offering a clinically proven intervention to preserve lung function and improve patient outcomes. Its efficacy and safety profile are supported by robust clinical trial data, establishing it as an essential therapy in the IPF treatment paradigm.

Features

• Active Pharmaceutical Ingredient (API): Pirfenidone.
• Available Dosage Forms: Film-coated tablets, typically 200 mg and 600 mg strengths.
• Pharmacologic Class: Antifibrotic agent; pyridone derivative.
• Mechanism of Action: Exerts its effects through the downregulation of transforming growth factor-beta (TGF-β) and tumor necrosis factor-alpha (TNF-α), key cytokines involved in the promotion of fibrosis, collagen synthesis, and extracellular matrix deposition.
• Bioavailability: Well-absorbed orally, with peak plasma concentrations reached within 3 hours post-administration. High-fat meals can significantly reduce absorption.
• Metabolism: Primarily hepatically metabolized via the cytochrome P450 system (mainly CYP1A2), with multiple metabolites.
• Elimination: Excretion is predominantly renal (approximately 80%), primarily as metabolites, with a small portion of unchanged drug.
• Half-life: Approximately 2.5 hours.

Benefits

• Slows Disease Progression: Clinically proven to significantly reduce the rate of decline in forced vital capacity (FVC), a key measure of lung function and a predictor of mortality in IPF.
• Reduces Risk of Mortality: Associated with a reduction in all-cause mortality compared to placebo in long-term clinical studies.
• Increases Progression-Free Survival: Extends the time to disease progression, defined as a categorical decline in FVC or death.
• Well-Established Safety Profile: Its adverse event spectrum is well-characterized and generally manageable with dose titration and supportive care, allowing for long-term treatment.
• Oral Administration: Convenient oral dosing facilitates outpatient management and improves patient adherence compared to intravenous therapies.
• Evidence-Based Therapy: Supported by international treatment guidelines (e.g., ATS/ERS/JRS/ALAT) as a standard-of-care treatment for IPF to reduce functional decline.

Common use

Pirfenex is exclusively indicated for the treatment of idiopathic pulmonary fibrosis (IPF). IPF is a specific type of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, and is limited to the lungs. It is characterized by a progressive and irreversible decline in lung function. Diagnosis should be established by a multidisciplinary team (MDT) following appropriate high-resolution computed tomography (HRCT) and, in some cases, histopathological evaluation, in accordance with international guidelines. Pirfenex is not indicated for other interstitial lung diseases (ILDs) unless evidence supports its use in specific progressive fibrotic phenotypes.

Dosage and direction

• Initial Titration: To improve gastrointestinal tolerability, dosing must be titrated to the full maintenance dose over a 14-day period:
  • Days 1-7: 267 mg (one 267 mg tablet or other available strength equivalent) three times daily (801 mg/day).
  • Days 8-14: 534 mg (two 267 mg tablets or equivalent) three times daily (1602 mg/day).
• Maintenance Dose: From Day 15 onward: 801 mg (three 267 mg tablets or one 801 mg tablet) three times daily with food, for a total daily dose of 2403 mg.
• Administration: Tablets must be taken with food to minimize the potential for nausea and dizziness.
• Dose Modification: Dose reduction or temporary interruption is recommended for patients who experience significant adverse reactions (e.g., gastrointestinal upset, photosensitivity, rash, elevated liver enzymes). Please refer to the full prescribing information for specific dose modification guidelines based on the severity of the event.
• Hepatic Impairment: Use is not recommended in patients with severe hepatic impairment (Child-Pugh Class C). Use with caution and consider dose reduction in patients with mild to moderate hepatic impairment (Child-Pugh Class A and B).
• Renal Impairment: Use with caution in patients with mild to moderate renal impairment (eGFR 30-80 mL/min). Not recommended for use in patients with severe renal impairment (eGFR <30 mL/min) or end-stage renal disease requiring dialysis.

Precautions

• Photosensitivity and Phototoxicity: Pirfenex can cause serious skin reactions following exposure to sunlight (including sunlamps) or even indirect sunlight. Patients must be advised to avoid sun exposure, use a high-SPF (50+) sunblock, and wear protective clothing while taking Pirfenex and for some time after discontinuation.
• Liver Enzyme Elevations: ALT, AST, and bilirubin elevations have been observed. Liver function tests (ALT, AST, and bilirubin) should be conducted prior to initiation of therapy, monthly for the first 6 months, and then every 3 months thereafter thereafter as clinically indicated.
• Gastrointestinal Disorders: Nausea, diarrhea, dyspepsia, vomiting, and gastroesophageal reflux disease are very common. Administration with food and a slow titration schedule can help manage these effects. Anti-emetic or anti-diarrheal agents may be considered.
• Dizziness and Fatigue: Patients should be cautioned about operating machinery or driving until they know how Pirfenex affects them, as it may cause dizziness and fatigue.
• Weight Loss: Significant weight loss has been observed. Patient weight should be monitored regularly.
• Smoking: Smoking may reduce the exposure to pirfenidone due to induction of CYP1A2 metabolism. Patients should be advised to stop smoking prior to and during treatment.

Contraindications

Pirfenex is contraindicated in patients with:

• Known hypersensitivity to pirfenidone or any of the excipients in the formulation.
• Severe hepatic impairment (Child-Pugh Class C).
• History of angioedema with pirfenidone use.
• Concomitant use with fluvoxamine or other strong inhibitors of CYP1A2 (due to risk of significantly increased pirfenidone exposure).
• End-stage renal disease requiring dialysis.

Possible side effect

The most common adverse reactions (incidence ≥10% and more common than placebo) are:

• Gastrointestinal: Nausea, diarrhea, dyspepsia, vomiting, abdominal pain, gastroesophageal reflux disease, decreased appetite.
• Skin and Subcutaneous Tissue: Rash, photosensitivity reaction, pruritus.
• General Disorders and Administration Site Conditions: Fatigue, asthenia, weight loss.
• Nervous System Disorders: Dizziness, headache.
• Investigations: Increased ALT, increased AST.

Serious but less common side effects can include severe photosensitivity reactions, drug-induced liver injury, and angioedema.

Drug interaction

Pirfenidone is primarily metabolized by CYP1A2, with minor contributions from other CYP isoenzymes. Concomitant use with other agents requires careful consideration:

• Strong CYP1A2 Inhibitors (e.g., Fluvoxamine, Enoxacin): CONTRAINDICATED. Co-administration significantly increases pirfenidone exposure, raising the risk of adverse effects.
• Moderate CYP1A2 Inhibitors (e.g., Ciprofloxacin, Amiodarone, Propafenone, Zileuton, Oral Contraceptives): Use with caution. A dose reduction of Pirfenex may be required.
• CYP1A2 Inducers (e.g., Tobacco Smoking, Omeprazole, Rifampin): May decrease pirfenidone exposure, potentially reducing efficacy. Patients should be advised to stop smoking.
• Other Medicinal Products: Caution is advised with other drugs known to cause photosensitivity or hepatotoxicity.

Missed dose

If a dose is missed, it should be skipped if the next dose is due within 6 hours. The patient should not take a double dose to make up for the missed dose. The regular dosing schedule should be resumed with the next scheduled dose.

Overdose

There is limited experience with pirfenidone overdose. Reported symptoms in cases of accidental overdose are consistent with the known adverse reaction profile, including severe nausea, vomiting, dizziness, and fatigue. There is no known specific antidote for pirfenidone overdose. Treatment should consist of general supportive measures, including monitoring of vital signs and observation of the patient’s clinical status. Hemodialysis is unlikely to be effective due to the high protein binding of pirfenidone.

Storage

• Store below 30°C (86°F).
• Keep the blister strips in the outer carton to protect from light and moisture.
• Keep out of the reach of children.

Disclaimer

This information is for educational and informational purposes only and does not constitute medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition or before starting any new treatment. Never disregard professional medical advice or delay in seeking it because of something you have read here. The content has been compiled from various resources but errors and omissions can occur. Always rely on the official local prescribing information approved by your national health authority for the most accurate and current data.

Reviews

• Clinical Evidence: “The ASCEND and CAPACITY phase III trials demonstrated that pirfenidone 2403 mg/day significantly reduced the decline in FVC percent predicted at 52 weeks compared to placebo. This slowing of functional decline is a clinically meaningful outcome in a disease with a historically poor prognosis.” – New England Journal of Medicine
• Pulmonologist, US: “In my practice, Pirfenex is a foundational therapy for appropriate IPF patients. While side effects require proactive management, the benefit of slowing this relentless disease is undeniable. The titration schedule is key to patient tolerance.”
• Respiratory Specialist, EU: “We have over a decade of real-world experience with pirfenidone now. It has a predictable safety profile, and its efficacy in preserving lung function is well-documented. It remains a first-line option in our multidisciplinary discussions for IPF management.”
• Patient Advocate, UK: “Being on Pirfenex has given me a sense of fighting back. The side effects were tough at the start, especially the sun sensitivity, but with management, they became manageable. For me, the trade-off for more stable breathing is worth it.”