Pirfenex: Slows Idiopathic Pulmonary Fibrosis Progression

Pirfenex

Price from 115.00 $

Show more

Product dosage: 200 mg
Package (num)	Per pill	Price	Buy
90	$1.28	$115.48 $115.48 (0%)	🛒 Add to cart
180	$1.01	$230.96 $181.76 (21%)	🛒 Add to cart
270	$0.84	$346.44 $226.95 (34%)	🛒 Add to cart
360	$0.78 Best per pill	$461.92 $281.17 (39%)	🛒 Add to cart

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 works to slow the decline in lung function, offering a vital therapeutic option for appropriate patients. Its efficacy is supported by robust clinical evidence from multinational phase III trials, establishing it as a fundamental intervention in the IPF treatment landscape.

Features

• Active Pharmaceutical Ingredient (API): Pirfenidone.
• Available Dosage Forms: Film-coated tablets (200 mg, 600 mg) and hard capsules (267 mg).
• Mechanism of Action: Multimodal antifibrotic, exerting effects through the downregulation of transforming growth factor-beta (TGF-β) and tumor necrosis factor-alpha (TNF-α), inhibition of collagen synthesis, and reduction of fibroblast proliferation.
• Pharmacokinetics: Rapidly and extensively absorbed following oral administration, with peak plasma concentrations (Tmax) achieved within 0.5 to 4 hours. High-fat meals significantly reduce absorption.
• Metabolism: Primarily hepatically metabolized via the CYP1A2 enzyme system, with minor contributions from other CYP isozymes (e.g., CYP2C9, 2C19, 2D6, 2E1).
• Excretion: Primarily renal (≈80%) as metabolites, with minimal (<1%) unchanged drug excreted in urine.
• Half-life: Approximately 2.5 hours.

Benefits

• Slows Disease Progression: Clinically proven to reduce the rate of decline in forced vital capacity (FVC), a key predictor of mortality in IPF.
• Improves Progression-Free Survival: Demonstrated in clinical trials to significantly increase the time to disease progression (absolute decline in FVC ≥10% predicted or death).
• May Prolong Survival: Pooled data from pivotal trials (ASCEND and CAPACITY) suggest a potential mortality benefit, reducing the risk of death from any cause at 1 year.
• Preserves Functional Capacity: By attenuating the loss of lung function, it can help maintain patients’ exercise tolerance and quality of life for a longer period.
• Well-Established Safety Profile: The side effect profile is well-characterized and generally manageable with appropriate dose titration and prophylactic strategies.

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 limited to the lungs. It is characterized by a histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Diagnosis should be established by a multidisciplinary team (MDT) including pulmonologists, radiologists, and pathologists experienced in interstitial lung disease, following comprehensive evaluation to exclude other known causes of interstitial lung disease (e.g., domestic and occupational environmental exposures, connective tissue disease, or drug toxicity).

Dosage and direction

• Initial Titration: To improve gastrointestinal tolerability, therapy must be initiated with a dose-escalation regimen over 14 days.
  • Days 1-7: 267 mg (one capsule) three times daily (801 mg/day).
  • Days 8-14: 534 mg (two capsules) three times daily (1602 mg/day).
• Maintenance Dose: From Day 15 onward, the recommended maintenance dosage is 801 mg (three capsules) three times daily with food, for a total daily dose of 2403 mg.
• Tablet Regimen: For the 600 mg tablet formulation, the maintenance dose is one tablet three times daily with food (1800 mg/day), following a similar 2-week titration schedule from a lower dose.
• Administration: Must be taken with food at the same time each day (e.g., breakfast, lunch, dinner) to minimize the incidence of nausea and dizziness. Swallow capsules/tablets whole; do not break, crush, or chew.

Precautions

• Photosensitivity and Phototoxicity: Pirfenex can cause serious skin reactions following exposure to sunlight (even through glass) or artificial light sources (e.g., tanning beds). Patients must be advised to use a high-SPF (≥50) broad-spectrum sunscreen, wear sun-protective clothing (long sleeves, hats), and avoid direct sun exposure throughout treatment and for several weeks after discontinuation.
• Liver Enzyme Elevations: ALT, AST, and bilirubin elevations have been observed. Liver function tests (ALT, AST, and bilirubin) must be conducted prior to initiation, monthly for the first 6 months, and then every 3 months thereafter thereafter. Dosage modification or discontinuation is required for significant elevations.
• Gastrointestinal Disorders: Nausea, diarrhea, dyspepsia, vomiting, and gastroesophageal reflux disease (GERD) are very common. Prophylactic use of antiemetics or acid-reducing agents (e.g., proton pump inhibitors) is recommended, especially during dose titration.
• Dizziness and Fatigue: Patients should be cautioned about engaging in activities requiring mental alertness (e.g., driving, operating machinery) until they know how Pirfenex affects them, particularly during the titration phase.
• Weight Loss: Monitor patient weight regularly. Significant unintentional weight loss may require nutritional support and dose adjustment.

Contraindications

Pirfenex is contraindicated in patients with:

• Hypersensitivity to pirfenidone or any of the excipients in the formulation.
• Moderate (Child-Pugh Class B) or Severe (Child-Pugh Class C) hepatic impairment.
• End-stage renal disease (ESRD) requiring dialysis.
• Concomitant use of strong CYP1A2 inhibitors (e.g., fluvoxamine, enoxacin). Use with caution with moderate CYP1A2 inhibitors (e.g., ciprofloxacin).

Possible side effect

The most common adverse reactions (incidence >5% and greater than placebo) are:

• Very Common (≥1/10): Nausea, rash, fatigue, diarrhea, dyspepsia, anorexia, photosensitivity reaction, abdominal pain, vomiting, asthenia, dizziness, GERD.
• Common (≥1/100 to <1/10): Weight decrease, insomnia, headache, dysgeusia (taste disturbance), hot flush, pruritus, sunburn, myalgia, arthralgia, decreased appetite.
• Serious side effects include hepatotoxicity and severe skin reactions.

Drug interaction

Pirfenidone is primarily metabolized by CYP1A2. Concomitant use requires careful management:

• 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 extreme caution. A dose reduction of Pirfenex may be required. Avoid ciprofloxacin if possible.
• CYP1A2 Inducers (e.g., Smoking, Omeprazole, Rifampicin): May decrease pirfenidone plasma concentrations, potentially reducing efficacy. Patients should be advised to stop smoking. Monitor for loss of effect.
• 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 3 hours. Do not take a double dose to make up for a missed one. Resume the normal dosing schedule with the next planned dose. Patients should be instructed to never take more than 3 doses (capsules or tablets) at one time.

Overdose

• Symptoms: There is limited experience with overdose. Based on the drug’s pharmacology, exaggerated adverse effects such as severe nausea, vomiting, dizziness, photosensitivity, and lethargy are anticipated.
• Management: There is no specific antidote for pirfenidone overdose. Treatment should consist of supportive and symptomatic measures, including monitoring of vital signs and observation of the patient’s clinical status. Gastric lavage may be considered if performed soon after ingestion. Due to its high protein binding, pirfenidone is not expected to be dialyzable.

Storage

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

Disclaimer

This information is for educational purposes only and 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 can occur. Drug dosages, indications, and safety profiles are subject to change. Always refer to the latest official prescribing information provided by the manufacturer or regulatory authorities.

Reviews

• “As a pulmonologist specializing in ILD, Pirfenex has fundamentally changed our approach to managing IPF. While not a cure, the ability to demonstrably slow FVC decline provides a tangible benefit for our patients, offering them more time with preserved lung function. The side effects are real but manageable with a proactive and educated approach, particularly regarding photoprotection.” – Dr. A. Sharma, MD, FCCP.
• “The clinical trial data for pirfenidone is compelling and it remains a first-line therapy in all major international IPF guidelines. In practice, patient education is paramount for adherence. Setting clear expectations about the goal of therapy (slowing progression, not reversing it) and diligently managing GI side effects and photosensitivity are the keys to successful long-term treatment.” – Prof. E. Laurent, Respiratory Medicine.
• “After my IPF diagnosis, starting on Pirfenex was daunting due to the long list of potential side effects. With my doctor’s guidance on taking it with food and being religious with sunscreen and hats, I’ve tolerated it well. My lung function tests have been stable for over 18 months now, which my team is very pleased with. It’s given me hope.” – Patient T.D. (anonymized).