Pirfenex: Slows Idiopathic Pulmonary Fibrosis Progression

Pirfenex

Product dosage: 200 mg
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Pirfenex (pirfenidone) is an oral antifibrotic agent specifically indicated for the treatment of idiopathic pulmonary fibrosis (IPF). It is a disease-modifying therapy that targets the underlying pathological mechanisms of IPF, primarily by reducing the production of growth factors and procollagens involved in fibrosis. Clinical trials have demonstrated its efficacy in significantly slowing the decline in lung function, as measured by forced vital capacity (FVC), and in reducing the risk of mortality. This medication represents a cornerstone in the management of this chronic, progressive, and ultimately fatal interstitial lung disease, offering a targeted approach to delay disease progression.

Features

• Active Pharmaceutical Ingredient: Pirfenidone.
• Available Dosage Forms: Film-coated tablets (200 mg, 267 mg, 400 mg, 600 mg) and hard capsules (267 mg).
• Mechanism of Action: Exerts antifibrotic, anti-inflammatory, and antioxidant effects. It downregulates the production of transforming growth factor-beta (TGF-β) and tumor necrosis factor-alpha (TNF-α), key cytokines involved in the promotion of fibroblast proliferation and collagen deposition.
• Pharmacokinetics: Rapidly and extensively absorbed following oral administration, with a time to maximum plasma concentration (Tmax) of approximately 0.5 to 4 hours. Administration with food reduces the rate and extent of absorption but minimizes high peak concentrations associated with adverse effects.
• Metabolism: Primarily metabolized in the liver via the cytochrome P450 system (mainly CYP1A2, with minor contributions from CYP2C9, 2C19, 2D6, and 2E1). It has several metabolites, most of which are excreted in urine.
• Half-life: The elimination half-life of pirfenidone is approximately 3 hours in healthy volunteers and may be prolonged in patients with hepatic impairment.

Benefits

• Slows Disease Progression: The primary and most significant benefit is the deceleration of the rate of decline in lung function. This is objectively measured by a reduced loss of forced vital capacity (FVC) over time compared to placebo.
• Reduces Mortality Risk: Long-term clinical data and meta-analyses have shown a consistent reduction in the risk of all-cause mortality in patients with IPF treated with pirfenidone.
• Preserves Exercise Capacity: By slowing the fibrotic process and the associated loss of lung volume, pirfenidone can help maintain functional capacity and exercise tolerance for a longer period.
• Delays Disease Exacerbations: Treatment is associated with a lower incidence of acute exacerbations of IPF, which are critical events associated with high morbidity and mortality.
• Disease-Modifying Action: Unlike purely symptomatic treatments, pirfenidone directly interferes with the core pathways of fibrosis, offering a targeted therapeutic strategy.
• Well-Established Safety Profile: The safety and tolerability of pirfenidone have been extensively characterized in large, multinational clinical trials and post-marketing surveillance, allowing for predictable management of common side effects.

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) comprising pulmonologists, radiologists, and, when necessary, pathologists with expertise in interstitial lung diseases, following a thorough 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

Dosage must be titrated to the full maintenance dose to improve gastrointestinal tolerability.

• Initial Dosage: 267 mg (one capsule or tablet, depending on formulation) three times daily (801 mg/day) for the first 7 days.
• Dose Escalation: Increase to 534 mg (two 267 mg capsules/tablets or one 600 mg tablet, depending on available strengths) three times daily (1602 mg/day) for the next 7 days.
• Maintenance Dosage: From day 15 onward, the recommended maintenance dosage is 801 mg three times daily (2403 mg/day). This is typically achieved by administering three 267 mg capsules/tablets three times daily, or other combinations to achieve the 801 mg dose per administration (e.g., one 600 mg tablet and one 200 mg tablet, or one 400 mg and one 200 mg tablet, if available).
• Administration: Tablets and capsules must be swallowed whole with food to reduce the incidence of nausea and dizziness. The medication should be taken with the largest meal of the day to further mitigate gastrointestinal upset.
• Dosage Modification: Dose reduction or temporary interruption is recommended for patients who experience significant adverse reactions (e.g., persistent GI symptoms, photosensitivity reaction, liver enzyme elevations). Treatment may be restarted at a lower dose once the adverse event resolves, followed by a gradual re-titration to the full maintenance dose as tolerated.

Precautions

• Photosensitivity and Phototoxicity: Pirfenidone can cause serious skin reactions, including rash and sunburn, after exposure to sunlight (including through glass) and artificial light sources (e.g., tanning beds). Patients must be advised to avoid direct sunlight, use a high-protection sunscreen (SPF 50 or higher), and wear protective clothing that blocks UV light (e.g., long sleeves, hats) during treatment and for some time after discontinuation.
• Liver Function: Elevations in liver enzymes (ALT, AST, ALP, GGT) and bilirubin have been observed. Liver function tests (ALT, AST, and bilirubin) should be conducted prior to initiating therapy, monthly for the first 6 months, and every 3 months thereafter. Therapy should be interrupted or discontinued for significant elevations.
• Gastrointestinal Disorders: Nausea, diarrhea, dyspepsia, vomiting, and gastroesophageal reflux disease are very common. These can often be managed by taking the drug with food, using antiemetic or antidiarrheal agents, or with temporary dose reduction.
• Dizziness and Fatigue: Patients should be cautioned about engaging in activities requiring mental alertness, such as driving or operating machinery, until they know how pirfenidone affects them.
• Weight Loss: Clinically significant weight loss has been reported. Patient weight should be monitored regularly.
• Smoking: Smoking has been shown to reduce the exposure to pirfenidone due to induction of CYP1A2 metabolism. Patients should be strongly advised to stop smoking before and during treatment, as efficacy may be compromised.

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).
• Severe renal impairment (end-stage renal disease requiring dialysis) or severe renal impairment (CrCl <30 mL/min) due to a lack of data in this population.
• History of severe photosensitivity reactions or phototoxicity on previous pirfenidone therapy, or other diseases that make the patient predisposed to light-induced reactions (e.g., porphyria, lupus erythematosus).

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: Dizziness, headache, insomnia.
• Investigations: Increased liver enzymes (ALT, AST).

Serious but less common side effects can include severe liver injury and severe photosensitivity reactions leading to hospitalization.

Drug interaction

Pirfenidone is primarily metabolized by CYP1A2, with minor pathways involving other CYP isoenzymes. Concomitant use of drugs that inhibit or induce these enzymes can significantly alter pirfenidone exposure.

• Strong CYP1A2 Inhibitors (e.g., Fluvoxamine, Enoxacin): Concomitant use is not recommended as they can markedly increase pirfenidone plasma levels, increasing the risk of adverse effects.
• Moderate CYP1A2 Inhibitors (e.g., Ciprofloxacin, Amiodarone, Propafenone, Zileuton, Oral Contraceptives): Use with caution. Dose reduction of pirfenidone may be required.
• CYP1A2 Inducers (e.g., Tobacco Smoking, Omeprazole, Rifampin): May significantly decrease pirfenidone plasma levels, potentially reducing its efficacy. Patients must be advised to stop smoking. Concomitant use of other inducers should be avoided.
• Other Interactions: Drugs that cause dizziness or photosensitivity as a side effect (e.g., certain antibiotics, antihistamines, neuroleptics) may have additive effects when taken with pirfenidone.

Missed dose

If a dose is missed, it should be skipped if the next scheduled dose is due within 6 hours. The patient should not take a double dose to make up for the missed one. They should resume the normal dosing schedule with the next dose. Maintaining a consistent dosing schedule with meals is important for managing side effects and efficacy.

Overdose

There is limited experience with pirfenidone overdose in humans. Based on its pharmacological profile, symptoms of overdose may be an exaggeration of its known adverse effects, including severe nausea, vomiting, dizziness, drowsiness, and photosensitivity reaction. 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. 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 bottle tightly closed to protect from moisture and light.
• Keep out of the reach of children and pets.
• Do not use after the expiration date printed on the packaging.

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, stopping, or changing any prescribed part of your care plan. Never disregard professional medical advice or delay in seeking it because of something you have read here. The product information provided may not be exhaustive and is subject to change. Always refer to the latest official prescribing information provided by the manufacturer or regulatory authorities in your country.

Reviews

• “As a pulmonologist specializing in ILD for over 20 years, the introduction of pirfenidone has fundamentally changed our treatment paradigm for IPF. While not a cure, the ability to demonstrably slow FVC decline gives our patients more meaningful time. Managing the GI side effects requires a proactive approach with patients, but it is almost always manageable with dose titration and supportive care.” – Dr. A. Sharma, MD, FCCP.
• “The clinical trial data for pirfenidone is robust and convincing. The consistent signal for mortality benefit across studies is particularly noteworthy. In real-world practice, it has proven to be a vital tool, though patient education on sun avoidance is absolutely critical for adherence and safety.” – Clinical Pharmacist, Major ILD Center.
• “After my IPF diagnosis, starting on Pirfenex was a decision I made with my doctor. The first few weeks were tough with nausea, but taking it with a big meal helped a lot. Two years later, my lung function tests have been stable. The strict sun protection is a lifestyle change, but a small price to pay for potentially more time.” – Patient, 68.