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 is a prescription antifibrotic medication specifically indicated for the treatment of idiopathic pulmonary fibrosis (IPF). It is designed to target the underlying disease mechanisms of IPF, a chronic and progressive lung condition characterized by scarring (fibrosis) of the lung tissue. By interfering with pathways involved in fibrosis, Pirfenex works to slow the decline in lung function, helping to preserve patients’ quality of life. This oral therapy represents a significant advancement in the management of this serious condition, offering a targeted approach to disease modification under the supervision of a specialist physician.

Features

• Contains the active pharmaceutical ingredient Pirfenidone.
• Available in 200 mg and 600 mg film-coated tablets for flexible dosing.
• Formulated for oral administration.
• Manufactured under strict Good Manufacturing Practice (GMP) standards.
• Prescription-only medication, ensuring supervised use.

Benefits

• Slows the rate of decline in forced vital capacity (FVC), a key measure of lung function.
• Reduces the risk of disease progression and acute exacerbations.
• Helps to preserve exercise capacity and physical functioning over time.
• Offers a well-characterized efficacy and safety profile based on extensive clinical trials.
• Provides a convenient oral dosing regimen that can be integrated into daily life.

Common use

Pirfenex is exclusively used for the treatment of idiopathic pulmonary fibrosis (IPF) in adult patients. IPF is a specific type of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults. It is not indicated for other interstitial lung diseases (ILDs) unless a diagnosis of IPF has been conclusively established by a multidisciplinary team, typically involving a pulmonologist and radiologist, following appropriate diagnostic criteria (e.g., high-resolution computed tomography - HRCT patterns). Its use is reserved for patients with mild to moderate impairment in lung function, as defined by specific pulmonary function test parameters.

Dosage and direction

The dosage of Pirfenex must be individually titrated to the recommended maintenance dose under the close supervision of a physician to manage potential side effects, particularly gastrointestinal events and photosensitivity.

• Initial Dose: 1 tablet of 200 mg taken three times daily (total 600 mg/day) with food for the first 7 days.
• Dose Escalation: Increase to 2 tablets of 200 mg three times daily (total 1200 mg/day) with food for the next 7 days.
• Maintenance Dose: From day 15 onward, the recommended maintenance dose is 1 tablet of 600 mg three times daily (total 1800 mg/day), taken with food.
• Administration: Tablets must be swallowed whole with a full meal to improve gastrointestinal tolerability. They should not be broken, crushed, or chewed.
• Dosage Adjustment: In patients who do not tolerate the maintenance dose, temporary dose reductions or interruptions may be necessary, followed by a re-titration attempt. Permanent dose reduction to 600 mg or 1200 mg daily (in divided doses) may be required for some patients.

Precautions

• Liver Function: Pirfenex can cause liver enzyme elevations. Liver function tests (ALT, AST, and bilirubin) must 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.
• Photosensitivity: Patients are at significantly increased risk for sunburn and rash from sunlight (including sun through windows) and even halogen and fluorescent lights. Patients must use a high-SPF (50+) sunscreen, wear protective clothing, and avoid direct sun exposure during treatment and for some time after discontinuation.
• Gastrointestinal Events: Nausea, diarrhea, dyspepsia, vomiting, and gastroesophageal reflux disease are very common. Taking the medication with food is essential. Anti-emetic or anti-diarrheal agents may be prescribed prophylactically or as needed.
• Weight Loss: Patients should be monitored for weight loss. The cause (e.g., nausea, diarrhea, anorexia) should be investigated and managed.
• Smoking: Smoking has been shown to significantly reduce the exposure to pirfenidone. Patients should be advised to stop smoking prior to and during treatment.
• Renal Impairment: Use with caution in patients with mild to moderate renal impairment (CLcr 30-80 mL/min). It is not recommended for use in patients with severe renal impairment (CLcr <30 mL/min) or end-stage renal disease requiring dialysis.

Contraindications

Pirfenex is contraindicated in the following patient populations:

• Patients with a known hypersensitivity to pirfenidone or any of the excipients in the formulation.
• Patients with a history of angioedema.
• Patients with severe hepatic impairment (Child-Pugh Class C).
• Concomitant use with fluvoxamine or other strong inhibitors of CYP1A2 (e.g., enoxacin).
• Concomitant use with CYP1A2 inducers (e.g., rifampicin, omeprazole [high dose], phenytoin).

Possible side effects

The most common adverse reactions (occurring in ≥10% of patients) are:

• Nausea
• Rash
• Abdominal pain
• Upper respiratory tract infection
• Diarrhea
• Fatigue
• Headache
• Dyspepsia (indigestion)
• Dizziness
• Vomiting
• Anorexia (loss of appetite)
• Photosensitivity reaction
• Insomnia

Serious side effects requiring immediate medical attention include:

• Severe liver injury: Signs include jaundice (yellowing of skin/eyes), dark urine, fatigue, nausea, vomiting, and right upper abdominal pain.
• Angioedema: Swelling of the face, lips, tongue, or throat, with potential difficulty breathing.
• Severe skin reactions, including Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN).

Drug interaction

Pirfenidone is primarily metabolized by the liver enzyme CYP1A2. Concomitant use with other drugs that affect this pathway can significantly alter pirfenidone levels, increasing the risk of toxicity or reducing efficacy.

• Strong CYP1A2 Inhibitors (CONTRAINDICATED): Fluvoxamine, enoxacin. Co-administration is prohibited as it can lead to a drastic increase in pirfenidone exposure and toxicity.
• Moderate CYP1A2 Inhibitors (Use with Caution): Ciprofloxacin, amiodarone, propafenone. Dose reduction of Pirfenex may be required.
• CYP1A2 Inducers (CONTRAINDICATED): Rifampicin, omeprazole (high dose >40mg/day), phenytoin, carbamazepine. Co-administration can significantly decrease pirfenidone exposure, rendering it ineffective.
• Other Interactions: Caution is advised with other drugs known to cause photosensitivity (e.g., tetracyclines, fluoroquinolones, thiazide diuretics, sulfonylureas, phenothiazines) due to the additive risk.

Missed dose

• If a dose is missed, it should be skipped if the next scheduled dose is due within 6 hours.
• Do not take a double dose to make up for a missed one.
• Resume the normal dosing schedule with the next meal.
• Inform your treating physician about the pattern of missed doses.

Overdose

• Symptoms: Expected to be an exaggeration of known adverse effects, particularly severe nausea, vomiting, dizziness, and photosensitivity reactions.
• Management: There is no specific antidote for pirfenidone overdose. Treatment consists of immediate discontinuation of the drug and institution of supportive and symptomatic care, including monitoring of vital signs and observation of the patient’s clinical status. Gastric lavage may be considered if ingestion was very recent. Hemodialysis is unlikely to be effective due to the high protein binding of pirfenidone.

Storage

• Store below 30°C (86°F).
• Keep the tablets in their original blister pack or bottle to protect them from light and moisture.
• 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 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 efficacy and safety profile described are based on clinical trial data; individual patient experiences may vary.

Reviews

• Clinical Trial Data (EXPERT PERSPECTIVE): The ASCEND and CAPACITY phase III clinical trials demonstrated that pirfenidone, the active ingredient in Pirfenex, significantly reduced the decline in FVC percent predicted compared to placebo over a 52-week period. A pooled analysis showed a 48% reduction in the proportion of patients with a ≥10% decline in FVC or death. Meta-analyses have consistently confirmed its efficacy in slowing disease progression in IPF. The safety profile is considered manageable with proactive monitoring and patient education.
• Pulmonologist Feedback: Specialists generally regard Pirfenex as a foundational therapy for appropriate IPF patients. The key to successful management is careful patient selection, thorough education on side effect management (especially photosensitivity), and diligent monitoring of liver function. The tolerability can be challenging initially for some patients, but with dose titration and supportive care, many can remain on long-term therapy.
• Patient Experience (AGGREGATED): Patient reports often highlight a period of adjustment during the initial titration phase, where gastrointestinal side effects are most prominent. Those who persist through this phase with medical support often report a stabilization of their breathing symptoms, though individual results vary. The requirement for strict sun protection is frequently cited as a significant lifestyle adjustment. Many express appreciation for having an active treatment option for a condition that was previously considered untreatable.