Pirfenex: Slows Idiopathic Pulmonary Fibrosis Progression
Pirfenex
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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 process, Pirfenex works to reduce the rate of decline in lung function, offering a vital therapeutic option for appropriate patients. Its efficacy is supported by robust clinical data demonstrating a significant impact on disease progression and functional capacity.
Features
- Active Pharmaceutical Ingredient: Pirfenidone.
- Available Dosage Forms: Film-coated tablets (200 mg, 400 mg, 600 mg).
- Pharmacologic Class: Antifibrotic agent.
- Mechanism of Action: Exerts antifibrotic, anti-inflammatory, and antioxidant effects; believed to downregulate the production of growth factors and procollagens, inhibiting the proliferation of fibroblasts and the deposition of extracellular matrix.
- Bioavailability: Well-absorbed orally, with peak plasma concentrations reached within 3 hours. High-fat meals significantly increase exposure.
- Metabolism: Primarily hepatically metabolized via multiple cytochrome P450 isoenzymes (mainly CYP1A2).
- Elimination: Excretion is primarily renal (≈80%) as metabolites.
Benefits
- Slows Disease Progression: Clinically proven to reduce the rate of decline in forced vital capacity (FVC), a key measure of lung function and a 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 analysis of phase III trials (ASCEND and CAPACITY) showed a significant reduction in the risk of all-cause mortality at one year.
- Preserves Exercise Tolerance: Associated with a reduction in the decline of six-minute walk distance (6MWD), an important measure of functional capacity.
- Offers a Pharmacological Standard of Care: Provides a well-characterized, evidence-based treatment option where few effective therapies exist.
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 characterized by a histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Diagnosis should be established by a multidisciplinary team experienced in interstitial lung diseases, based on high-resolution computed tomography (HRCT) and, when necessary, histologic evaluation. It is not indicated for other interstitial lung diseases.
Dosage and direction
- Initial Titration: To improve gastrointestinal tolerability, the dosage must be titrated to the full maintenance dose over a 14-day period.
- Days 1-7: 267 mg (one 267 mg tablet) three times daily (801 mg/day).
- Days 8-14: 534 mg (two 267 mg tablets) three times daily (1602 mg/day).
- Maintenance Dose: From Day 15 onward, the full maintenance dose is 801 mg (three 267 mg tablets) three times daily with food, for a total daily dose of 2403 mg.
- Administration: Tablets must be taken with food at the same time each day (e.g., with breakfast, lunch, and dinner) to minimize the risk of nausea and dizziness. The tablets should be swallowed whole and must not be chewed, broken, or crushed.
- Dosage Modification: Dose reduction or interruption is recommended for management of adverse reactions (e.g., gastrointestinal symptoms, photosensitivity reaction, rash, elevated liver enzymes). Consult the full prescribing information for specific guidance.
Precautions
- Photosensitivity and Phototoxicity: Pirfenex can cause serious skin reactions following exposure to sunlight (including sunlamps) or even brief exposure to indirect sunlight. Patients must be advised to avoid sun exposure, use a high-SPF (50+) broad-spectrum sunscreen, 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, monthly for the first 6 months, and then every 3 months thereafter. Dosage modification or discontinuation is required for significant elevations.
- Gastrointestinal Disorders: Nausea, diarrhea, dyspepsia, vomiting, and gastroesophageal reflux disease are very common. These can often be managed by taking the medication with food, dose titration, and/or concomitant antiemetic or antacid therapy.
- 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.
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 (CrCl <30 mL/min) or end-stage renal disease requiring dialysis.
- Concomitant use of strong or moderate CYP1A2 inhibitors (e.g., fluvoxamine, enoxacin), due to the risk of significantly increased pirfenidone exposure and toxicity.
Possible side effect
The most common adverse reactions (incidence ≥10% and more frequent 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 liver injury and severe photosensitivity reactions.
Drug interaction
Pirfenidone is primarily metabolized by CYP1A2, with minor contributions from other CYP isoenzymes. Significant interactions include:
- CYP1A2 Inhibitors (Strong/Moderate): Concomitant use is contraindicated (e.g., fluvoxamine, enoxacin) or not recommended as they can dramatically increase pirfenidone plasma levels. This includes some fluoroquinolone antibiotics.
- CYP1A2 Inducers: Agents like omeprazole, cigarette smoking, and charbroiled foods may decrease pirfenidone exposure, potentially reducing its efficacy. Smokers may require a higher dose, though smoking cessation is strongly advised for IPF patients; a dose adjustment will be needed upon cessation.
- Other Agents: Caution is advised with other drugs known to cause photosensitivity (e.g., tetracyclines, fluoroquinolones, thiazides) or hepatotoxicity.
Missed dose
If a dose is missed, it should be skipped if the next dose is due within 3 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. Single doses up to 4005 mg and repeated doses up to 3000 mg/day for up to 7 days have been reported with adverse events consistent with the known safety profile (nausea, vomiting, dizziness). In case of suspected overdose, symptomatic and supportive medical treatment should be initiated. There is no known specific antidote. Hemodialysis is unlikely to be effective due to the high protein binding of pirfenidone.
Storage
- Store at room temperature (20°C to 25°C / 68°F to 77°F); excursions permitted between 15°C and 30°C (59°F to 86°F).
- Keep the bottle tightly closed in its original packaging to protect from light and moisture.
- Keep out of reach of children and pets.
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 course of treatment. Never disregard professional medical advice or delay in seeking it because of something you have read here. The information provided is based on the product’s prescribing information but may not be exhaustive.
Reviews
- Clinical Expert Consensus (2023): “Pirfenidone remains a foundational therapy in our armamentarium against IPF. The data on slowing FVC decline is incontrovertible. While the side effect profile requires diligent management, particularly regarding GI upset and photosensitivity, the benefit in altering the disease’s natural history is significant for the majority of our patients.”
- Pulmonologist, Academic Medical Center: “In my practice, patient education is paramount when initiating Pirfenex. Setting clear expectations about the titration schedule, the necessity of taking it with food, and the critical importance of sun protection makes tolerability much higher. For those who can tolerate it, it provides a tangible sense of actively managing their disease.”
- IPF Patient (3 years on therapy): “The first few months were tough with the nausea, but my doctor worked with me on timing my doses and it got much better. Knowing that I’m on a medication that is actively working to slow this disease down gives me peace of mind. The sun sensitivity is very real—I never leave the house without my hat and SPF 70, but it’s a small price to pay.”