Pulmofib267 mg

Pulmofib is an antifibrotic medicine that is FDA-approved and internationally guideline-supported for the treatment of:

• Idiopathic Pulmonary Fibrosis (IPF) — a chronic, progressive, fibrosing interstitial lung disease of unknown cause. Pulmofib is indicated in adults to slow the decline in lung function (forced vital capacity) and disease progression. This is the established, primary approved indication.

Off-label / adjunct use (limited evidence): In some specialist pulmonology settings, Pulmofib is used off-label in other progressive fibrosing interstitial lung diseases (PF-ILD) with an IPF-like pattern, based on specialist judgement and limited trial data. This use should only be undertaken under the direct supervision of a pulmonologist experienced in interstitial lung disease, and is not a substitute for the established IPF indication.

Pulmofib does not cure pulmonary fibrosis and does not reverse existing lung scarring; it is used to slow further progression.

Pirfenidone is available as:

• Capsules: 267 mg
• Film-coated tablets: 267 mg, 534 mg, and 801 mg

Each dosage unit contains Pirfenidone as the active pharmaceutical ingredient, along with standard pharmaceutical excipients. The 534 mg and 801 mg tablet strengths are designed to reduce pill burden once the maintenance dose is reached.

Pulmofib is a small-molecule, orally administered antifibrotic and anti-inflammatory agent belonging to the pyridone chemical class. It was developed specifically to target the underlying fibrotic and inflammatory processes that drive idiopathic pulmonary fibrosis, a disease that was previously managed with only supportive care or immunosuppressive regimens of unproven benefit.

Clinical trials (including the CAPACITY and ASCEND studies) demonstrated that Pulmofib slows the rate of decline in forced vital capacity (FVC) and reduces the risk of disease progression and all-cause mortality in patients with IPF compared with placebo, leading to regulatory approval in multiple countries including the United States, European Union, Japan, and Bangladesh.

Pulmofib belongs to the therapeutic class of Antifibrotic agents, specifically pyridone-derivative antifibrotic and anti-inflammatory drugs used in the management of idiopathic pulmonary fibrosis.

The precise mechanism of action of Pirfenidone is not completely understood, but it is believed to exert combined antifibrotic, anti-inflammatory, and antioxidant effects through several pathways:

• Inhibition of fibroblast proliferation and the fibroblast-to-myofibroblast transition.
• Down-regulation of transforming growth factor-beta (TGF-β), a key cytokine that stimulates collagen and extracellular matrix production.
• Reduction of tumor necrosis factor-alpha (TNF-α) and other pro-inflammatory cytokine production.
• Reduction in the synthesis and accumulation of collagen and other extracellular matrix proteins in lung tissue.

By reducing these fibrotic and inflammatory drivers, Pirfenidone slows the progressive scarring of lung tissue characteristic of IPF.

Pharmacokinetics: Pirfenidone is absorbed orally with peak plasma concentration reached in roughly 1–3 hours; taking it with food reduces peak concentration and nausea. It is highly protein-bound (~99%) and is metabolized predominantly by the hepatic enzyme CYP1A2 (with minor contributions from CYP2C9, 2C19, 2D6, and 3A4), with a plasma elimination half-life of approximately 2.4 hours.

Pulmofib should be taken:

• By mouth, with food (a meal), to reduce the likelihood of nausea, dizziness, and other gastrointestinal effects.
• Capsules/tablets should be swallowed whole with water and not crushed or chewed unless otherwise directed.
• At consistent times each day, divided into three doses (morning, afternoon, evening).
• Patients should avoid or minimize sun/UV lamp exposure and use sunscreen and protective clothing while on treatment, due to photosensitivity risk.

Do not stop or change the dose of Pulmofib without consulting the prescribing physician, even if symptoms feel stable, as this can affect long-term disease control.

Pulmofib is metabolized mainly by CYP1A2, so drugs and habits that alter CYP1A2 activity significantly change its blood levels:

• Fluvoxamine (strong CYP1A2 inhibitor): markedly increases Pulmofib exposure (several-fold); concurrent use is contraindicated (see Contraindications). Fluvoxamine should be discontinued before starting Pulmofib.
• Ciprofloxacin and other moderate CYP1A2 inhibitors (e.g., amiodarone): can meaningfully increase Pulmofib exposure; dose reduction of Pulmofib and closer monitoring for adverse effects is advised, particularly at higher ciprofloxacin doses.
• Strong CYP1A2 inducers (e.g., rifampin, omeprazole to a lesser extent): may reduce Pulmofib plasma concentration and efficacy; avoid combined use if possible, or monitor for reduced effect.
• Cigarette smoking: induces CYP1A2 and can significantly lower Pulmofib blood levels, potentially reducing effectiveness. Patients should be advised to stop smoking before and during treatment, and to inform their physician of any change in smoking status, as dose adjustment may be needed.

Patients should inform their physician of all prescription, over-the-counter, and herbal products they use before starting Pulmofib.

Side effects reported with Pulmofib range from common and manageable to rare and serious.

Patients should seek prompt medical attention for signs of liver problems (yellowing of skin/eyes, dark urine, severe fatigue), severe skin reactions, or symptoms of angioedema/anaphylaxis while taking Pulmofib.

Pregnancy: There are no adequate and well-controlled studies of Pulmofib in pregnant women. Animal reproduction studies have not shown clear evidence of harm at clinically relevant exposures, but human data are insufficient to establish safety. Pulmofib should be used during pregnancy only if clearly needed and the potential benefit justifies the potential risk to the fetus; use only under close physician supervision.

Lactation: It is not known whether Pulmofib passes into human breast milk. Because many drugs are excreted in breast milk and the effect on a nursing infant is unknown, a decision should be made to discontinue nursing or discontinue Pulmofib, taking into account the importance of the drug to the mother — this should be decided in consultation with a physician.

The following precautions should be observed with Pulmofib therapy:

• Hepatotoxicity: Elevations in liver enzymes (ALT, AST) and bilirubin have occurred. Liver function tests should be performed before starting treatment, then monthly for the first 6 months, and every 3 months thereafter. Dose reduction, interruption, or discontinuation may be required based on results and symptoms (e.g., jaundice, dark urine, fatigue, right upper abdominal pain).
• Photosensitivity reaction and rash: Common with Pulmofib. Patients should avoid or minimize exposure to direct sunlight and sunlamps/tanning beds, wear sun-protective clothing, and apply a broad-spectrum sunscreen daily.
• Gastrointestinal effects: Nausea, vomiting, diarrhea, and dyspepsia are common; taking Pulmofib with food and, if needed, temporary dose reduction can improve tolerability.
• Weight loss and decreased appetite: Body weight should be monitored periodically during treatment.
• Dizziness and fatigue: Patients should use caution when driving or operating machinery until they know how Pulmofib affects them.
• Angioedema: Rare cases have been reported; discontinue immediately and seek emergency care if swelling of the face, lips, tongue, or throat, or difficulty breathing occurs.
• Renal and hepatic impairment (mild-moderate): Use with caution and appropriate monitoring; see Use in Special Populations for adjustment details. Severe impairment is contraindicated (see Contraindications).
• Smoking: Reduces effectiveness of Pulmofib through CYP1A2 induction; smoking cessation is strongly advised (see Interactions).

Limited clinical experience with Pulmofib overdose is available. In healthy volunteers, doses up to 4,005 mg/day (divided) have been tolerated with adverse effects generally similar to, but more frequent than, those seen at the recommended dose (notably gastrointestinal symptoms and photosensitivity).

In case of a suspected overdose, seek immediate medical attention or contact a poison control center/emergency services. There is no specific antidote; management is supportive, including monitoring of vital signs and clinical status, and treatment of specific symptoms as they arise. Do not attempt to manage a suspected overdose at home without medical guidance.

Store at room temperature (below 30°C), away from light and moisture. Keep out of reach of children.

Renal Impairment

No dose adjustment is needed in mild-to-moderate renal impairment. Pulmofib is not recommended in end-stage renal disease requiring dialysis (see Contraindications).

Hepatic Impairment

Use with caution and enhanced liver monitoring in mild-to-moderate hepatic impairment (Child-Pugh A or B); a lower dose may be considered if tolerability issues arise. Pulmofib is not recommended in severe hepatic impairment (see Contraindications).

Elderly

No overall differences in safety or efficacy have been observed in elderly patients compared with younger adults; no specific dose adjustment based on age alone is required, though age-related renal/hepatic decline should be considered.

Smokers

Smoking reduces Pulmofib plasma levels via CYP1A2 induction; smoking cessation is advised before and during treatment (see Interactions).

Pediatric Patients

See Pediatric Uses.

Pulmofib is intended for long-term, continuous use as a chronic disease-modifying therapy for IPF; it is not a short-course treatment. Treatment is generally continued indefinitely to maintain the slowing of disease progression, unless intolerable side effects, disease progression beyond benefit, or physician assessment indicates otherwise. Duration is determined by the treating pulmonologist based on ongoing lung function monitoring, tolerability, and overall clinical status. Do not stop treatment abruptly without medical advice.

Pirfenidone belongs to the pyridone derivative class of small molecules and is classified therapeutically as an antifibrotic agent with antioxidant and anti-inflammatory properties, used specifically in interstitial lung disease/idiopathic pulmonary fibrosis management.

Pirfenidone acts by inhibiting multiple pathways involved in pulmonary fibrogenesis: it suppresses fibroblast proliferation and the transformation of fibroblasts into collagen-producing myofibroblasts, down-regulates production of the profibrotic cytokine TGF-β, and reduces the pro-inflammatory cytokine TNF-α. The net effect is reduced deposition of collagen and extracellular matrix in lung tissue, slowing the structural scarring and decline in lung function seen in idiopathic pulmonary fibrosis.

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The safety and efficacy of Pulmofib in pediatric patients (below 18 years) have not been established. Idiopathic pulmonary fibrosis is overwhelmingly a disease of middle-aged and older adults and is rare in children, so Pulmofib is not indicated for use in the pediatric population. Pulmofib should not be given to children outside of a formal clinical trial setting.