Pirfinex

Indications

Pirfenidone is used to treat idiopathic pulmonary fibrosis (IPF), a long-term lung disease in which the lung tissue becomes thickened and scarred for no known reason, making breathing gradually harder. It slows down the loss of lung function. It does not cure the disease or reverse scarring that has already happened. It is started by a chest specialist.

How It Works

Pirfenidone reduces the production of substances that drive scarring and inflammation in the lungs (such as TGF-beta), so new scar tissue forms more slowly.

Dosage and Administration

As prescribed by the specialist. The dose is built up slowly over about 2 weeks to reduce stomach upset and is taken three times a day. Always take it with food or straight after a meal, as this greatly reduces nausea and dizziness. Swallow tablets or capsules whole with water. If you miss treatment for 14 days or more, do not restart at the full dose; ask your doctor, because the dose needs to be built up again. The doctor may lower the dose or pause treatment if side effects occur.

Interactions

Do not take with Fluvoxamine (an antidepressant), which raises pirfenidone levels strongly. Ciprofloxacin, Enoxacin and some other medicines that block the liver enzyme CYP1A2 also raise levels, so the dose may need to be lowered. Smoking and medicines such as Rifampicin and Omeprazole can lower levels. Avoid grapefruit juice. Tell your doctor about all medicines, including herbal products.

Contraindications

Allergy to pirfenidone or any ingredient; a history of angioedema (sudden deep swelling of the face, lips or throat) with pirfenidone; severe liver disease; severe kidney disease or dialysis; and use together with Fluvoxamine.

Side Effects

Common: nausea, indigestion, loss of appetite, weight loss, tiredness, dizziness, diarrhoea, and rash or sunburn after sun exposure (photosensitivity). Pirfenidone can raise liver enzymes, so regular blood tests are needed, usually monthly for the first 6 months and then every 3 months.

Get urgent medical help if you have swelling of the face, lips, tongue or throat, difficulty breathing, a severe or blistering rash or peeling skin, or signs of liver problems such as yellow skin or eyes, dark urine, or pain in the upper right side of the stomach.

Pregnancy and Lactation

There is little information on use in pregnancy, so it is best avoided unless the specialist decides it is necessary. It is not known whether it passes into breast milk; breastfeeding is not recommended during treatment.

Precautions and Warnings

Avoid direct sunlight and sunlamps: wear a high-factor sunscreen (SPF 50), a hat and long sleeves every day. Stop smoking, as it reduces the effect of the medicine. Tell your doctor if you have liver or kidney problems. It can cause dizziness and tiredness, so take care when driving. Keep all blood test appointments.

Overdose

Taking too much may increase nausea, dizziness, stomach upset and liver strain. Contact a doctor or go to hospital, taking the pack with you.

Therapeutic Class

Antifibrotic agent (medicine for pulmonary fibrosis).

Storage

Store below 30°C in a dry place, away from light and out of the reach of children.