1- What is Pirfenzi?

Pirfenzi contains the active pharmaceutical ingredient pirfenidone approved to treat patients with idiopathic pulmonary fibrosis (IPF).

2- Mechanism of Action

Pirfenidone mechanism of action is still unknown. However, pirfenidone may reduce progression and growth of fibroblasts through its antifibrotic effects. It also has anti-inflammatory effects due to reduction of progression of inflammatory cells.

3- Indications

Pirfenidone is used in treating patients with idiopathic pulmonary fibrosis (IPF).

4- Dosage and Administration

The usual dose of pirfenidone is three 267 mg capsules three times a day (in total nine 267 mg capsules a day) with or just after food or a meals.

*Reminder:

1.The recommended dose of drug is to be given gradually to reach nine capsules a day according to the table below:

Should the treatment with pirfenidone be stopped for longer than two weeks and the doctor may plan to restart the drug, the aforesaid table should be observed. In case of major adverse effects including indigestion, sunlight hypersensitivity reactions and rash, the doctor may decide to lower the dose or temporarily discontinue the treatment.

2.Avoid taking grapefruit while under treatment with this drug.

3.Due to its reducing effects on blood levels of pirfenidone, smoking while under treatment with pirfenidone is not at all recommended.

4.Swallow this medicine whole. Do not chew or crush.

5- Form and strength

Pirfenzi is available in 267 mg capsules for oral administration.

6- Warnings and Precautions

• Effects on CNS:

Pirfenidone may cause dizziness or tired feeling. Therefore, take caution in doing things that need full alertness like driving or working with machinery. Taking the drug with or just after food or a meals can reduce dizziness. In case of worsening symptoms or no improvement, your doctor may lower the dose or discontinue treatment.

• Increased liver enzymes:

There are reports of more than three times increased liver enzymes in patients taking pirfenidone, and the doctor may decide to lower the dose or discontinue treatment. Generally, it is recommended to check liver function prior to treatment, every month for the next six months and every three months after that.

• Hypersensitivity reactions to sunlight and rash:

The occurrence of hypersensitivity reactions to sunlight in patients taking pirfenidone is higher than others. They should avoid sun as much as possible and use sunscreens (sun protection factor (SPF) greater than or equal to 50) and should wear a hat and clothes to minimize sunlight exposure. In case of hypersensitivity reactions to sunlight and rash, the doctor may decide to lower the dose or discontinue treatment.

• Indigestion:

Digestion problems such as nausea, vomiting, diarrhea and indigestion have been reported in pirfenidone users. They occur more in the first three months of starting it and become less with time. The doctor may decide to lower the dose or discontinue treatment in severe cases.

• Weight loss:

Weight and appetite loss have been reported in pirfenidone users. To this effect, the patient weight should be monitored while under treatment.

7- Contraindications

• Hypersensitivity to the active substance or to any formulation of pirfenidone.
• History of angioedema with pirfenidone.
• Concomitant use of fluvoxamine.
• Severe hepatic impairment or end stage liver disease.
• Severe renal impairment (Creatinine Clearance less than 30 milliliter per minute) or end stage renal disease requiring dialysis.

8- Adverse effects

Very common (greater than or equal to 10 percent of patient population)

• CNS: tiredness, headache, dizziness
• Skin: rash, sensitivity to sunlight
• Digestive: nausea, diarrhea, stomach pain, indigestion, appetite loss, vomiting, heartburn
• Respiratory: upper respiratory tract infection, sinusitis

Common (1 to 10 percent of patient population)

• CNS: insomnia (sleep problems), non-cardiac chest pain, drowsiness
• Skin: itch, macula, sunburn, erythema
• Endocrine and metabolic: weight loss, hot flashes
• Digestion: swelling of the abdomen, appetite loss, bloating, taste alteration, flatulence
• Liver: increased liver enzymes
• Neuro-musculoskeletal: muscle pain, lethargy

9- Drug interactions

• Since pirfenidone can interact with other drugs, make sure to inform your doctor of the list of drugs you are currently taking before using it. Some of pirfenidone drug interactions are as follows:
• Concurrent use of pirfenidone and ciprofloxacin increases levels of pirfenidone.
• Concurrent use of pirfenidone and CYP1A2 enzyme inhibitors like fluvoxamine increases levels of pirfenidone
• Concurrent use of pirfenidone and CYP1A2 enzyme inducers like omeprazole decreases levels of pirfenidone

10- Use in Specific Populations

• Pregnancy

There is still not enough information concerning the use of pirfenidone in pregnancy. In animal studies the incidence of fetal harm has been observed. Therefore, use this drug in pregnancy only with your doctor discretion and decision.

• If you are pregnant or want to become pregnant, tell your doctor about your condition and decision before taking this medicine.

• Nursing

At present, there is no established information regarding the amount of pirfenidone excreted in mother milk. To this effect, decision to continue using the drug or to stop nursing should be made by the doctor.

• Pediatrics

The safety and efficacy of pirfenidone in individuals under 18 have so far not been established.

• Geriatrics

Given the studies conducted, safety and efficacy of pirfenidone in the elderly are the same as other individuals and there is no need for dose titration in these patients.

• Patients with liver disorders

-In patients with moderate liver problems, titrating the dose is not needed and caution should be taken while using the drug in such patients.

-Using this drug in patients with severe liver problems is not recommended.

-In the case of increased liver enzymes ALT and AST to more than three times the normal levels (with no increase in bilirubin levels) other causes and drugs that may lead to increased liver enzymes should be stopped. Given the clinical symptoms, the doctor should decide whether to continue treatment with pirfenidone, lower the dose, or temporarily discontinue treatment. In the case of restarting the drug, the dose should gradually be increased and liver enzymes are regularly checked.

-In case ALT and AST levels increase to more than three times the normal levels along with increased bilirubin levels, treatment should be discontinued.

-Discontinue treatment in case ALT and AST levels rise to more than five times the normal levels.

• Patients with kidney problems

-In general, there is no need for dose titration in patients with moderate kidney problems. Caution should be taken in using this drug in such patients and lower the dose or discontinue treatment with the doctor’s discretion.

-In patients with severe kidney problems and in need of dialysis, using this drug is not recommended.

11- Drug Overdose

In case of an overdose, contact healthcare providers or poison control centers so that based on current symptoms the necessary measures can be taken.

12- Missed dose

In case you have not taken this drug for more than two consecutive weeks, your doctor may restart treatment by gradually increasing the dose to reach the desired dose in two weeks.

– In case using the drug was stopped for fewer than two weeks, there is no need for gradual increase of doses and it can be taken as previously scheduled.

*Keep out of reach and sight of children.

13-  Storage

Store below 30˚C. Keep the container tightly closed. Keep the drug out of the sight and reach of children.

14- D.Nurse-Patient Helpline Team

You can contact Drugs Expert Consultation Center at 0935 220 3041-4 to get answers to your inquiries and also get to know nursing support centers in your city.Expert Consultation Center is always ready to answer the questions of our valued customers.