Table 3.
Summary of recommendations for the prevention and management of pirfenidone-related AEs
| General approaches to preventing and managing pirfenidone-related AEs |
| Pirfenidone administration and dose modifications |
| Pirfenidone capsules (267 mg) should be taken individually, over the course of a meal: fine tuning should take into account cultural dietary habits |
| If nausea is experienced in the morning, the morning dose may be delayed or reduced |
| A maximum of three capsules (801 mg) should be taken with the main meal of the day, while fewer [1 or 2 capsules (267–534 mg)] may be taken with lighter meals |
| Upon treatment initiation, the up-titration scheme can be extended to 4 weeks until the daily recommended dose is reached |
| Temporary treatment interruptions should be considered if symptoms do not resolve following dose reduction |
| Following dose interruption, pirfenidone could be re-introduced with a slower re-escalation scheme to the full dose |
| All treatment-related decisions should be made following discussion with the patient and with the aim of balancing quality of life and efficacy benefits |
| Additional measures to manage GI AEs |
| Prokinetic agents may help mitigate GI AEs |
| Additional measures to prevention and manage skin AEs |
| Prevention of photosensitivity reactions |
| Change behavior to avoid sun exposure: |
| • Seek to avoid sun exposure as much as possible, especially at mid-day, in the late afternoon, and during seasonal high UV periods. Remember that the sun’s UVA component can penetrate clouds, clothing, and car windows |
| • Avoid sun exposure for a few hours after the meal at which pirfenidone was administered |
| Protect skin from the sun with appropriate clothing: use of wide-brimmed hats, sunglasses, long-sleeve shirts, and trousers is recommended, as are gloves for driving and outdoor activities |
| Protect skin from the sun with sunscreen: frequent and thorough application of a broad-spectrum SPF50 sunscreen with both UVA and UVB protection is mandatory |
| Management of skin rashes |
| In case of rashes, pirfenidone dose should be reduced. If rashes still persist after 7 days, pirfenidone therapy should be discontinued for 15 days and may be slowly re-introduced once symptoms have resolved |
| If rashes are due to an allergic reaction, pirfenidone therapy should be permanently discontinued |
| In cases of severe photosensitivity reactions, pirfenidone should be discontinued and replaced with prednisone 25 mg/day for 7–10 days. After disappearance of the skin reaction, pirfenidone may be re-introduced following a very slow re-escalation |
| Education |
| Patient (and spousal) education should be undertaken at several levels (physician, nurse, and pharmacist) and should be supported by existing educational materials (e.g., patient information leaflet) |
| Physicians play a central role in patient education and in providing best advice by considering the patient’s clinical history, potential outdoor occupations/hobbies/sport, or possible concomitant treatment with other photosensitizing drugs |
AE adverse event, GI gastrointestinal, SPF sun protection factor, UV ultraviolet