Drug reference
The exact mechanism of action of pirfenidone is not yet understood, but it is believed to slow Respiratory system down the progression of idiopathic pulmonary fibrosis by exerting both antifibrotic and anti-inflammatory properties.
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Treatment of mild to moderate idiopathic pulmonary fibrosis (initiated under specialist supervision) ▶BY MOUTH ▶Adult: Initially 267 mg 3 times a day for 7 days, then increased to 534 mg 3 times a day for 7 days, then increased to 801 mg 3 times a day
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DUE TO INTERACTIONS ▶Caution with concomitant use with ciprofloxacin— reduce dose of pirfenidone to 534 mg three times daily with high-dose ciprofloxacin (750 mg twice daily).
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▶Photosensitivity Avoid exposure to direct sunlight—if photosensitivity reaction or rash occurs, dose adjustment or treatment interruption may be required (consult product literature). ▶Treatment interruption If treatment is interrupted for 14 consecutive days or more, the initial 2 week titration regimen should be repeated; if treatment is interrupted for less than 14 consecutive days, the dose can be resumed at the previous daily dose without titration.
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NICE decisions ▶Pirfenidone for treating idiopathic pulmonary fibrosis (February 2018) NICE TA504 Pirfenidone (Esbriet ®) is recommended as an option for Respiratory system treating idiopathic pulmonary fibrosis in adults only if: . the patient has a forced vital capacity (FVC) between 50% and 80% predicted, and . the manufacturer provides pirfenidone with the discount agreed in the patient access scheme, and . treatment is stopped if there is evidence of disease progression (an absolute decline of 10% or more in predicted FVC within any 12-month period). Patients whose treatment was started within the NHS before this guidance was published should have the option to continue treatment, without change to their funding arrangements, until they and their NHS clinician consider it appropriate to stop. www.nice.org.uk/guidance/ta504 Scottish Medicines Consortium (SMC) decisions SMC No. 835/13 The Scottish Medicines Consortium has advised (August 2013) that pirfenidone (Esbriet ®) is accepted for restricted use within NHS Scotland for the treatment of mild to moderate idiopathic pulmonary fibrosis. Pirfenidone is restricted for use in patients with a predicted forced vital capacity less than or equal to 80%, and only whilst pirfenidone is available at the price agreed in the patient access scheme.
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→Appendix 1: pirfenidone
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▶Common or very common Appetite decreased. arthralgia. asthenia. constipation. cough. diarrhoea. dizziness. drowsiness. dyspnoea. gastrointestinal discomfort. gastrointestinal disorders. headache. hot flush. increased risk of infection. insomnia. musculoskeletal chest pain. myalgia. nausea. photosensitivity reaction. skin reactions . sunburn. taste altered. vomiting. weight decreased ▶Uncommon Angioedema ▶Rare or very rare Agranulocytosis SIDE-EFFECTS, FURTHER INFORMATION Gastrointestinal side-effects may require dose reduction or treatment interruption—consult product literature.
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Manufacturer advises avoid—no information available.
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Manufacturer advises avoid—no information available.
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Manufacturer advises caution in mild to moderate impairment (risk of increased exposure); avoid in severe impairment (no information available).
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Avoid use if eGFR less than 30 mL/minute/1.73 m2.
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▶Monitor for weight loss. ▶Test liver function before treatment, then at monthly intervals for the next 6 months, and then every 3 months thereafter; review if abnormal liver function tests—dose reduction, treatment interruption or discontinuation may be required (consult product literature).
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There can be variation in the licensing of different medicines containing the same drug. Tablet CAUTIONARY AND ADVISORY LABELS 11, 21, 25 ▶Esbriet (Roche Products Ltd) Pirfenidone 267 mg Esbriet 267mg tablets | 63 tablet P £501.92 | 252 tablet P £2,007.70 Pirfenidone 801 mg Esbriet 801mg tablets | 84 tablet P £2,007.70 Capsule CAUTIONARY AND ADVISORY LABELS 21, 25, 11 ▶Esbriet (Roche Products Ltd) Pirfenidone 267 mg Esbriet 267mg capsules | 252 capsule P £2,007.70 | 270 capsule P £2,151.10 Respiratory depression, respiratory distress syndrome and apnoea Respiratory stimulants Overview Respiratory stimulants (analeptic drugs) have a limited place in the treatment of ventilatory failure in patients with chronic obstructive pulmonary disease. They are effective only when given by intravenous injection or infusion and have a short duration of action. Their use has largely been replaced by ventilatory support including nasal intermittent positive pressure ventilation. However, occasionally when ventilatory support is contra-indicated and in patients with hypercapnic respiratory failure who are becoming drowsy or comatose, respiratory stimulants in the short term may arouse patients sufficiently to co-operate and clear their secretions. Respiratory stimulants can also be harmful in respiratory failure since they stimulate non-respiratory as well as respiratory muscles. They should only be given under expert supervision in hospital and must be combined with active physiotherapy. There is at present no oral respiratory stimulant available for long-term use in chronic respiratory failure. RESPIRATORY STIMULANTS
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Driving and skilled tasks Dizziness or malaise may affect performance of skilled tasks (e.g. driving).
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Initially 267 mg 3 times a day for 7 days, then
First-line fragment. Read the complete Indications and Dose section above.
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