Pulmonary fibrosis

Dose as per specialist.

Prescribing Notes:

Before initiating systemic vascular endothelial growth factor (VEGF) pathway inhibitors (nintedanib), carefully consider the risk of aneurysm and artery dissection in patients with risk factors. In patients who receive a systemic VEGF pathway inhibitor, reduce as far as possible any modifiable risk factors such as hypertension. For further advice see MHRA Drug Safety Update, July 2020.
Pirfenidone is approved for the treatment of mild to moderate idiopathic pulmonary fibrosis for use in patients with a predicted forced vital capacity less than or equal to 80%.
Serious liver injury has been reported in patients treated with pirfenidone. For further advice on liver function testing see MHRA Drug Safety Update, November 2020.
Patients should be monitored on a regular basis. The need for ongoing treatment should be reviewed after 3 months and 12 months.
Due to different formulations of nintedanib being licensed for different indications prescribe by brand name only.

20/11/2023

Addition of Ofev (SMC 2513), ERFC August 23.

12/12/2022

Switch to generic pirfenidone, ERWG Nov 22.

16/02/2022

East Region Formulary content agreed.

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Dose as per specialist.

More information for health professionals on the NHS Lothian Interstitial Lung Disease Service can be found on the NHS Lothian intranet pages.
Nintedanib is approved for use for the treatment of other chronic fibrosing interstitial lung diseases with a progressive phenotype.

12/12/2022

Prednisolone and mycophenolate mofetil added, ERWG Nov 22.

16/02/2022

East Region Formulary content agreed.