Formulary

3.11 Antifibrotics

First Line
Second Line
Specialist
Hospital Only
Nintedanib
  • Capsules 100mg, 150mg

Notes

  1. NICE TA347: Nintedanib (Vargatef) in combination with docetaxel is recommended, within its marketing authorisation, as an option for treating locally advanced, metastatic or locally recurrent non small cell lung cancer of adenocarcinoma histology that has progressed after first line chemotherapy (July 2015).
  2. NICE TA379: Nintedanib (Ofev) is recommended as an option for treating idiopathic pulmonary fibrosis (January 2016), only if:
    1. the person has a forced vital capacity (FVC) between 50% and 80% of predicted
    2. the company provides nintedanib with the discount agreed in the patient access scheme and
    3. treatment is stopped if disease progresses (a confirmed decline in percent predicted FVC of 10% or more) in any 12‑month period.
  3. NICE TA747: Nintedanib (Ofev) is recommended, within its marketing authorisation, as an option for treating chronic progressive fibrosing interstitial lung diseases (PF ILD) in adults (November 2021).
  4. NICE TA864: Nintedanib (Ofev) is recommended as an option for treating idiopathic pulmonary fibrosis in adults (February 2023), only if:
    1. they have a forced vital capacity of above 80% predicted
    2. the company provides it according to the commercial arrangement.
  5. MHRA Drug Safety Update (July 2020): Systemically administered VEGF pathway inhibitors: risk of aneurysm and artery dissection
    1. Before initiating a systemic VEGF pathway inhibitor, carefully consider the risk of aneurysm and artery dissection in patients with risk factors (see Drug Safety Update for a list of risk factors)
    2. In patients who receive a systemic VEGF pathway inhibitor, reduce as far as possible any modifiable risk factors such as smoking and hypertension
    3. Monitor patients for and treat hypertension in accordance with recommendations in the SPC (click here).
Pirfenidone
  • Tablets 267mg

Notes

  1. NICE TA504: Pirfenidone for treating idiopathic pulmonary fibrosis (NHS England commissioned) (February 2018).
  2. MHRA Drug Safety Update (November 2020): Pirfenidone (Esbriet): risk of serious liver injury; updated advice on liver function testing
    1. Refer to the Drug Safety Update or manufacturer's SPC for guidance on liver function monitoring
    2. Follow new guidance on testing liver function promptly in patients who report symptoms or have clinical signs that might indicate they have liver injury and adjust the dose or discontinue treatment according to new recommendations
    3. Monitor closely for signs of toxicity if pirfenidone is being used concomitantly with inhibitors of one or more other CYP isoenzymes involved in the metabolism of pirfenidone (see table in Drug Safety Update for more information).

Other drugs

Acetylcysteine
  • Effervescent tablets 600mg

Indications

  • Idiopathic pulmonary fibrosis (NHS England commissioned) (unlicensed indication)