Interstitial Lung Disease

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.

Prescribing Notes:

• More information for health professionals on the NHS Lothian Interstitial Lung Disease Service can be found on the NHS Lothian intranet pages. See also information on shared care of medicines. In NHS Borders and NHS Fife, refer to local board prescribing resources for further information.

Nintedanib

• Nintedanib is approved for use for the treatment of other chronic fibrosing interstitial lung diseases with a progressive phenotype.
• Before initiating systemic vascular endothelial growth factor (VEGF) pathway inhibitors (nintedanib), carefully consider the risk of aneurysm and artery dissection in patients with risk factor. 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. 

Mycophenolate mofetil and Azathioprine

• Refer to local board prescribing resourced for further information.

Methotrexate 

• To avoid prescribing, dispensing, and administration errors, only the 2.5mg strength of methotrexate should be prescribed and dispensed. The patient should be advised on the dose and frequency for taking methotrexate. New measures have been implemented to prompt healthcare professionals to record the day of the week for intake and to remind patients of the dosing schedule and the risks of overdose due to continued reports of inadvertent overdose. For further advice, see MHRA Drug Safety Update, September 2020. 
• The patient should be advised to report immediately any signs of methotrexate toxicity.
• Regular monitoring of full blood count, renal function and liver function should be undertaken in line with local protocols. 
• Folic acid 5mg should be prescribed weekly, 24 hours after methotrexate to reduce toxicity, This can be increased to daily (apart from day of methotrexate) if side affects are troublesome. This option could be considered routinely to avoid the risk of nausea/mouth ulcers and the patient stopping the drug.