05 February 2025
In this month’s blog Clare Bennett (Rheumatology Specialist Nurse) spoke to ILD specialist nurse, Sister Sarah Agnew based at Liverpool University Hospitals. Their discussion focussed on ILD related to rheumatic disease.
What is the role of an ILD nurse?
An ILD nurse looks after patients with any of the over 200 ILDs and their families from diagnosis until in most cases, end of life. We manage drug treatment, symptoms, diet and nutrition, oxygen, palliative care and emotional support. We look at the holistic needs of the patients and coordinate referrals to other appropriate support services.
How do you think Rheumatology and ILD nurses can work together?
It is really important that both specialities work together to the benefit of the patient. We need to support each other with the different aspects of the patient’s symptoms so we are optimising their symptom management.
What are the challenges with antifibrotics in patients with ILD-related to rheumatic disease?
This group of ILD patients will usually have had a chronic condition for a while and are often on immunosuppression. Adding in nintedanib adds to their tablet burden and can also make it more challenging to tolerate both medications due to the side effect profile of both drugs.
How do you explain the role of antifibrotics to a patient?
It can be challenging to explain the role of antifibrotics. They do not improve patients’ symptoms; they cannot reverse the scarring that is already there. Analogies we use are “once an egg is hard boiled, you can’t change it back to a soft boiled egg”; that’s the same with lung scarring.
They are an investment in the future. We would expect someone taking antifibrotics to have had less progression in 12 months than someone not on them. They have the potential to slow the progression by up to 50% over 12 months but it will still progress.
When do you consider stopping an antifibrotic?
Generally patients can stay on treatment long term. We would consider stopping the drug if the patient is getting significant side effects that we haven’t been able to manage e.g. diarrhoea, poor appetite and weight loss. It may require a discussion about the impact on someone’s quality of life.
Sometimes you may pause the drug with a view to restarting once the symptoms are under control. They do usually require active management.
We also need to have conversations with patients when their condition progress and they are approaching the end of their life. This can be challenging and needs to be done carefully to ensure patients don’t feel we are extinguishing all hope, we are changing the focus of the hope.
If immunosuppression has been optimised, are there other treatments available?
Unfortunately, there is currently only 1 antifibrotic available for this group of ILD patients. This may change in the future.
If patients are unable to tolerate antifibrotics then discussions around best supportive care and optimising symptom management is key.
Can you give us any best practice pointers for this group of patients?
Patients should have an annual full lung function test to assess for progression as a minimum. Patients should also have an annual ECHO to assess for pulmonary hypertension. They should also be referred for pulmonary rehabilitation, if appropriate.
When we see a patient in the rheumatology clinic with ILD-any top tips?
Check oxygen saturations after walking the patient into your clinic room. Patients with ILD are more likely to experience a drop in oxygen levels with exertion, even if their resting levels are normal.
If they have Raynaud’s then the finger probe may not be accurate, an ear probe will be better.
If your patient reports increased shortness of breath, especially on exertion, along with either a drop in forced vital capacity on lung function tests or changes on a CT scan, they would meet the criteria for antifibrotic therapy.
Have a discussion with your local ILD nurse, we are always happy to discuss patients and advise and support the rheumatology team (and we need your advice as well!).
__