Conditions and Treatments

Interstitial lung disease, commonly called ILD, is a term used to describe a wide range of conditions which can cause inflammation and sometimes scarring (or fibrosis) of the lungs. The most common of these conditions are:

• Idiopathic Pulmonary fibrosis (IPF)
• Hypersensitivity Pneumonitis
• Sarcoidosis

ILD can also be associated with rheumatological or connective tissue diseases such as rheumatoid arthritis. There are many other rarer conditions causing ILD, e.g., drug induced ILD, and cystic lung disease. 

- Asthma and lung UK - find my lung condition

Diagnosis
Patients are referred to the ILD clinic when a GP or other hospital specialist suspects an underlying ILD . You will be assessed in the clinic and further tests may be organised, such as lung function tests and a CT scan of your lungs. All new patients will be discussed in a specialist meeting called the multidisciplinary team (MDT) meeting, in order to reach a consensus on diagnosis. The MDT may recommend further tests or treatments. 

Please see our ILD Outpatient clinic section for further information on what to expect when coming to an outpatient clinic appointment. 

Treatments
The treatment of ILD is dependent on the underlying cause. Medication treatment(s) can be broadly separated into two categories:

• Medication to treat inflammation (which is potentially reversible) example.g., steroids or immunosuppression medications
• Anti-fibrotic medication - to slow development of scarring in the lungs.
   
• Immunosuppression
  There are many different medications which may be used to try and treat inflammation in the lungs. These vary depending on the underlying condition or cause, as some medications may work better for particular conditions. For example, if you have an underlying connective tissue disease such as rheumatoid arthritis, we work alongside your rheumatologist to find a medication that helps both your joints and the lungs together. We will also consider your other health conditions and treatments to weigh up which medication(s) may be best. There may be more than one medication suitable so you will be given information to read before making a decision. You will have the opportunity to discuss which medication suits you best with guidance from a nurse specialist.
  
  When starting on new medications, you will be reviewed in the drug monitoring clinic by a nurse specialist. This is so we can monitor how you are getting on with the new medication, check for any side effects and also monitor blood tests. The nurse specialist will explain this process when you are seen in the clinic. 
  
  Examples of commonly used immunosuppression medications in ILD include:
  - Steroids / prednisolone
  - Mycophenolate mofetil
  - Azathioprine
  - Methotrexate
  - Rituximab 
  - Infliximab
  
  These medications are initiated by hospital specialists, but some can be prescribed by the GP after a period of monitoring under the care of the ILD team (referred to as a shared-care protocol). 
•  Anti-fibrotic medication
  There are currently two anti-fibrotic medications available, Nintedanib and Pirfenidone. These can be used in idiopathic pulmonary fibrosis (IPF) and in certain progressive fibrotic interstitial lung diseases if recommended by the ILD MDT.
  
  Nintedanib
   Video about nintedanib therapy
  
  Pirfenidone
  Video about Pirfenidone therapy
  
  Further information about antifibrotic information can be found on action for pulmonary fibrosis website by clicking here.
  
  Antifibrotic medications can only be prescribed by the hospital specialist. 

Self-Management
There are many things you can do to help manage your symptoms and slow progression of your disease. Prior to developing this website, we asked our ILD patients in Cardiff what further information they would like to know about their condition. The most frequent request was further information on self-management. We have created a dedicated section of our webpage on ILD self-management, which can be found here. This section of the website contains information about diet, exercise, vaccination, smoking cessation, local support groups, online workshops and classes.

Pulmonary rehabilitation (PR)
Patients with a chronic lung condition can be referred by the respiratory specialist team to participate in pulmonary rehabilitation. This is a 6-week multi-disciplinary programme, where you attend 3 times a week and each session is made up of an educational element, exercise and relaxation, based on recognised rehabilitation standards. The Pulmonary Rehabilitation Unit is based in the University hospital of Llandough. The team consists of a Respiratory Consultant, Specialist Interstitial Lung Disease (ILD) Nurse, Co-ordinator, Physiotherapist, Generic Technicians, Dietician and Occupational Therapist.

Evidence shows that accessing PR improves people’s ability to walk further, helps them feel less tired and breathless when carrying out day-to-day activities. Around 90% per cent of patients who complete a PR programme have higher activity and exercise levels, and report an improved quality of life. PR has been shown to support better self-management and reduction in exacerbations, reduction in numbers of acute and emergency admissions. 

Please ask a member of the ILD team if you would like further information or if you would like to discuss referral to pulmonary rehabilitation.

Oxygen Therapy
Not everyone with ILD will need oxygen therapy. Some patients may need oxygen therapy in the future if their disease progresses. We will continue to assess this when you are seen in the clinic. Unfortunately, oxygen does not specifically help with the symptom of shortness of breath alone and is only a treatment that is used when the oxygen levels are persistently low. For more information about breathlessness, please click here or see the ILD self-management section of our website. For more information about when oxygen might be used and how it is used, please click here.

Supportive Care Team
The supportive care team works very closely alongside the ILD team in Cardiff. The supportive care team is a specialised multidisciplinary team who work with patients to help manage symptoms. The ILD team may suggest referring you to see the supportive care team if they feel this may be beneficial for you.

Lung transplant
A lung transplant is an operation to remove and replace a diseased lung with a healthy human lung from a donor.  A donor is usually a person who's died, but in very rare cases a section of lung can be taken from a living donor.

A lung transplant may be considered if an individual has advanced lung disease that is not responding to other methods of treatment. 

The demand for lung transplants is far greater than the available supply of donated lungs. This means a transplant will only be carried out if it's thought there's a relatively good chance of it being successful.

For more information on lung transplant, please see the links below.
- NHS lung transplant
- Lung transplant action for pulmonary fibrosis

Palliative Care 
Palliative Care is available to patients whose disease or illness is no longer responsive to curative treatment.  For further information about palliative care services in Cardiff, please see our palliative care team page. 
- Caring for someone with a long-term lung condition at the end of life - Marie Curie
- Managing breathlessness  booklet - Marie Curie