Interstitial Lung Disease

What is Interstitial Lung Disease?

Interstitial lung diseases (ILDs) are a group of approximately 200 conditions which cause inflammation and scarring (fibrosis) of the lung. The conditions may have a known cause, for example smoking or an exposure at work, or may have no known cause (idiopathic).

The most common condition we treat is idiopathic pulmonary fibrosis (IPF). This is scarring of the lung of unknown cause. This is a chronic condition which typically develops over years and progresses, causing breathlessness and cough. The rate at which IPF progresses varies a lot from person to person. We have medications, called antifibrotics, which can slow the rate of progression in IPF.

Other conditions we treat include sarcoidosis (an inflammatory disorder affecting the whole body), lung diseases caused by connective tissue disease (such as rheumatoid arthritis and systemic sclerosis), hypersensitivity pneumonitis (inflammation and scarring caused by breathing something in), occupational lung diseases (such as asbestosis) and cystic lung diseases.

Our Team

The ILD team is made up of doctors, including consultants, clinical fellows and training registrars, specialist nurses, specialist pharmacists and our administrative team.  During your first consultation with the team, you are likely to see a consultant or clinical fellow and a specialist nurse.  Follow-up consultations may be with doctors, specialist nurses or pharmacists, depending on the treatments you will be taking.

We run clinics at Wythenshawe Hospital (North West Lung Centre, Respiratory Day Unit and Main Outpatients) and Trafford General (Main outpatients).

We have close ties with the local hospitals that refer patients to us and we will try to facilitate follow-up closer to home through the local teams where possible.

We run several specialist and joint clinics.  These include:

• Twice-monthly joint Rheumatology-ILD clinic which includes Rheumatology and Respiratory consultants
• Monthly Renal-Respiratory clinic which includes a Renal (kidney) and Respiratory consultant. Both these clinics are run at Withington Hospital.
• Monthly Familial Pulmonary Fibrosis clinic through which we offer genetic testing.

Possible treatment options

We use two broad types of treatment in ILD.

In patients where we feel there is lung inflammation, we will often prescribe steroid medication (prednisolone) and immunosuppressive medications (mycophenolate mofetil, azathioprine, methotrexate).

• Immunosuppressive medication requires blood monitoring. Our specialist pharmacy team will offer medication counselling prior to starting immunosuppressive medication and will provide the initial prescription.
• We will liaise with your GP practice to arrange an ongoing prescription and blood monitoring.

In patients where we feel there is a progressive scarring lung disease (pulmonary fibrosis), we will often prescribe antifibrotic (anti-scarring) medication.

• Currently we have two antifibrotic medications available, pirfenidone and nintedanib.
• These are specialist medications which are currently only available through specialist prescribing centres like Wythenshawe hospital.
• There are criteria for which patients are eligible to receive these medications, and we will assess this when we review you in the clinic and at our MDT meeting. We need to receive approval prior to commencing these medications.
• Antifibrotic medications require blood monitoring.  If we plan to start you on antifibrotic medication we will offer medication counselling through our specialist nursing team.  We deliver counselling via face-to-face individual of group sessions and via telephone appointments.

What tests might I have?

During your initial work-up for ILD, we will frequently arrange blood tests (typically to look for connective tissue disease), perform lung function tests and arrange CT scans of the chest. Some of these tests may have been done at your local referring hospital but we may need to repeat tests to look for changes.

On occasion we will recommend a bronchoscopy test (camera test into the lungs). We arrange this to wash out lung cells which can help us make a diagnosis or rule out infection prior to starting treatment.

In some very rare cases, we refer patients to the Thoracic (lung) Surgery team for a lung biopsy via video-assisted thoracic surgery (VATS). This is an operation to remove a sample of lung to help make a diagnosis. This is reserved for cases where the diagnosis is unclear, and the treatment choice is dependent on this information.

Following your initial consultation, it is likely that we will need to discuss your case at our multidisciplinary team (MDT) meeting to confirm a diagnosis and obtain approval for some specialist medications. This is an internal meeting held between the doctors, nurses and pharmacists where we discuss cases.

Support Groups

There are several fantastic support groups in the region for patients with pulmonary fibrosis and their carers. These include:

• Tameside Pulmonary Fibrosis Support Group
• Bolton Pulmonary Fibrosis Support Group
• Salford ILD Support Group
• Wigan ILD Support Group
• Manchester ILD Support Group.

Details for these support groups can be found on the Action for Pulmonary Fibrosis website.

Contact Details

The ILD service is an outpatient service.  If you are unwell and need urgent assessment, we recommend contacting your GP or attending your local urgent care service.

We run several hotline numbers for issues related to your condition.  These lines are not always staffed at the time.  We encourage patients to leave a telephone message with your name, hospital or NHS number and return contact number.  We aim to return your call as soon as possible.

Please use the following contact details depending on the nature of your enquiries:

For appointment and general enquiries:

ILD Admin team

• Telephone: 0161 291 5054
• Email: ildsecmanc@mft.nhs.uk

For issues related to antifibrotic medications and other nursing enquiries:

ILD Nursing team

• Telephone: 0161 291 4936
• Email: ildnursemanc@mft.nhs.uk

For issues related to immunosuppression medication and other pharmacy enquiries:

ILD Pharmacy team

• Telephone: 0161 291 4273
• Email: ildpharmacymanc@mft.nhs.uk

Research

We are at the cutting edge of ILD research and offer patients a wide range of opportunities to participate in clinical research.

We offer the latest drug trials in ILD which are delivered through the Clinical Research Facility at Wythenshawe Hospital. We also perform lots of other research studies looking at various aspects of ILD including new diagnostic tests and investigating the origins of disease.

We will let you know about research opportunities at your clinic appointments.

Referral information for GPs

Referrals to the ILD service from healthcare providers can be made via the admin team on ildsecmanc@mft.nhs.uk.

We run educational sessions for healthcare professionals.  This includes our annual Manchester ILD Course which is a 2-day national teaching program which we have been running since 2017.

For details of our educational events please see our Eventbrite page: