What is Connective Tissue Disease?
Connective tissue disease is a broad term to describe a group of diseases caused by autoimmune conditions such as rheumatoid arthritis, systemic scleroderma, myositis, or lupus.
What is Interstitial Lung Disease (ILD)?
The interstitium is the area of tissue that lies between the air sacs and the blood vessels of your lungs and allows oxygen to travel from your lungs and into your body. When this tissue is damaged, it can cause Interstitial Lung Disease, or ILD. This is a broad umbrella term for over 200 different types of diseases that can affect the lung tissue.
There are 4 different types of ILD:
- Idiopathic – when the cause is Unknown. This includes Idiopathic Pulmonary Fibrosis (IPF)
- ILD with a known cause – This can be due to drugs, occupational exposure, or connective tissue diseases (e.g., systemic sclerosis, rheumatoid arthritis, lupus, inflammatory myopathies)
- Granulomatous – includes sarcoidosis or hypersensitivity pneumonitis
- Miscellaneous
Symptoms
CTD-ILD can affect all parts of the respiratory system, including the lung tissue, airways, lining, muscles, and blood vessels and cause symptoms such as cough and breathlessness
As CTD-ILD can affect many systems of the body, the condition can also cause symptoms such as muscle weakness, joint pain, rashes, thickening, fingertip ulcers, dry eyes and mouth, chest pain, leg swelling, reflux, bowel problems, weight loss, fatigue or fevers.
Healthcare Journey
Diagnosis
If CTD-ILD is suspected, you will usually be referred by your GP or rheumatologist to a respiratory specialist or an ILD expert. As diagnosis can be complicated, a multidisciplinary team will often be involved which may consist of respiratory specialists, radiologists, and sometimes a rheumatologist.
A number of tools are used to diagnose CTD-ILD, such as:
- blood tests
- a lung function or breathing test
- an echocardiogram
Depending on the patient, additional specialised tests many be required, including:
- an MRI of your muscles
- a close-up photograph of your nail bed called a nailfold capillaroscopy
- a bronchoscopy, which is a camera test to look for infection or inflammation in the lungs
- a biopsy of the lungs or other places such as skin or muscles
Treatment
When looking at treatment, it is important to look at what the treatment goals are. Your specialists could be looking to maintain stability, reverse inflammation, slow the progression of scarring or, in rare cases, ‘rescue’ therapy will be used after a flare-up.
Not everyone with CTD-ILD will need treatment. There are several factors that your treating healthcare team will look at when determining your treatment, including:
- Severity – based on your symptoms, CT results and lung function
- Disease progress – is your condition stable or getting worse?
- Risk factors – your age and type of CTD
- Patient factors – symptoms, comorbidities, and your wishes
- Other factors – infections, heart issues, fertility and/or past treatments
Specific treatments vary significantly for everyone, but may include:
- Steroids
- Immuno-modifying therapies
- Anti-fibrotic medications
- Symptom control
- Oxygen therapy
Non-pharmacological therapies are also important for people living with CTD-ILD, including:
- Pulmonary rehabilitation
- Psychological support
- Good nutrition
- Welfare
- Smoking cessation
Your healthcare team will also be thinking about the future of your condition by monitoring:
- Complications and flare-ups
- When to start and stop treatments
- Clinical trials
- In rare cases, lung transplantation
The content of this blog was based on the 2022 ‘Introduction to Connective Tissue Disease-associated Interstitial Lung Disease’ webinar by Dr Adelle Jee, Respiratory and Sleep Physician and CTD-ILD consumer advocate, Tania Hyde.