Connective Tissue Disease-Associated ILD (CTD-ILD)

Connective tissue diseases — including systemic sclerosis, rheumatoid arthritis, the inflammatory myopathies, Sjögren's syndrome and mixed connective tissue disease — can involve the lungs as well as the joints, skin and muscles, resulting in interstitial lung disease (CTD-ILD). Dr Mackintosh works closely with rheumatologists across Brisbane and Queensland to assess and manage CTD-ILD.

A joint-care approach

Assessment typically includes baseline and serial pulmonary function testing, HRCT imaging and relevant autoimmune serology, reviewed together with the treating rheumatologist and, for complex presentations, at multidisciplinary team meeting. Dr Mackintosh has particular research and clinical interest across the range of CTD-ILDs, including systemic sclerosis-associated ILD — the subject of his review Systemic Sclerosis Associated Interstitial Lung Disease: A Comprehensive Overview (Semin Respir Crit Care Med, 2019) — and rheumatoid arthritis-associated ILD, having contributed as an investigator to the TRAIL1 trial of pirfenidone in RA-ILD (Lancet Respir Med, 2023).

Treatment

Management depends on the pattern and behaviour of disease identified, and may include immunosuppression directed at the underlying connective tissue disease, antifibrotic therapy where fibrosis is progressive despite treatment (see progressive pulmonary fibrosis), and, in selected severe cases, more intensive treatment options. Coordinated respiratory and rheumatology follow-up is important throughout.

Patient information

Connective Tissue Disease-associated Interstitial Lung Disease (CTD-ILD) and Scleroderma with Interstitial Lung Disease — fact sheets, Lung Foundation Australia and the Centre of Research Excellence in Pulmonary Fibrosis.
Antisynthetase Syndrome — booklet on this myositis-associated form of CTD-ILD, Centre of Research Excellence in Pulmonary Fibrosis.
Understanding & Managing Scleroderma — booklet, Scleroderma Australia.
Scleroderma, Rheumatoid Arthritis and Sjögren's Syndrome information sheets — Arthritis Australia.

Frequently asked questions

What is connective tissue disease-associated ILD?

CTD-ILD is interstitial lung disease occurring in the context of an autoimmune connective tissue disease, such as systemic sclerosis, rheumatoid arthritis, the inflammatory myopathies, Sjögren's syndrome or mixed connective tissue disease. Not everyone with a connective tissue disease develops lung involvement.

Should I be screened for lung involvement if I have a connective tissue disease?

Screening depends on the specific condition and risk factors. Baseline HRCT and pulmonary function testing is often recommended in higher-risk conditions such as systemic sclerosis, even without respiratory symptoms, since early lung involvement can be silent.

How is CTD-ILD treated?

Treatment depends on the pattern and behaviour of disease, and may include immunosuppression targeting the underlying connective tissue disease, antifibrotic therapy where fibrosis is progressive, and in selected severe cases, more intensive treatment such as autologous stem cell transplantation.

For referring clinicians

Dr Mackintosh welcomes referrals from rheumatologists and GPs for respiratory assessment of patients with known or suspected connective tissue disease, including baseline screening in high-risk conditions such as systemic sclerosis.

Referral information