Connective tissue disease-associated interstitial lung disease (CTD-ILD)

The connective tissue disease-associated interstitial lung disease (CTD-ILD) fact sheet has been designed to provide an understanding of the causes, symptoms and diagnosis of CTD-ILD. The resource provides information to support with symptom management and provides an overview of the treatments available for CTD-ILD.

CTD-ILD is a group of interstitial lung diseases (ILD) caused by connective tissue diseases such as systemic sclerosis, rheumatoid arthritis, and inflammatory myopathies. These diseases affect body parts like joints and skin but can also involve other organs. ILD is characterized by thickening or scarring of lung tissue, and not everyone with connective tissue disease will develop CTD-ILD.

Symptoms of CTD-ILD vary widely and can include fatigue, weight loss, fever, breathlessness, persistent dry cough, muscle weakness, joint pain, Raynaud’s phenomenon, skin rashes, fingertip ulcers, dry eyes, and gastrointestinal issues like reflux and swallowing problems.

Diagnosis involves a multidisciplinary approach, including blood tests, high-resolution CT scans, lung function tests, echocardiograms, and possibly bronchoscopy or biopsy. Diagnosis can be challenging and may require multiple visits to healthcare providers.

Treatment options depend on individual circumstances and may include immunosuppressive medications (e.g., prednisolone, mycophenolate) and anti-fibrotic medications (e.g., nintedanib). Non-pharmacological treatments such as pulmonary rehabilitation, oxygen therapy, psychological support, nutrition, and smoking cessation are also important. Treatment aims to stabilise or reverse the disease or slow its progression.

Management of CTD-ILD requires a team approach, involving GPs, respiratory specialists, rheumatologists, cardiologists, pharmacists, physiotherapists, psychologists, and specialist nurses. Patients are encouraged to maintain a healthy lifestyle including – keep vaccinations up to date, exercise regularly and maintain a healthy diet. It is also important to stay connected with your healthcare team and seek support from family, friends, and support groups. If you or someone you know is affected by CTD-ILD, reaching out to the Lung Foundation Australia can provide valuable support and information. They are dedicated to helping patients navigate their journey and access the best possible care. For more information and support, individuals can contact the Lung Foundation Australia’s Information and Support Centre at 1800 654 301 or visit their website.