ILD - Center for Interstitial Lung Disease

What is Connective Tissue Disease-Related ILD?

Connective tissue disease related to interstitial lung disease (CTD-ILD), now known as systemic autoimmune rheumatic disease-ILD (SARD-ILD), is a lung condition that affects patients with a connective tissue disease. Common connective tissue diseases include:

• Mixed connective tissue disease (MCTD)
• Polymyositis/dermatomyositis and the anti-synthetase syndrome (AS)
• Rheumatoid arthritis
• Scleroderma/Systemic Sclerosis
• Sjoren’s disease
• Systemic lupus erythematosus (SLE)

Connective tissue disease-related-- ILD causes inflammation or scarring (fibrosis) of the lungs and can prevent oxygen from being absorbed in the lungs. Each type of connective tissue disease may affect the lungs in different ways.

Patients are often diagnosed with a connective tissue disease first and develop CTD-ILD later. However, some patients may be diagnosed with ILD first before developing their connective tissue disease.

Signs and Symptoms

Although the lungs may be affected in 60 to 80 percent of connective tissue disease patients, many people may have mild symptoms at first.

Symptoms may include:

• Breathlessness or shortness of breath
• Crackling sound in lungs when examined with a stethoscope
• Dry, persistent cough
• Fatigue and weakness

Symptoms get worse as the lungs develop more scar tissue. Shortness of breath may occur only during exercise. As the disease progresses, patients may become breathless while doing everyday activities such as getting dressed, showering, or even eating.

Patients should see a doctor if they experience worsening shortness of breath. Because connected tissue disease-related ILD is a condition that impacts multiple systems in your body, your care will be managed by a multidisciplinary team that includes pulmonologists, rheumatologists and other specialists. Developing symptoms of ILD may be the first sign of connective tissue disease for some patients.

Diagnosis

Your pulmonologist will review your medical history and conduct a physical exam. The exam will include listening to the lungs through a stethoscope to check for a crackling sound.

Connective tissue disease-related ILD is usually a progressive condition. The two diseases may progress together or separately from each other. It’s important to diagnose your specific connective tissue disease to get the right care. When your pulmonologist is evaluating your lungs, they may conduct one or more of the following tests.

Breathing and Exercise Tests

Pulmonary function tests: This test uses breathing exercises to measure the airflow and volume of air in the lungs. It also tests the ability of your lungs to take oxygen from the air.

Imaging Tests

High resolution computed tomography (HRCT): This is a type of CT scan that creates high-resolution images of the lungs. These images are taken without IV contrast.

Lab Tests

Blood tests: Bloodwork checks for antibodies related to connective tissue diseases. For patients who develop lung problems before the more common symptoms of CTD, blood tests may be the only evidence.

Further testing by a rheumatologist may be required if your results show signs of connective tissue disease.

Treatment

Your pulmonologist, rheumatologist and other specialists will work together closely to develop the best treatment for your specific connective tissue disease-related ILD. Even if your connective tissue disease is controlled with treatment, the ILD may not be. Patients with CTD-ILD will require ongoing monitoring.

Medications

Medications for connective tissue disease-related ILD generally suppress the immune system or reduce inflammation in the lungs to prevent future scarring. Your doctor may also use newer medications that directly slow down the production of scar tissue in the lungs. These medications can slow disease progression.

• Corticosteroids: Steroids like prednisone can reduce inflammation. These medications may be used to slow down or stop the progression of connective tissue disease-related ILD and can be prescribed for CTD-ILD flares.
• Cyclophosphamide: This medication suppresses the immune system to slow disease progression and improve symptoms like shortness of breath.
• Mycophenolate: Treatment with mycophenolate decreases immune response, which helps prevent or slow lung damage. Using this medication may enable patients to decrease their dosage of corticosteroids.
• Nintedanib: This medication can help prevent scarring in the lungs.

Therapies

Beyond medication, other treatment options for ILD include:

Oxygen Therapy: ILD can lead to low levels of oxygen in the blood. Improving oxygen levels in the blood with supplemental oxygen can help relieve strain on the heart and lungs, decreasing the symptoms of shortness of breath and fatigue. Some patients may need oxygen therapy all the time, while others may only need it during sleep or exercise.

Starting oxygen therapy can be a complex process because of the potential stigma. There are many different options for oxygen systems, and many patients can be more active. Learn more about oxygen therapy. 

Pulmonary Rehabilitation: A pulmonary rehabilitation program is recommended for most ILD patients to help you achieve your highest level of functioning. This includes: 

• Breathing and energy-saving techniques

• Education

• Exercise conditioning

• Nutritional counseling

• Psychosocial support

• Respiratory therapy evaluation

Pulmonary rehabilitation aims to improve quality of life by:

• Decreasing respiratory symptoms and complications

• Encouraging self-management and control over daily functioning

• Improving physical condition and exercise performance

• Increasing emotional well-being

• Reducing hospitalizations

Lifestyle Management

It’s important to follow your doctors’ recommendations for adapting your lifestyle to best manage your disease.

Patient Resources

Living with ILD

Procedures

Lung transplant may be an option for patients with worsening symptoms who haven’t responded to medication.

Clinical Trials

Clinical trials help determine new treatment options for diseases and conditions. Patients with connective tissue disease-related ILD have access to clinical trials and should speak with their physician to determine what trials might work best for them.