Connective Tissue Disease-Associated ILD
Overview
Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a small number of patients with a connective tissue disease. Examples of connective tissue diseases — also known as rheumatologic, collagen vascular or autoimmune diseases — include scleroderma, rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis and mixed connective tissue disease.
Patients are often diagnosed with the connective tissue disease first and develop CT-ILD later, although in some cases, the opposite occurs.
CT-ILD causes inflammation or scarring (fibrosis) of the lungs, or both. The exact cause of lung damage is unknown.
Our approach to connective tissue disease-associated ILD
UCSF offers specialized care for all types of interstitial lung disease (ILD), including connective tissue disease-related ILD. Treatments include medications, care for other health problems that often affect patients with ILD, and a special exercise and education program designed for patients with chronic lung disease. Our specialists review each case as a team to ensure that every patient gets the right diagnosis and most effective care.
For patients who get worse despite treatment, lung transplantation may be an option. UCSF is home to a high-performing lung transplant program with the expertise to handle the most complex, challenging cases.
Awards & recognition
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Among the top hospitals in the nation
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Best in Northern California and No. 4 in the nation for pulmonology & lung surgery
Signs & symptoms
Some patients with CT-ILD don't have symptoms. For others, common symptoms include:
- Shortness of breath with activity
- Cough
- Fatigue
- "Crackle" sound heard when listening to the chest with a stethoscope
- Symptoms of a connective tissue disease, such as joint pain and swelling, rash, dry eyes, dry mouth and acid reflux
Diagnosis
The diagnosis of CT-ILD requires input from pulmonologists, radiologists, and in some cases, pathologists experienced in interstitial lung disease.
In making a diagnosis, your doctor will start by conducting a thorough medical history and physical examination. This will include discussing your health history, reviewing medications and potential occupational and environmental exposures, listening to your chest with a stethoscope to check for a crackling sound, and carefully examining your skin and joints.
In addition, your doctor may conduct the following tests:
- Pulmonary function testing. This test involves a series of breathing maneuvers that measure the lungs' airflows, the volume of air in your lungs, and the ability of your lungs to extract oxygen from the air. This allows your doctor to objectively assess your lung function.
- High resolution computed tomography (HRCT). This is a special type of CT scan that provides high-resolution images of your lungs. Images are taken in several different positions: with you lying on your back, lying on your chest, and when breathing air out of your chest. These various images are extremely valuable in determining whether or not you have CT-ILD. Having an HRCT will feel no different than having a regular CT scan. The test is performed on an open-air table and takes only a few minutes.
- Blood tests. Blood tests for the presence of various antibodies are used to evaluate patients for a connective tissue disease. Some patients with connective tissue diseases develop lung problems before the more typical symptoms arise; in these cases, blood test results may be the only evidence of CT-ILD.
For some patients, interstitial lung disease is the first manifestation of a connective tissue disease. If this is the case, we may refer you to a rheumatologist for further evaluation.
Treatments
CT-ILD is treated with anti-inflammatory or immunosuppressive medications. You may recognize some or all of these medications if they were prescribed to you for your connective tissue disease.
The most common medications used to treat CT-ILD are:
- Corticosteroid (prednisone)
- Cyclophosphamide (Cytoxan)
- Mycophenolate mofetil (Cellcept)
Pulmonary rehabilitation is another important and effective treatment for patients with CT-ILD. Pulmonary rehabilitation is an exercise and education program designed for patients with chronic lung disease.
It's also important to pay close attention to any associated medical problems, such as gastroesophageal reflux disease (GERD) and pulmonary hypertension.
For patients with worsening symptoms that don't respond to medication, lung transplantation may be an option.
UCSF Health medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.
