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Interstitial Lung Disease
UT Southwestern Medical Center brings together skilled specialists from several disciplines to provide high-quality care for people who have interstitial lung disease and pulmonary fibrosis. Our team-based approach to care offers patients the expertise and advanced treatment options they need to relieve symptoms and improve their quality of life.
Advanced Care for Interstitial Lung Disease (Pulmonary Fibrosis)
Interstitial lung disease, or ILD, is a group of chronic, noncancerous, and noninfectious lung disorders in which lung tissue develops inflammation and becomes damaged. These problems can result in scarring, called pulmonary fibrosis (PF), which affects the ability to breathe and get enough oxygen.
For more than a decade, the Interstitial Lung Disease Program at UT Southwestern has provided comprehensive evaluations and treatment for all ILDs. Our lung specialists offer a team-based, multidisciplinary approach to ILD diagnosis and treatment.
We work to provide state-of-the-art, comprehensive care to improve our patients’ quality of life, to educate our patients and their caregivers, and to conduct research to understand the causes of ILD and develop improved therapeutics.
Types and Causes of Pulmonary Fibrosis
Almost 200 types of pulmonary fibrosis exist, and 95 percent of them fall into one of the following categories:
- Familial PF, a rare type that runs in families due to an inherited genetic mutation
- Granulomatous diseases, such as sarcoidosis, which cause a type of inflammation in the lungs called granulomas
- Idiopathic PF, which has no known cause
- PF from autoimmune diseases such as rheumatoid arthritis, scleroderma, lupus, Sjogren’s syndrome, and polymyositis
- PF from certain medications such as chemotherapy or radiation therapy for cancer treatment, some anti-arrhythmic drugs (to treat irregular heartbeat), and long-term use of certain antibiotics
- PF from exposures to inhaled substances such as tobacco smoke, asbestos, silica, and bioaerosols such as bird or animal droppings
Symptoms of Pulmonary Fibrosis
The inflammation and scarring that occur with an ILD can lead to symptoms such as:
- Dry cough
- Fatigue and weakness
- Muscle and joint pain
- Rapid, shallow breathing
- Shortness of breath, especially during exercise
- Unexplained weight loss
- Widening and rounding of fingertips (clubbing)
Diagnosis of Pulmonary Fibrosis
Our team, which includes pulmonologists, an ILD-trained nurse practitioner, a thoracic radiologist, and a pulmonary pathologist, is skilled at evaluating and diagnosing PF. We begin with a thorough evaluation that includes a:
- Physical exam
- Review of personal and family medical history
- Discussion of symptoms
To help confirm a diagnosis, our ILD specialists might recommend one or more of these tests:
- Blood tests to check for autoimmune diseases and to monitor patients taking medications that can affect liver function or blood counts
- Bronchoscopy to view inside the lungs and airways using a thin, flexible tube with a tiny light and camera (bronchoscope), and to take mucus or tissue samples for testing (biopsy)
- Chest X-ray to produce images of the lungs to rule out other causes for symptoms
- High-resolution computed tomography (CT) scan to produce more detailed images of the chest that can show the pattern of scar tissue and other lung damage, using specialized X-ray technology that takes cross-sectional images
- Echocardiogram to produce video images of the heart using ultrasound (sound waves) in a noninvasive procedure
- Endobronchial ultrasound (EBUS) to evaluate and diagnose lung disorders using a bronchoscope with an ultrasound probe to create images of the inside of the lungs
- Exercise stress test, typically a 6-minute walk to measure how well the lungs and heart function during exercise
- Pulmonary function tests to evaluate lung function, such as the volume of air flow in and out of the lungs, how well the lungs move oxygen into the bloodstream, and lung size
- Surgical biopsy to obtain a larger tissue sample, which is sometimes needed to confirm a diagnosis and is performed by our thoracic surgeons
Treatment for Pulmonary Fibrosis
The care team works to develop an individualized treatment plan based on each patient’s overall health, specific symptoms, and other health conditions. Treatment plans can include one or more options, such as:
Medications to treat ILD and its symptoms include:
- Pirfenidone (Esbriet) and nintedanib (Ofev) to slow progression of the disease
- Prednisone and other immunosuppressant medications to reduce the amount of inflammation in the lungs
- Anti-acid medications to treat gastroesophageal reflux disease (GERD), which frequently occurs in people with PF
- Nose sprays and inhalers to help treat cough
Our nonsurgical therapies include oxygen therapy, which can ease breathing, reduce the risk of complications from low blood oxygen levels, and prevent high blood pressure in the lungs (pulmonary hypertension).
At UT Southwestern, we also offer pulmonary rehabilitation, a long-term, outpatient program with specialized therapists. Pulmonary rehab can improve lung function and overall health, and we always recommend it after a lung transplant. Our services include:
- Breathing and coughing techniques
- Counseling and support
- Nutritional counseling
- Physical exercise
For people with advanced PF and severe lung damage, a single or double lung transplant can improve the ability to breathe and exercise. Over a recent 30-month period, the lung transplant program at UT Southwestern had the highest survival rate in the nation for one-year post-transplant patients. Since our lung transplant program began in 1990, it has consistently ranked among the top 10 in the United States.
Pulmonary Fibrosis Foundation Care Center Network Site
The UT Southwestern Interstitial Lung Disease Program is proud to be a designated site of the Pulmonary Fibrosis Foundation (PFF) Care Center Network (CCN). We are one of two designated sites in North Texas and one of just 60 nationwide. This designation ensures that the UTSW ILD program has met the PFF’s rigorous standards.
The goal of the network is to improve patients’ quality of life. Receiving care at a PFF CCN site assures patients that their care is in the capable hands of an experienced multidisciplinary team with:
- Extensive expertise in diagnosing and managing ILD and pulmonary fibrosis
- A commitment to supporting patients and their families
- A strong history of education and research to advance the field
As a designated site, UT Southwestern participates in the PFF Patient Registry. The Registry’s goals are to define and refine best practices, improve patients’ quality of life, and advance research in the field. The Registry will become the largest collection of data in the field, and we are excited by the many opportunities that will provide to improve ILD treatment.
As one of the leading centers in the world for the study of the underlying genetic causes of pulmonary fibrosis, we are conducting several research studies, such as:
- An active genetic research program that partners with patients of the Interstitial Lung Disease & Pulmonary Fibrosis Clinic to advance our understanding of these diseases
- Ongoing clinical trials to test new medications
We often ask patients about their interest in participating in these research studies and a multicenter patient registry. See all available clinical trials for pulmonary fibrosis and other lung diseases.
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Dallas, Texas 75390 214-645-3453