Interstitial Lung Disease (Pulmonary Fibrosis)

The UT Southwestern Interstitial Lung Disease Program has been in operation for more than a decade. We offer a team-based, multidisciplinary approach to diagnosis and treatment of interstitial lung disease.

We have a threefold mission:

1. To provide state-of-the-art comprehensive care to improve the quality of life of our patients
2. To educate our patients and their caregivers
3. To conduct research to understand the causes of interstitial lung disease and to develop improved therapeutics

What Is Interstitial Lung Disease?

Interstitial lung disease, or ILD, is an umbrella term that encompasses 130 to 200 chronic noncancerous and noninfectious lung disorders. Other terms or descriptors of this group of diseases include interstitial pulmonary fibrosis and pulmonary fibrosis.

These diseases share a common feature of dysfunction at the interface between the air sacs and blood supply of the lung. ILD is typically associated with symptoms like chronic cough and shortness of breath. The clinical course of ILD varies widely. Determining the specific type of interstitial lung disease is essential in order to predict prognosis and outline appropriate therapies. The Interstitial Lung Disease Program at UT Southwestern Medical Center provides comprehensive evaluations and treatment for all ILDs. 

Subtypes

Although there are almost 200 potential forms of ILD, the vast majority (95 percent) will fall into one of the following categories:

• Autoimmune disease – People with autoimmune disease like rheumatoid arthritis, scleroderma, systemic lupus erythematosis, Sjogren’s syndrome, and polymyositis can develop ILD, and sometimes the ILD is the initial manifestation. Other patients have some autoimmune features but do not meet established criteria for diagnosis with one of those well recognized conditions.
• Inhalation exposures – A large number of inhaled substances can cause ILD, including tobacco smoke, asbestos, silica, and bioaerosols.
• Medications – A large number of medications can cause ILD. The most common are chemotherapeutic agents, some antiarrythmic drugs and long term use of certain antibiotics.
• Granulomatous diseases – These diseases cause a type of inflammation in the lung called granulomas. Sarcoidosis is the most common of these disorders, followed by hypersensitivity pneumonitis.
• Idiopathic Interstitial Pneumonia – The cause for these diseases is unknown. The most common one in this category is Idiopathic Pulmonary Fibrosis or IPF.
• Genetic – For some individuals pulmonary fibrosis runs in families due to an inherited genetic mutation.

Symptoms

Regardless of the cause, the inflammation and or scarring leads to several common symptoms. While most cases of interstitial lung disease develop gradually, cases can develop suddenly.

The most common symptoms include:

• Shortness of breath, especially with exertion
• Cough
• Fatigue and weakness
• Labored breathing

Testing

Due to the large number of different ILDs, the evaluation of pulmonary fibrosis usually requires testing that includes:

• Pulmonary function tests – These tests require a series of breathing maneuvers and measure three things. They measure how well the air moves from your lungs, the size of your lungs and the ability of oxygen to move from your lungs into your blood. The pattern of changes seen help us determine the cause for your ILD and we also use these to see how you are doing over time.
  
• High-resolution CT – This is a CT scan that takes high resolution images. This is the most important test in your initial evaluation and a high quality exam including images lying on your back in full inspiration and expiration combined with images lying on your stomach in full inspiration are required. If you had a CT prior to coming to clinic but it wasn’t high resolution or didn’t have all those maneuvers we will need a new scan performed here.
• 6 minute walk – This test is a measure of your ability to exercise. We also use it to determine whether or not you need oxygen and if so how much.
• Blood tests – We oftentimes perform a series of blood tests to evaluate for autoimmune disease. In addition, other tests may be necessary in individual cases and several of the medications used to treat these disorders require us to follow serial liver function tests and/or blood counts.
• Biopsy – A biopsy is not needed in all cases. However in some cases a lung biopsy is necessary for making the appropriate diagnosis. There are two types of lung biopsies: a transbronchial lung biopsy and a surgical lung biopsy. The first is less invasive and is obtained by bronchoscopy, which is performed as a day procedure under conscious sedation. However, for some ILDs, the diagnosis cannot be made readily from the small lung biopsies obtained by bronchoscopy so a surgical lung biopsy is performed. We partner with the UTSW Thoracic Surgery department when a surgical lung biopsy is required.

Making the Diagnosis

After an extensive history and physical exam and completion of the above tests a diagnosis can almost always be reached. The ILD team at UT Southwestern includes pulmonologists, an ILD-trained nurse practitioner, a thoracic radiologist, and a pulmonary pathologist who meet weekly to discuss cases and reach agreement about the underlying ILD diagnosis. Multidisciplinary Discussions (MDD) are the current gold standard for diagnosing these diseases and our face-to-face meetings are an essential component of our team-based approach to care. 

Therapy

Our goal is to develop an individualized comprehensive treatment plan for each patient. The therapy depends heavily on the underlying diagnosis. Interventions may include recommendations to remove causative exposures, to exercise, to use supplemental oxygen, and to take certain medications. The medications may include ones designed to suppress the immune system or to reduce the progression of lung fibrosis. 

We consider that addressing coexisting conditions such as sleep apnea, gastroesophageal reflux, and pulmonary hypertension are important to your overall care. Where appropriate, we will work closely with the UTSW pulmonary hypertension and lung transplantation teams, which are located across the hall in the adjacent clinical space.

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 the only designated site in North Texas, and one of just 40 nationwide. This selection ensures that the UTSW ILD program has met the rigorous standards required by the Pulmonary Fibrosis Foundation.

The goal of the network is to improve the quality of life of affected patients. Being a PFF CCN site ensures that you will be managed by an experienced multidisciplinary team with extensive expertise in the diagnosis and management of ILD, a commitment to supporting the patient and their family, and strong history of education and research to advance the field. 

As a designated site we will be participating the in the PFF Patient Registry. The goals of registry are to define and refine best practices, improve the quality of life of patients and advance research in the field. The Registry will eventually become the largest collection of data in the field and we are excited by the many opportunities that it will provide to improve treatment of these conditions.

Research

In addition to the Pulmonary Fibrosis Foundation Patient Registry, we have several other important ongoing research studies. Our center is one of the leading centers in the world for the study of the genetic underpinnings of pulmonary fibrosis. We have an active genetic research program and have partnered with many patients seen in the Interstitial Lung Disease & Pulmonary Fibrosis Clinic to advance our understanding of these diseases. In addition, we have several ongoing clinical trials that are designed to test new medications. You may be asked about your interest in participating in these research studies, as well as an important multicenter patient registry.

Please visit our Find a Clinical Trial page to see all available pulmonary fibrosis clinical trials.