Interstitial Lung Disease Program (ILD)
The interstitial lung diseases include many complex diseases ranging from idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated ILD to occupational lung disease and sarcoidosis. The Yale ILD Program is the only one of its kind in Connecticut. Patients come from throughout the state as well as from New York, Rhode Island, New Jersey, Massachusetts, as well as from countries outside the United States to be evaluated and treated at a center with exceptional experience with these diseases.
More than 100 diseases are included under the heading of interstitial lung diseases and include:
- Idiopathic pulmonary fibrosis
- Connective tissue disease-associated interstitial lung disease
- Hypersensitivity pneumonitis
- Bronchiolitis Obliterans Organizing Pneumonia (BOOP)
- Cryptogenic Organizing Pneumonia (COP)
- Non-Specific Interstitial Pneumonia (NSIP)
- Respiratory Bronchiolitis-Associated ILD (RB-ILD)
- Desquamative Interstitial Pneumonia (DIP)
- Lymphocytic Interstitial Pneumonia (LIP)
- Pulmonary Alveolar Proteinosis
- Lymphangioleiomyomatosis (LAM)
- Eosinophilic pneumonias
- Langerhan's cell histiocytosis
- Chronic eosinophilic pneumonia
- Pulmonary vasculitis
- Pulmonary hemorrhage syndromes
- Familial pulmonary fibrosis
ILD patients with inflammatory lung disease (including connective tissue disease associated ILD, vasculitis, hypersensitivity pneumonitis and drug-induced ILD) sometimes require immunosuppressive medications. The ILD center has developed monitoring programs with nursing involvement to ensure that patient safety is maintained for patients treated with such agents. In addition, the ILD center has developed close working relationships with transplant programs around the country to ensure efficient referral and a quick path to active listing when appropriate.
The Yale ILD Program has initiated a patient support group for those affected by IPF. We have offered educational programs on subjects such as oxygen systems and use, disease education, clinical trials education, and lung transplantation. Patients and their families have genuinely enjoyed these sessions.
We have collaborated with the Pulmonary Fibrosis Foundation, a national patient advocacy organization, to create a patient-focused webinar entitled “Almost Everything You Need to Know About IPF.” This program features members of the Yale ILD Program and is permanently posted on the Foundation’s web page. (Link: http://www.pulmonaryfibrosis.org/almosteverything)
The Yale ILD Program offers clinical trial enrollment for patients with idiopathic pulmonary fibrosis as part of a multidisciplinary and integrated approach to treatment. Yale is one of 26 Centers of Excellence in the NHLBI-funded IPFNet consortium, which offers clinical studies for idiopathic pulmonary fibrosis (IPF). Results of these studies have been reported in the Lancet and the New England Journal of Medicine during this past year. The Yale ILD Program also offers phase II and III clinical trials of investigational agents through industry sponsors. Because IPF is a disease with no known treatment, the ILD program works to give patients the opportunity to receive novel agents and to contribute to the medical knowledge of this disease.
The particular focus of research in the laboratories of Dr. Erica Herzog and Dr. Jack Elias has been examining the role of alternatively activated macrophages and Semaphorin 7a in IPF and other fibrotic lung diseases, including scleroderma. The Yale ILD program has been recently selected to participate in a multicenter NIH-funded program on sarcoidosis.
The research of the Interstitial Lung Disease Program is dedicated to understanding common mechanisms promoting pulmonary fibrosis. This condition, which is characterized by the accumulation of scar tissue in the lung, is a major cause of death in Americans. Work in Dr. Herzog’s laboratory has identified a number of novel pathways that might control the development of this disease. Our studies focus on the role of Semaphorin 7a, a protein that is implicated in both brain development and inflammation, in the pathogenesis of pulmonary scar. We are also performing studies of fibrosis using an artificial lung technique that was developed here at Yale. Finally, we are heavily involved in the search for predictive biomarkers that might allow physicians to identify patients with active disease and understand what separates these people from those with stable disease. It is hoped that this unique combination of translational studies will allow better insight into diseases such as Idiopathic Pulmonary Fibrosis, Scleroderma related Interstitial Lung Disease, and Sarcoidosis, with the ultimate goal of developing preventative or treatment strategies.