Simmons Center for Interstitial Lung Disease

Program Description

The Interstitial Lung Disease (ILD) program is based within the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases at the University of Pittsburgh. This center was established in 2001 through the generous donation of the Simmons family with the aim of providing a unique environment in which disease management and diagnosis, patient and family education, and cutting edge research are seamlessly integrated.

Working in a multidisciplinary team, physicians and nurses at the center apply the most advanced approaches to the diagnosis and management of ILD such as idiopathic pulmonary fibrosis (IPF), Sarcoidosis and ILD associated with connective tissue disease, researchers apply cutting edge technologies to find new ways to diagnose and treat these diseases and nursing and quality of life experts educate and support the patients and their caregivers and family members.

Focus

The diagnosis and management of interstitial lung disease, a complex and diverse group of lung diseases, requires significant expertise and often requires interaction between multiple specialties. At the Simmons Center, evaluation and treatment by a multidisciplinary team that includes experts in pulmonary medicine, rheumatology, cardiology, dermatology, radiology, pathology, thoracic surgery, lung transplantation, genomics and quality of life exemplifies the strength of the University of Pittsburgh Medical Center and offers patients the benefits of an integrated approach to their disease.  The patients are offered to participate in NIH and industry sponsored research opportunities to study causes and potential treatments for their diseases. In particular the researchers and clinicians at the Simmons Center work closely to translate basic research on interstitial lung disease into new treatments that may save lives in the future and to new diagnostic approaches that will allow personalizing the management of their disease.  Quality of life assessment and disease management education are key components of the patient’s encounter with the Simmons Center. Patients and their families are offered participation in Support Groups, Regional Education Events and other outreach activities aimed to improve the ability of patients and their caregivers to handle the burden of disease.

The Clinical Team

The Simmons Center is a comprehensive multidisciplinary program incorporating research scientists, clinicians, nurse specialists, and rehabilitation medicine staff. The center is under the direction of Dr. Daniel Kass, and Dr. Kevin F. Gibson serves as the clinical director.

Additional PACCM faculty involved with the Center include, Dr. Yingze Zhang, and Dr. Kristen Veraldi. Among the the members of the Simmons Center from other divisions and departments are Dr. Luis Ortiz (EOH), Dr Tim Oury, Dr. Sam Yousem & Dr. Frank Schneider (Pathology), Dr. Chester Oddis, Dr. Thomas Medsger, and Dr. Robyn Domsic (Rheumatology), and Dr. Jonathon D’Cunha (Thoracic Surgeon) The research infrastructure at center includes multiple research coordinators, a huge collection of well phenotyped lung and peripheral blood samples and a detailed patient database. Current research efforts include basic investigations focused on the mechanisms of the lung fibrosis, injury and repair, genomics and proteomics of lung fibrosis and the role of the genetics in determining the fibrotic phenotype. As part of the special attention given at the program to facilitation of rapid translation from bench to bedside, scientists at the center are also active in translational research, studies of biomarkers of disease progression and development of new therapeutic drug targets. Scientists at the program are funded by NIH grants, and by the industry and enjoy a deep collaborative relationship with investigators all across the world.

Daniel J Kass, MD is the Director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease. Dr. Kass is a physician-scientist who specializes in the care of patients with interstitial lung disease (ILD). The most common ILD is idiopathic pulmonary fibrosis (IPF). Because IPF is currently untreatable, the overall goal of Dr Kass’ research efforts is to identify novel and personalized therapies for the patients who suffer from IPF. To achieve this goal, his laboratory studies two distinct aspects of the biology of fibroblasts, the principal effectors of pulmonary fibrosis. First, through analysis of the transcription factor twist1, Dr Kass’ lab is currently studying how fibroblasts interact with the immune system to regulate inflammation in pulmonary fibrosis. Second, the lab is also studying how the endogenous hormone relaxin may regulate the differentiation of fibroblasts into the highly active scarring cells known as myofibroblasts. He also serves as an investigator in multiple NIH- and pharmaceutical company-sponsored clinical trials.

Dr. Kevin Gibson is the medical director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease. He is in charge of all clinical research in the center and is the PI on several NIH-funded clinical trials and a number of pharmaceutical supported clinical trials. Dr. Gibson’s research is currently focuses on translational studies to identify unique biomarkers of disease activity in idiopathic pulmonary fibrosis and other interstitial lung diseases, and studies of gene expression profiling in lung and peripheral blood in idiopathic pulmonary fibrosis.

Yingze Zhang, PhD is interested in the molecular and genetic basis of fibrotic ILD including IPF. Her research is focused on the identification of both genes and peripheral blood biomarkers associated with the development of IPF and other fibrotic ILD/or associated with the disease progression/outcome of these patients. Her laboratory is also conducting mechanistic research to dissect the genetic and molecular basis of IPF.

Diseases

While patients with every ILD are seen at the Dorothy P. and Richard P. Simmons Center for ILD, most of the patient’s diagnoses fall within the following three main diseases (see chart below):

Idiopathic Pulmonary Fibrosis (IPF) 
The most common form of interstitial lung disease, marked by progressive scarring of the lungs that gradually interferes with a person’s ability to breathe. The diagnosis of IPF is challenging and there are no know therapies, except lung transplantation, although many new experimental drugs are being evaluated.

Sarcoidosis 
Sarcoidosis is an inflammatory disease of unknown origin impacts the lung but also other organs. It is variable in presentation, response to therapy and outcome.

ILD associated with autoimmune disease
Many autoimmune or rheumatologic diseases such as scleroderma, dermatomyositis and rheumatoid arthritis that affect the joints, the skin and other organs also affect the lung.  In many cases the outcome of the patient is dependent on the severity of their lung disease. In close collaboration with the division of rheumatology the physicians at the Simmons Center aggressively seek the extent of lung disease in these conditions and their optimal management.

Occupational lung disease
Scarring lung disease (pulmonary fibrosis) occurs as a consequence of a variety of occupational exposures including asbestosis, coal dust, beryllium, hard metals, and diisothiocyanates. At the Simmons Center, we a employ a multidisciplinary approach to the diagnoses of occupational lung disease using experienced clinicians, pathologists, radiologists, and occupational hygienist.