Interstitial Lung Disease Program
Interstitial lung disease is a term used to describe a group of conditions that cause scarring and/or inflammation in space between the air sacs of the distal lung. In most patients, inflammation and scarring gradually interfere with gas-exchange and cause slowly progressive shortness of breath, chronic dry hacking cough and decreased blood oxygen levels. Management of patients with interstitial lung diseases is often complex and varies depending on the underlying cause. For patients referred to our clinic, we typically solicit input from a wide range of health care providers including pulmonologists, thoracic surgeons, rheumatologists, nurses and respiratory therapists.
The interstitial lung disease program at Thomas Jefferson University evaluates all forms of this lung condition including:
Acute Interstitial Pneumonia (AIP or Hamman-Rich Syndrome)
Connective tissue-related lung fibrosis (Scleroderma, Systemic Lupus Erythematosis, Sjogren’s, Rheumatoid arthritis)
Drug-induced lung fibrosis
Idiopathic forms of lung fibrosis
Langerhan's Cell Histiocytosis (aka Eosinophilic granuloma or Histiocytosis X)
Occupational-related lung fibrosis (e.g. Asbestosis)
Pulmonary Alveolar Proteinosis (PAP)
Radiation-induced lung fibrosis
The first step for patients referred to our clinic is to undergo a comprehensive evaluation to determine the cause and the best course of treatment for their disease. Although we follow evidence-based guidelines for all conditions, we also offer enrollment in clinical trials for those patients with conditions that have limited effective treatments. We are currently enrolling patients with idiopathic pulmonary fibrosis in the GS-US-322-0207 study trial, a global study of a novel humanized monoclonal antibody that binds to LOXL2. This therapy aims to reduce morbidity and mortality from this serious condition.
In addition to clinical expertise, our basic science research group, headed by Dr. Summer’s laboratory, is investigating novel pathogenic mechanisms in the development of lung fibrosis. The ultimate goal of this work is to identify therapeutic targets for treating fibrotic lung conditions.
If you wish to discuss research going on in our institution or would like to discuss referral to one of our physicians who specializes in treating interstitial lung diseases, we welcome the opportunity.