Interstitial Lung Disease Center

Idiopathic pulmonary fibrosis (IPF) is an idiopathic interstitial pneumonia (IIP) that has a high mortality with a median survival ranging from 2.5 to 3.5 years. The prevalence of IPF in the U.S. is approximately 3 to 42 cases per 100,000 population with an estimated 60,000 living cases in the US. At present the only proven therapeutic option for patients with IPF is lung transplantation (which has a 5-year survival of only 42%).

IPF is a specific form of chronic interstitial lung disease (CILD)/pneumonia associated with the histologic pattern of usual interstitial pneumonia (UIP). Cardinal features of IPF include progressive cough, dyspnea, bilateral interstitial infiltrates on radiographs, a restrictive ventilatory defect on pulmonary function tests (PFTs), and progressive fibrosis and destruction of the lung parenchyma. The term IPF should be restricted to patients with the appropriate clinical features and the histologic pattern of UIP. A definitive diagnosis of UIP requires surgical lung biopsy (SLB) , but the diagnosis of UIP can be affirmed with confidence by thin-section high-resolution CT (HRCT) scans in the appropriate clinical setting (eg, rule out connective tissue disease, asbestosis and hypersensitivity pneumonitis). IPF is one of the most frustrating disorders to manage, since current treatment is ineffective. Epidemiology of IPF demonstrates it is a rare disease with a cited prevalence rate ranging from 3 to 42 cases per 100,000 population. Despite its rarity, IPF accounts for more than 16,000 deaths annually in the US; this mortality rate is higher than a number of malignancies, including bladder cancer, multiple myeloma, and acute myelogenous leukemia. While there have been a few therapeutic trials involving IPF patients, none have demonstrated efficacy. However, these trials have added insight into the clinical course of patients with IPF. Specifically, these studies have taught us that physiologic parameters are not the best endpoints for this disease (eg, the largest clinical trial involving IPF patients showed that physiologic parameters (evaluated every 12 weeks) remained surprisingly stable and did not predict overall mortality (~20%) that occurred over a 1.5-year period). In fact many patients died from a UIP exacerbation defined as an acute, clinically significant deterioration of an unidentifiable cause. While these UIP exacerbations are usually fatal, anecdotal favorable responses have been reported with high-dose corticosteroids. Thus, the identification of baseline and/or changes in biomarker fingerprints that predict clinical outcomes in IPF could translate to surrogate endpoints that decrease the patient numbers and time required to test potential novel treatments in a clinical trial (eg, trails would become more appealing to pharmaceutical companies). A careful analysis of plasma samples, combined with appropriate statistical modeling to relate findings to patient features and clinical outcomes, provides a unique opportunity that has never before been available.

The UCLA Interstitial Lung Disease Program is the first in the west coast specifically dedicated to the care of patients with this group of disorders. The program offers a multidisciplinary approach to the diagnosis and treatment of patients with interstitial lung disease. It includes experts in the fields of pulmonary medicine, thoracic surgery, radiology, pathology, nutrition, and rehabilitation medicine. After a comprehensive evaluation, we will discuss the diagnosis and management of your patient, which may include standard immunosuppressive therapy, antifibrotic therapy with novel agents, as well as, lung transplantation. Reflecting our expertise in interstitial lung disease, we are one of the original IPFnet Network Sites funded by the NHLBI for research in Idiopathic Pulmonary Fibrosis. In addition, our program is an active participant in a variety of ongoing clinical trials and future studies are planned as well.

Faculty

Pulmonary Medicine
Joseph P. Lynch, III, M.D., Associate Chief, Medical Director
John A. Belperio, M.D.
Jane Deng, M.D.
Rajan Saggar, M.D.
Rajeev Saggar, M.D.

Lung Transplantation
David J. Ross, M.D., Medical Director
John A. Belperio M.D.
Joseph P. Lynch, III, M.D.
Rajan Saggar, M.D.
Rajeev Saggar, M.D.
Samuel Weigt, M.D.

Pathology
Michael Fishbein, M.D.

Chest Radiology
Jonathan Goldin, M.D., Ph.D
Rob Suh, M.D.

Thoracic Surgery
Robert Cameron, M.D.

Clinical Research Nurse
Susan Golleher, R.N.

Study Coordinators
Eileen Callahan

(310) 794-2466

Paul Lopez
(310) 794-3299

Additional links
Patient Support Group

Location
University of California, Los Angeles
Pulmonary Outpatient Clinic, Suite 365
200 Medical Plaza
Los Angeles, CA 90095

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University of California at Los Angeles