Winter 2008

UCSF Makes Strides in Interstitial Lung Disease

Interstitial lung disease (ILD) can be confusing for patients and physicians because it includes more than 200 distinct types of lung disorders. These range from focal and transient inflammatory conditions to diffuse and chronic pulmonary fibrosis. Some forms of ILD are treatable as long as they are caught early, before fibrosis sets in; others are relentlessly progressive.

UCSF's Interstitial Lung Disease Clinic, co-directed by Jeffrey A. Golden, M.D., and Laura L. Koth, M.D., was developed to address this complicated group of conditions and to assist physicians, in collaborative consultation, in diagnosis and management. Part of the UCSF ILD Center of Excellence, the ILD Clinic integrates multidisciplinary diagnosis, treatment guidance, support and education for patients, and access to clinical trials.

"We encourage pulmonologists and internists to refer potential ILD cases to us right away," notes Sally McLaughlin, the advanced lung disease nurse with the UCSF ILD Clinic. "We have made great strides in the accurate diagnosis of ILD. And if we can diagnose a patient with, say, scleroderma lung disease, before it progresses to end-stage fibrosis, we have a good chance of treating it."

UCSF's multidisciplinary team of experts in ILD consists of specialists including radiologists, pathologists and pulmonologists. "We have weekly meetings in which we go over every case," McLaughlin says, "so we are very familiar with these disorders. If need be, we also consult others, such as rheumatologists, experts in occupational lung diseases and cardiothoracic surgeons."

ILD Clinic physicians work closely with the UCSF Lung Transplant Program. "There is a seamless connection between the ILD Clinic and transplantation, which is one reason why a large number of our lung transplants are for ILD," says Dr. Harold R. Collard, M.D., a member of the interstitial lung disease program at UCSF.

After making a diagnosis, the UCSF ILD specialists make recommendations about treatment and discuss further workup and management of comorbitidies with community physicians. "Our colleagues in the community are the key," Collard says. "We make every effort to be available to them as needed to provide ongoing support." In an effort to provide additional access and educational opportunity for community practitioners, the UCSF ILD Clinic will be hosting a CME course on ILD on April 5 in San Francisco.

The UCSF ILD Clinic also helps patients become knowledgeable, proactive managers of their condition. "We help educate patients about the expected course of their disease and various treatment options," Collard says. Recently, UCSF hosted a free seminar on idiopathic pulmonary fibrosis, which more than 200 patients and family members attended. "Patients and their families sometimes feel there is little hope," McLaughin explains. "We try to emphasize what can be done to improve health."

In addition to an hour-long visit with a clinician, patients at the UCSF ILD Clinic receive consultation with nursing and research staff, who provide additional information and assistance. This may include helping patients connect with advocacy groups, providing referrals to pulmonary rehabilitation programs that teach patients self-care principles for maintaining quality of life, and discussing the pros and cons of participating in research studies. The clinic staff is also exploring ways of using both the Osher Center for Integrative Medicine and the symptom management service at UCSF to help patients cope with lung disease.

Clinical Trials

Several clinical trials are usually being conducted through the UCSF ILD Clinic at any given time. Currently, these include the Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis (STEP-IPF), which is studying the effect of sildenafil on exercise capacity and quality of life in people with advanced IPF; the Bosentan Use in ILD (BUILD-3) trial, which is studying the effects of bosentan on lung function; and several local research studies. "Our goal is to have a clinical trial for every patient who is interested," Collard says.

Making a Referral

To be seen in the UCSF ILD Clinic, a patient will need to have recent full pulmonary function tests (spirometry, lung volume and diffusing capacity) as well as a high-resolution CT scan (1mm to 1.5mm collimation with supine, prone and expiratory images). The clinic staff will be happy to provide assistance with scheduling these tests.

For more information on the UCSF ILD Clinic's research studies, contact Michele DesMarais at (415) 353-2060.

To make a clinical referral, contact Sally McLaughlin at (415) 353-2577.

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