Research Examines Variations of Rare Lung Disease

CINCINNATI—Lymphangioleiomyomatosis, or LAM, is a rare but serious lung disease that may cause severe respiratory symptoms in patients. The often-fatal disease has no cure.

Researchers say the key to learning more about LAM might lie in better understanding how symptoms differ among LAM patients

UC scientists are recruiting LAM patients to participate in a new research study that examines why symptoms of the disease are different in certain subgroups of people with the goal of finding more successful therapies.

LAM occurs when an unusual type of cell begins to grow out of control and spread to restricted areas in the body, including the lungs, kidneys, lymph nodes and vessels.

A team led by Jean Elwing, MD, an assistant professor in UC’s pulmonary, critical care and sleep division, is enrolling female LAM patients to see if pulmonary hypertension and pulmonary vascular disease may be contributing to their respiratory symptoms.

Pulmonary hypertension is a blood vessel disorder of the lungs in which pressure in the pulmonary artery, the blood vessel that leads from the heart to the lungs, rises above normal levels.

“We plan to evaluate a group of women with LAM who are experiencing shortness of breath for the presence of pulmonary hypertension,” she says. “We will look at participant’s medical history, pulmonary function tests, exercise tolerance, echocardiogram results and previous biopsy samples.

“We are hopeful this information will increase our understanding of how LAM can manifest in the patients it affects.  In the future, this information may be useful in developing better management strategies for this disease.”

Elwing says this study will compare LAM patients who also have pulmonary hypertension with those who do not to differentiate between the groups.

She says that researchers are hoping to enroll up to 40 people in this study.

“Some participants will be seen once in clinic and undergo testing with an echocardiogram while participating in this study,” she says.  “The individuals who have already undergone a clinical pulmonary hypertension evaluation may be able to participate through a review of select medical records and lung biopsy specimens. We welcome any patient with LAM who is interested in participating to contact us and discuss possible participation.”

This study is investigator-initiated and is funded by a Rhen Family Grant from the University of Cincinnati.