New approach to deadly lung disease may improve mortality rates

A new UAB study will investigate the role of autoantibodies and the benefit of therapeutic plasma exchange on a deadly lung disease.

steve duncan 2017 streamSteve Duncan, M.D.A new study at the University of Alabama at Birmingham seeks to discover whether a novel therapy can cut the death rate of a nearly always fatal pulmonary disease by more than half. Investigators, armed with a $3.8 million grant over five years from the National Institutes of Health, want to determine whether a regimen of therapeutic plasma exchanges, coupled with the drug rituximab and intravenous immunoglobulin, could be a beneficial therapy for patients with acute exacerbations of idiopathic pulmonary fibrosis.

“Severe acute exacerbations of IPF are medically untreatable and often fatal within days,” said Steve Duncan, M.D., professor in the Division of Pulmonary, Allergy and Critical Care Medicine in the Department of Medicine. “Based on a 2015 pilot study and clinical experience, we hypothesize that autoantibodies, which are implicated in many autoimmune diseases, may be associated with these acute exacerbations.”

Autoimmune diseases are caused by a dysfunctional immune response in which the body creates autoantibodies that attack the individual’s own proteins. IPF has not been characterized as an autoimmune disease, but Duncan believes it is possible that autoantibodies are involved in the disease, or in the development of acute exacerbations.

Duncan conducted the pilot study in 2015 with 11 patients who underwent a series of therapeutic plasma exchanges to remove autoantibodies from the blood stream. Half of those patients survived for at least six months, a dramatically higher survival rate over the expected 90 percent fatality rate. Since the publication of those study findings in the journal PLOS ONE, Duncan has treated 26 additional patients with therapeutic plasma exchange under a compassionate use protocol, with six-month survival rates of 60 percent.

Duncan will now launch a multisite study of 51 patients, who will receive nine plasma exchanges over two weeks. The patients’ blood is pumped into a machine that removes autoantibodies, then returned to the patient. Rituximab is added to attack and kill B cells, which are responsible for autoantibody production. Intravenous immunoglobulin is used to suppress B cells that may have escaped the rituximab.

“Based on our previous work, we believe that some patients with acute exacerbations of IPF could benefit from this therapy, based on approaches to treat autoimmune diseases like rheumatoid arthritis and lupus,” Duncan said. “The word ‘idiopathic’ basically means we don’t understand the origins of a disease. We hope this study will shed light on whether autoantibody production is involved in acute exacerbations of IPF.” 

In addition to UAB, the other clinical sites for the study are Harvard University, Temple University and University of Pittsburgh.

Idiopathic pulmonary fibrosis is a disease of unknown origin with no approved treatments. It results in scar tissue building up in the lungs. It affects more than 100,000 people in the United States and 5 million worldwide. The median survival rate is less than three years, and only 20 percent of patients survive five years beyond diagnosis.

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