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Up to 25 percent of people with pulmonary alveolar proteinosis (PAP), a rare autoimmune disease that affects lung function, die within five years of their diagnosis.
Dr. Robert Barrington, assistant professor in the department of microbiology and immunology at the University of South Alabama College of Medicine, recently was awarded a two-year research grant totaling $80,000 from the American Lung Association to explore ways to improve this statistic.
Current therapy for PAP patients involves whole lung lavage, an invasive procedure that often must be performed every 1-2 years.
In the process of researching lupus, a chronic autoimmune disease, Dr. Barrington and his lab discovered what he described as “a little bit of serendipity.”
“We found the study models became extremely ill but not from lupus. We started examining other tissues and discovered autoimmune PAP was the cause. We are excited because this is the first observation of this autoimmune disease in lab models and we are therefore positioned to learn how this disease originates,” Dr. Barrington said. “By identifying underlying mechanisms of autoimmune PAP, we hope to identify new therapeutic targets in treating this disease and to also establish whether there are shared mechanisms between PAP and other autoimmune diseases such as lupus.”
PAP is a potentially deadly disease whereby disease-causing antibodies impair the functions of key cells in the lungs. This process leads to a buildup of fluid in the lungs, which in turn causes the patient to have difficulty breathing. Currently it is not known how these antibodies are generated. The goal of Dr. Barrington’s work is to understand this process and explore potential ways to block antibody production thereby improving patient care.
With this grant award, Dr. Barrington and his lab will have the support they need to continue their research on PAP. “It is a real honor to have our work recognized by the American Lung Association and to represent a nationally renowned foundation,” Dr. Barrington said. “Without our ongoing support from the University, the department of microbiology and Immunology and the USA Center for Lung Biology, the progress on this research would not have been possible.”
Dr. Barrington says he is preparing to submit the first manuscript for publication on this project, with his work’s ultimate goal of having a positive impact on patients with PAP. Dr. Barrington hopes this new model can be applied to helping those with PAP and potentially other autoimmune diseases.
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