A new study demonstrates that reducing autoantibodies could help treat idiopathic pulmonary fibrosis — particularly in people who have a severe form of the disease. The research, entitled “Autoantibody-Targeted Treatments for Acute Exacerbations of Idiopathic Pulmonary Fibrosis,” was published on June 17th in the online journal PLoS One.

IPF refers to a thickening and scarring of the lungs that is caused by unknown factors. The disease results in difficulty breathing and is eventually fatal. Understanding the factors that lead to scarring is critical for developing treatments for IPF.

Serious attacks can occur in IPF, called acute exacerbations (AE). These are not medically treatable and can rapidly result in death. Autoantibodies may underlie acute exacerbations in IPF.

Led by Michael Donahue of the Department of Medicine, University of Pittsburgh, the researchers studied 11 people with a severe form of IPF. To block autoantibodies, they used a technique called therapeutic plasma exchange and prescribed the medication rituximab, supplemented with intravenous immunoglobulin. Therapeutic plasma exchange involves replacing the blood so that potentially harmful molecules such as autoantibodies are removed. Rituximab is a medication that blocks B cells, which can produce autoantibodies. The scientists also measured autoantibodies and matrix metalloproteinase-7, another marker for the progression of IPF. They compared the outcome to outcomes that are typically found in patients with the same severe form of IPF (called historical controls).

Nine of the study participants had improvements of pulmonary gas exchange (a measure of breathing) after treatment, compared to only one historical control. Autoantibodies that were found in the study participants before receiving the treatments went down in those subjects who responded to treatment, although the metalloproteinase-7 levels did not change. The most remarkable finding was that after one year 46% of the study participants were still alive, compared to none of the historical controls. The experimental treatments did not cause any serious side-effects.

In their report, the researchers concluded that “This pilot trial indicates specific treatments that reduce autoantibodies might benefit some severely-ill AE-IPF patients. These findings have potential implications regarding mechanisms of IPF progression, and justify considerations for incremental trials of autoantibody-targeted therapies in AE-IPF patients.”

The study is an initial step toward the treatment of patients who previously had no hope for survival. Larger studies are needed, and will hopefully provide a new possibilities for this difficult-to-treat disease.

The study authors noted that, “The results of the trial indicate autoantibody-reductive therapies may benefit some patients with this otherwise refractory and highly lethal syndrome.”