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Autoantibodies against BPI fold containing family B member 1 (BPIFB1) to diagnose autoimmune interstitial lung disease (ILD)

Patient and mouse studies suggest autoantibodies against BPIFB1 may help diagnose autoimmune ILD. In a large cohort of patients with autoimmune polyglandular syndrome type 1 (APS1), an autoantibody to BPIFB1 was detected in 9.6% of patients with APS1 and 100% of patients with both APS1 and ILD. In additional subjects with idiopathic ILD, the antibody was detected in 12% of patients, whereas the antibody was not detected in age-matched controls. In Bpifb1 knockout mice, immunization with recombinant BPIFB1 triggered an immune response, and transfer of these immune cells into immunodeficient mice led to the development of a lung disease similar to ILD. Next steps could include using Bpifb1-/- mouse models to understand the mechanisms underlying autoimmune ILD and to identify therapeutics.

SciBX 6(44); doi:10.1038/scibx.2013.1278
Published online Nov. 14, 2013

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Shum, A.K. et al. Sci. Transl. Med.; published online Oct. 9, 2013;
Contact: Mark S. Anderson, University of California, San Francisco, Calif.

Contact: Anthony K. Shum, same affiliation as above