Dr. Shum is an immunologist and pulmonologist whose research lies at the intersection of autoimmunity and interstitial lung disease (ILD). Through his research program and clinical practice, he has sought to understand how the lung functions as an immune target in autoimmune diseases and the critical factors of the lung which serve to precipitate or propagate pulmonary inflammation. To approach these questions, Dr. Shum studies Monogenic disorders of autoimmunity and immune dysregulation that lead to ILD. Through his work he identified a novel lung-specific antigen named BPIFB1 that is targeted in patients with autoimmune lung disease. Importantly, he demonstrated that autoantibodies to BPIFB1 could be used as a biomarker to detect patients with lung autoimmunity. His lab also contributed to the discovery of COPA syndrome, a novel Mendelian disorder of immune dysregulation manifested by ILD and arthritis. He developed an animal model of COPA syndrome and showed that mutant Copa mice develop autoimmunity and ILD because of defects in thymocyte development and selection. His work helped implicate the innate immune adapter molecule STING in the molecular pathogenesis of disease by showing that missorting of STING at the Golgi by mutant COPA leads to its aberrant activation. Dr. Shum directed studies revealing that targeting STING signaling may be a promising approach to treat COPA syndrome. His lab demonstrated that inhibition of STING signaling significantly reversed inflammation in mutant Copa mice and in COPA syndrome patient cells. Dr. Shum’s future work aims to understand the mechanisms by which activation of STING in the lung stroma causes ILD in COPA syndrome and other forms of ILD. Through his discoveries, Dr. Shum strives to develop novel therapies for patients afflicted by ILD which remains a highly morbid disease.
Anthony Kinmen Shum, MD