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Autoinflammatory Disease and the Human Condition

This is the annual WALS Mider Lecture. Former NHGRI Scientific Director, and an NIH Distinguished Investigator, Dr. Dan Kastner’s career at the NIH has focused on using genetic and genomic strategies to understand inherited disorders of inflammation, often stimulated by patients with relatively rare disorders seen at the NIH Clinical Center hospital. The systemic autoinflammatory diseases are a group of disorders characterized by seemingly unprovoked fever and inflammation, without the high-titer autoantibodies or antigen-specific T cells typically seen in autoimmune diseases. They are now recognized as disorders of the phylogenetically ancient innate branch of the immune system. Studies of monogenic autoinflammatory diseases have provided key insights into the molecular building blocks and regulation of human innate immunity and have been the basis for life-changing targeted therapies for a broad group of human illnesses. In this lecture Dr. Kastner will highlight the contributions of the Intramural Research Program in establishing this field of research, and he will discuss autoinflammation in the context of host defense against pathogens, aging, and the contribution of common genetic variants to human disease. Dan Kastner, M.D., Ph.D., NHGRI, NIH