Autoinflammation: Interferonopathies and Other Autoinflammatory Diseases

Abstract

The family of autoinflammatory diseases (AIDs) continues to expand and now includes over 40 genetically defined disorders. Their defining feature is a dysregulated inflammatory innate immune response. Many AIDs have overlapping clinical characteristics, and dermatological manifestations are common. Autoinflammatory features have also been recognized in more common dermatological conditions such as psoriasis. Furthermore, there is an increasing understanding that immunodeficiencies, autoimmune disorders, and even some allergic disorders share overlapping autoinflammatory features. The discovery that certain somatic mutations, arising within the bone marrow and restricted to the myeloid cell lineage can cause acquired AID heralds a new era of discoveries in this field.