Pediatric Recurrent Fever and Autoinflammation from the Perspective of an Allergist/Immunologist

Autoinflammatory diseases are monogenic and polygenic disorders due to dysregulation of the innate immune system. The inherited conditions have been clustered with primary immunodeficiencies in the latest practice parameters, however, these diseases have unique clinical presentations, genetics and available therapies. Given the presentation of fevers, rashes and mucosal symptoms observed in many of these syndromes, patients are likely to present to an allergist/immunologist. While there has been attention in the literature to diagnosis and treatment of rare, genetically-defined autoinflammatory disorders, physicians are challenged by increasing numbers of patients with intermittent or periodic fevers who face unnecessary morbidities due to a lack of a diagnosis.
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Source Type: research