Autoinflammatory disorders may involve genetic mutations of the inflammasome or an environmental trigger in a genetically susceptible host. Dr. Jay Mehta discussed a practical, clinical approach to caring for patients with autoinflammatory disorders, such as periodic fever syndromes, during the 2022 ACR Education Exchange.
Evidence-based classification criteria for rare, hereditary, autoinflammatory fevers have been developed to aid clinicians in better understanding the differences between these rare conditions.
A number of autoinflammatory syndromes that result from genetic mutations have been described recently. The vast majority occur in children. However, three periodic fever syndromes are important for rheumatologists who treat adults to know about. The goal of this review is to provide a concise description of each condition, and to help the clinician understand…
CHICAGO—Joseph Breen, PhD, program officer at the National Institutes of Health in Bethesda, Md., opened the chronic fatigue syndrome (CFS) session at the Federation of Clinical Immunology Societies (FOCIS) 2017 meeting by asking presenters to describe the current state of the science to the key immunology stakeholders gathered in the room. The hope was that…
A 51-year-old Caucasian female was referred by a local rheumatologist to the Center of Autoinflammatory Diseases at Stony Brook University, N.Y., for an unusual disease presentation. The patient had had recurrent polyarthritis, fever and rash for the previous three years. She described having a migratory polyarthritis affecting the shoulders, knees, ankles and bilateral forefoot, with…
The patient was a 48-year-old woman who saw her primary care physician for a flu-like illness three months prior to admission. Her symptoms initially improved, but recurred one month later; she was treated symptomatically, and again symptoms resolved. Two months later, she presented to an outside facility’s emergency department with fever to 103ºF, with associated…
Patients experience fevers for many reasons, but can they help physicians diagnose disease? Using modern technology and social media, Jonathan S. Hausmann, MD, is working to further the understanding of body temperatures…
I’m writing in regard to the article, “Fever of Unknown Origin” (TR, March 2016), by Charles Radis, DO. In regard to the non-caseating granulomas, there was no mention of epithelioid or giant cells. In addition, it was mainly [complement] C4 in the center of the granulomas, which is often found in sarcoidosis. Moreover, whether these…
SAN FRANCISCO—Early in his career, Daniel Kastner, MD, PhD, scientific director at the National Human Genome Research Institute, saw a 24-year-old patient with a lifelong history of recurrent fever and severe episodes of arthritis. A colleague told him it was most likely familial Mediterranean fever (FMF). There was little then known about its mechanisms, and…
Settling into room 501 at Maine Medical Center, Mrs. N was on her way to the bathroom when she felt it coming on. One moment she was okay; the next, her chest felt damp and cold, even as her face flushed and her temperature spiked. Her forehead glistened beads of warm sweat. She felt the…