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Idiopathic anaphylaxis

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Idiopathic anaphylaxis is not uncommon and can be found in as many as 60% of patients referred to an allergist for anaphylactic events. It is an extremely frustrating condition for patients because they have no means of avoiding a known allergen and events occur spontaneously and unprovoked. The objective of this study was to synthesize and review the medical literature on idiopathic anaphylaxis and present a strategy for diagnosis, prevention, and treatment of episodes. A literature review was performed after a search of PubMed using the key words of idiopathic anaphylaxis. Idiopathic anaphylaxis is more common in adults than in children and in women than in men. The symptoms and signs are identical to those found in other causes of anaphylaxis. Perhaps the most common entities to be considered in the differential diagnosis are somatoform reactions. The two most recent advances in our ability to detect the cause are the discovery that episodes can be caused by mast cell‐activating syndromes, systemic mastocytosis, and IgE to galactose-alpha-1,3-galactose. Patients experiencing idiopathic anaphylactic episodes should, if possible, avoid taking drugs that may complicate therapy or worsen a reaction. Fortunately, the vast majority of cases follow a benign course. Although many recurrent episodes of anaphylaxis remain idiopathic, recent advances in our knowledge, especially the description of mast cell‐activating disorders and the discovery of anaphylactic reactions to galactose-α-1,3-galactose have allowed us to find causes in events that previously would have been classified as idiopathic. In addition, the majority of such patients exhibit a diminishing frequency of reactions over time and can be managed in a way that prevents mortality and a severe effect on their quality of life.
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Keywords: Anaphylactic shock; Munchausen's anaphylaxis; anaphylaxis; antihistamines; corticosteroids; epinephrine; galactose-α-1,3-galactose; idiopathic anaphylaxis; ketotifen; mast cell‐activating syndrome; omalizumab; somatoform anaphylaxis; systemic mastocytosis; unexplained anaphylaxis

Document Type: Research Article

Affiliations: Divisions of Allergy and Immunology, Departments of Medicine and Pediatrics, University of Tennessee, College of Medicine Memphis, Tennessee, USA

Publication date: January 1, 2014

More about this publication?
  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma and by having the potential to directly impact the quality of patient care. AAP welcomes the submission of original works including peer-reviewed original research and clinical trial results. Additionally, as the official journal of the Eastern Allergy Conference (EAC), AAP will publish content from EAC poster sessions as well as review articles derived from EAC lectures.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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