- Leslie C Grammer, MD
Leslie C Grammer, MD
- Professor of Medicine
- Northwestern University Feinberg School of Medicine
Anaphylaxis is often associated with an identifiable trigger, such as a food, medication, or insect sting. Idiopathic anaphylaxis is diagnosed when no specific trigger can be identified after an appropriate evaluation and when conditions, such as systemic mastocytosis, have been ruled out. (See "Anaphylaxis: Confirming the diagnosis and determining the cause(s)".)
This topic will review the pathogenesis, diagnosis, treatment, and prognosis of idiopathic anaphylaxis. Other aspects of anaphylaxis are discussed separately. (See "Anaphylaxis: Emergency treatment" and "Differential diagnosis of anaphylaxis in children and adults".)
Anaphylaxis is defined as a serious allergic reaction that is rapid in onset and may cause death . Symptoms of anaphylaxis involve multiple body organ systems (table 1). (See "Anaphylaxis: Acute diagnosis", section on 'Definition and diagnosis'.)
The diagnosis of anaphylaxis is based upon a detailed description of the acute episode, including antecedent activities and events. The diagnosis of idiopathic anaphylaxis is made when a patient has signs and symptoms consistent with anaphylaxis, but no specific trigger can be identified, and other diseases have been ruled out.
Idiopathic anaphylaxis is more common in adults, although it also occurs in children [2-4]. In several series, up to 70 percent of patients with idiopathic anaphylaxis were female and approximately 50 percent of patients were atopic [2,5,6]. Patients with idiopathic anaphylaxis may also have episodes of anaphylaxis caused by known triggers, such as food, medications, and exercise . The prevalence of idiopathic anaphylaxis in the United States is estimated at approximately 1 in 10,000 .
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- Mast cell activation
- Lymphocyte and basophil activation
- CLINICAL MANIFESTATIONS
- DIAGNOSIS AND REFERRAL
- Evaluate for possible triggers
- Exclude other disorders
- - Exclusion of a monoclonal mast cell population
- DIFFERENTIAL DIAGNOSIS
- Mast cell activation disorders
- Systemic mastocytosis
- Acute management
- Long-term management
- - Combination therapy with glucocorticoids and antihistamines
- - Other treatments
- - Glucocorticoid-dependent idiopathic anaphylaxis
- Management of refractory symptoms
- - Omalizumab
- - Rituximab
- Medications to be avoided
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS