CHILDHOOD ANAPHYLAXIS: NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

CHILDHOOD_ANAPHYLAXIS_NEW_DEVELOPMENTS _IN_DIAGNOSIS_AND_TREATMENT_kapak

IDIOPATHIC ANAPHYLAXIS

Sümeyye Baysal1 Feride Candan2

1Şanlıurfa Training and Research Hospital, Department of Pediatric Immunology and Allergic Diseases, Şanlıurfa, Türkiye
2Batman Training and Research Hospital, Department of Pediatric Immunology and Allergic Diseases, Batman, Türkiye

Baysal S, Candan F. Idiopathic Anaphylaxis. In: Harmancı K, editor. Childhood Anaphylaxis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.123-132.

ABSTRACT

Anaphylaxis is a severe, rapidly progressing systemic hypersensitivity reaction that can be life-threatening. While most cases are associated with identifiable allergens, some individuals experience recurrent anaphylactic episodes without an apparent trigger, a condition known as idiopathic anaphylaxis (IA). First described by Bacal et al. in 1978, IA remains a diagnostic and therapeutic challenge. By definition, IA is diagnosed after ruling out all known triggers, including food allergies, medications, insect venom, mast cell disorders, and alpha-gal syndrome. The unpredictable nature of IA significantly impacts patients’ quality of life, leading to anxiety, lifestyle modifications, and fear of recurrence.

Epidemiological studies estimate the lifetime prevalence of anaphylaxis to be between 0.3% and 1.6%. The exact incidence of IA is difficult to determine, though idiopathic cases account for approximately 30-60% of anaphylaxis cases in adults and 10% in pediatric populations in early studies. IA appears to be more common in women and in individuals with atopic conditions. In recent years, the prevalence of IA has decreased, likely due to advancements in diagnostic methods that have identified previously unrecognized triggers.

The pathophysiology of IA remains unclear, but hypotheses suggest dysregulated T-cell responses, excessive mast cell activation, and, although less significant, defects in histamine metabolism playroles. Additionally, IA may be linked to conditions such as mast cell activation syndromes and systemic mastocytosis. Clinical manifestations include urticaria, angioedema, bronchospasm, hypotension, and gastrointestinal symptoms.

Management of IA begins with the immediate administration of intramuscular epinephrine in suspected cases, followed by antihistamines, corticosteroids, and supportive therapy. For patients with frequent episodes, long-term prophylactic treatment options include antihistamines, leukotriene antagonists, and biologic agents such as omalizumab. Further research into the diagnosis and treatment of IA is essential to improving patient outcomes and quality of life.

Keywords: Anaphylaxis; Mast cells; Hypersensitivity; Epinephrine; Histamine; Autoimmunity

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