Inborn Errors of Immunity Guidelines Pocket Guide - Guideline Central

Inborn Errors of Immunity

American College of Allergy, Asthma, and Immunology, American Academy of Allergy, Asthma & ImmunologyPublished: June 16, 2026

  • Key Points
  • Diagnosis
  • Management
  • Tables
    • International Union of Immunological Societies (IUIS) Classification of Human IEI 2024
    • Examples of Secondary Causes of Immunodeficiency
    • Primary Immune Deficiency Treatment Consortium (PIDTC) 2022 Definitions for SCID
    • Recommendations for the Care of Infants With SCID Diagnosis
    • Detection Capabilities of Genetic Testing Platforms for Various Genomic Findings
    • Examples of Specific Laboratory Testing for CIDs With or Without Syndromic Features
    • Defects of Phagocyte Numbers and Function
    • Examples of Diagnostic Assays for Defects of Innate Immune System Based on Infection Susceptibility (Complement Defects Not Included)
    • Complement Deficiencies and Associated Infection and Diseases
    • Examples of IEI Associated With Chronic Viral Infections and Specific Nonviral Infections That May Warrant Evaluation or Monitoring
    • Examples of IEI Associated With Autoimmune Cytopenias
    • Examples of IEIs Associated With Increased Cancer Risk
    • Antibiotic Prophylaxis for Selected IEI
    • Special Considerations for Antibiotic Treatment in At-Risk Individuals
    • Management of Lung Disease
    • Management of Gastrointestinal Disease
    • Management of Dermatologic Disease
    • Management of Rheumatologic and Musculoskeletal Disease
    • Management of Hematologic/Oncologic Disease
    • Vaccination Considerations in IEI
    • Key Examples of Precision Medicine in IEI
    • Food and Drug Administration (FDA)-Approved Precision Agents With Applications in IEI
    • QoL Tools Used in IEI


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