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Disclaimer is resource is for informational purposes only, intended as a quick-reference tool based on the cited source guideline(s), and should not be used as a substitute for the independent professional judgment of healthcare providers. Practice guidelines are unable to account for every individual variation among patients or take the place of clinician judgment, and the ultimate decision concerning the propriety of any course of conduct must be made by healthcare providers aer consideration of each individual patient situation. Guideline Central does not endorse any specific guideline(s) or guideline recommendations and has not independently verified the accuracy hereof. Any use of this resource or any other Guideline Central resources is strictly voluntary. Abbreviations AAAAI, American Academy of Allergy, Asthma and Immunology; ACAAI, American College of Allergy, Asthma and Immunology; ACMG, American College of Medical Genetics and Genomics; ADA, adenosine deaminase; aHUS, atypical uremic syndrome; AIHA, autoimmune hemolytic anemia; ALPS, autoimmune lymphoproliferative syndrome; AMD, age-related macular degeneration; AN, autoimmune neutropenia; APDS, activated phosphoinositide 3-kinase delta syndrome; APS, antiphospholipid syndrome; AR, autosomal recessive; AT, ataxia-telangiectasia; BCG, bacillus Calmette-Guerin; CBC, complete blood cell count; CDC, Centers for Disease Control and Prevention; CFH, complement Factor H; CGD, chronic granulomatous disease; CID, combined immunodeficiency; CMC, chronic mucocutaneous candidiasis; CMV, cytomegalovirus; CNS, central nervous system; CNV, copy number variation; CSF, cerebrospinal fluid; CT, computed tomography; CTTI, cultured thymus tissue implantation; CVID, common variable immunodeficiency; def., deficiency; DHR, dihydrorhodamine oxidation assay; EBV, Epstein-Barr virus; EKG, electrocardiogram; FDA, Food and Drug Administration; FHLH, familial hemophagocytic lymphohistiocytosis; GLILD, granulomatous and lymphocytic interstitial lung disease; GM-CSF, granulocyte-macrophage colony-stimulating factor; GOF, gain of function; HIES, hyperimmunoglobulin E syndrome; HIV, human immunodeficiency virus; HLH, hemophagocytic lymphohistiocytosis; HPV, human papilloma virus; HSCT, hematopoietic stem cell transplant; HSV, herpes simplex virus; ICF, immunodeficiency, centromeric instability and facial anomalies; IEI, in born error(s) of immunity; IFN, interferon; ITP, immune thrombocytopenic purpura; IUIS, International Union of Immunological Societies; IVIG, intravenous immunoglobulin; LAD, Leukocyte Adhesion Deficiency; MAS, macrophage activation syndrome; MHC, major histocompatibility complex; MMR, measles, mumps, rubella; MSMD, Mendelian susceptibility to mycobacterial disease; NBS, universal newborn screening; NK, natural killer; NSAID, nonsteroidal anti-inflammatory drug; PAD, primary antibody deficiency; PAP, pulmonary alveolar proteinosis; PCR, polymerase chain reaction; PIDTC, Primary Immune Deficiency Treatment Consortium; PIRD, primary immune dysregulatory disorders; PJP, Pneumocystis jirovecii pneumonia; PNH, paroxysmal nocturnal hemoglobinuria; PRO, patient-reported outcome; QoL, quality of life; RA, rheumatoid arthritis; RALD, RAS-associated autoimmune leukoproliferative disorder; RSV, respiratory syncytial virus; SAD, specific antibody deficiency; SCID, severe combined immunodeficiency; SCN, severe congenital neutropenia; SIGAD, selective IgA deficiency; SLE, systemic lupus erythematosus; SNP, single nucleotide polymorphism; SNV, single nucleotide variant; SS, Sjogren's syndrome; SSc, systemic sclerosis; THI, transient hypogammaglobulinemia of infancy; TLR, toll-like receptor; TMP/SMX, trimethoprim-sulfamethoxazole; TNF, tumor necrosis factor; TREC, T-cell receptor excision circle; VZV, varicella zoster virus; WAS, Wiskott- Aldrich syndrome; WHIM, warts, hypogammaglobulinemia, infections and myelokathexis; WHO, World Health Organization; XL, X-linked Source Orange JS, Chinen J, Horner CC, et al. Inborn errors of immunity guideline: Guidance from the Joint Task Force on Practice Parameters, the American Academy of Allergy, Asthma & Immunology (AAAAI), the American College of Allergy, Asthma and Immunology (ACAAI) and the Clinical Immunology Society (CIS). Ann Allergy Asthma Immunol 2026; 136:426−493. doi:10.1016/j. anai.2025.10.026 1258 Upsala Rd Sanford, FL 32771 TEL: 407.878.7606 Order additional copies at GuidelineCentral.com Copyright © 2026 All rights reserved AAAAIIMM06263a COR.0008.USA.26
