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Selective IgE deficiency disease

MedGen UID:
96020
•Concept ID:
C0398694
•
Disease or Syndrome
Synonyms: Selective IgE deficiency; selective IgE deficiency disease; Selective IgE Immunodeficiency; selective IgE immunodeficiency; Selective immunoglobulin E deficiency; selective immunoglobulin E deficiency
SNOMED CT: Selective IgE deficiency (234540007); Selective immunoglobulin E deficiency (234540007)
 
Monarch Initiative: MONDO:0003738

Definition

A rare dysgammaglobulinemia characterized by low or undetectable serum levels of immunoglobulin class E (IgE). It is an uncommon primary antibody deficiency. It is most likely an inherited immunodeficiency. It may be caused by decreased or inefficient class-switching from progenitor B cells without any corresponding decreases in the other isotypes. Most affected persons appear asymptomatic but may show a predisposition to autoimmune and respiratory diseases. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSelective IgE deficiency disease

Professional guidelines

PubMed

Who's your data? Primary immune deficiency differential diagnosis prediction via machine learning and data mining of the USIDNET registry.
Méndez Barrera JA, Rocha Guzmán S, Hierro Cascajares E, Garabedian EK, Fuleihan RL, Sullivan KE, Lugo Reyes SO
Clin Immunol 2023 Oct;255:109759. Epub 2023 Sep 9 doi: 10.1016/j.clim.2023.109759. PMID: 37678719
Urticaria and Angioedema: Understanding Complex Pathomechanisms to Facilitate Patient Communication, Disease Management, and Future Treatment.
Konstantinou GN, Riedl MA, Valent P, Podder I, Maurer M
J Allergy Clin Immunol Pract 2023 Jan;11(1):94-106. doi: 10.1016/j.jaip.2022.11.006. PMID: 36610760
The treatment of psoriasis with IL-10: rationale and review of the first clinical trials.
Asadullah K, Döcke WD, Sabat RV, Volk HD, Sterry W
Expert Opin Investig Drugs 2000 Jan;9(1):95-102. doi: 10.1517/13543784.9.1.95. PMID: 11060663

Recent clinical studies

Etiology

Isotype deficiencies (IgG subclass and selective IgA, IgM, IgE deficiencies).
Imam K, Huang J, White AA
Allergy Asthma Proc 2024 Sep 1;45(5):317-320. doi: 10.2500/aap.2024.45.240055. PMID: 39294903
Acquired Angioedema in Selected Neoplastic Diseases.
Zając M, Bożek A, Kozłowska R, Grzanka A
Medicina (Kaunas) 2023 Mar 24;59(4) doi: 10.3390/medicina59040644. PMID: 37109602Free PMC Article
STAT mutations as program switchers: turning primary immunodeficiencies into autoimmune diseases.
Lorenzini T, Dotta L, Giacomelli M, Vairo D, Badolato R
J Leukoc Biol 2017 Jan;101(1):29-38. Epub 2016 Nov 1 doi: 10.1189/jlb.5RI0516-237RR. PMID: 27803128
Selective IgE deficiency and cardiovascular diseases.
Magen E, Mishal J, Vardy D
Allergy Asthma Proc 2015 May-Jun;36(3):225-9. doi: 10.2500/aap.2015.36.3825. PMID: 25976439Free PMC Article
Selective IgE deficiency, immune dysregulation, and autoimmunity.
Magen E, Schlesinger M, David M, Ben-Zion I, Vardy D
Allergy Asthma Proc 2014 Mar-Apr;35(2):e27-33. doi: 10.2500/aap.2014.35.3734. PMID: 24717782

Diagnosis

Isotype deficiencies (IgG subclass and selective IgA, IgM, IgE deficiencies).
Imam K, Huang J, White AA
Allergy Asthma Proc 2024 Sep 1;45(5):317-320. doi: 10.2500/aap.2024.45.240055. PMID: 39294903
Impaired Response to Polysaccharide Vaccine in Selective IgE Deficiency.
Noonan E, Straesser MD, Makin T, Williams A, Al-Hazaymeh A, Routes JM, Verbsky J, Borish L, Lawrence MG
J Clin Immunol 2023 Aug;43(6):1448-1454. Epub 2023 May 12 doi: 10.1007/s10875-023-01501-y. PMID: 37169968
Inborn errors of immunity and related microbiome.
Hazime R, Eddehbi FE, El Mojadili S, Lakhouaja N, Souli I, Salami A, M'Raouni B, Brahim I, Oujidi M, Guennouni M, Bousfiha AA, Admou B
Front Immunol 2022;13:982772. Epub 2022 Sep 13 doi: 10.3389/fimmu.2022.982772. PMID: 36177048Free PMC Article
Selective IgE deficiency and cardiovascular diseases.
Magen E, Mishal J, Vardy D
Allergy Asthma Proc 2015 May-Jun;36(3):225-9. doi: 10.2500/aap.2015.36.3825. PMID: 25976439Free PMC Article
Atopiform dermatitis.
Bos JD
Br J Dermatol 2002 Sep;147(3):426-9. doi: 10.1046/j.1365-2133.2002.05010.x. PMID: 12207579

Therapy

Urticaria and Angioedema: Understanding Complex Pathomechanisms to Facilitate Patient Communication, Disease Management, and Future Treatment.
Konstantinou GN, Riedl MA, Valent P, Podder I, Maurer M
J Allergy Clin Immunol Pract 2023 Jan;11(1):94-106. doi: 10.1016/j.jaip.2022.11.006. PMID: 36610760
Selective IgE Deficiency Predicts Poor or No Response of Chronic Spontaneous Urticaria to Omalizumab.
Picado C, Mascaró JJ, Vlagea A, Pascal M, Muñoz-Cano R, Bartra J
J Investig Allergol Clin Immunol 2022 Dec 15;32(6):504-506. Epub 2022 Feb 28 doi: 10.18176/jiaci.0796. PMID: 35225792
Humoral immunodeficiencies: conferred risk of infections and benefits of immunoglobulin replacement therapy.
Gernez Y, Baker MG, Maglione PJ
Transfusion 2018 Dec;58 Suppl 3(Suppl 3):3056-3064. doi: 10.1111/trf.15020. PMID: 30536429Free PMC Article
Selective IgE deficiency and cardiovascular diseases.
Magen E, Mishal J, Vardy D
Allergy Asthma Proc 2015 May-Jun;36(3):225-9. doi: 10.2500/aap.2015.36.3825. PMID: 25976439Free PMC Article
Selective IgE deficiency, immune dysregulation, and autoimmunity.
Magen E, Schlesinger M, David M, Ben-Zion I, Vardy D
Allergy Asthma Proc 2014 Mar-Apr;35(2):e27-33. doi: 10.2500/aap.2014.35.3734. PMID: 24717782

Prognosis

Constant vigilance! Managing threats to the skin barrier.
Chu V, Ong PY
Ann Allergy Asthma Immunol 2024 Jun;132(6):678-685. Epub 2024 Feb 13 doi: 10.1016/j.anai.2024.02.004. PMID: 38360106
Who's your data? Primary immune deficiency differential diagnosis prediction via machine learning and data mining of the USIDNET registry.
Méndez Barrera JA, Rocha Guzmán S, Hierro Cascajares E, Garabedian EK, Fuleihan RL, Sullivan KE, Lugo Reyes SO
Clin Immunol 2023 Oct;255:109759. Epub 2023 Sep 9 doi: 10.1016/j.clim.2023.109759. PMID: 37678719
Impaired Response to Polysaccharide Vaccine in Selective IgE Deficiency.
Noonan E, Straesser MD, Makin T, Williams A, Al-Hazaymeh A, Routes JM, Verbsky J, Borish L, Lawrence MG
J Clin Immunol 2023 Aug;43(6):1448-1454. Epub 2023 May 12 doi: 10.1007/s10875-023-01501-y. PMID: 37169968
Selective IgE Deficiency Predicts Poor or No Response of Chronic Spontaneous Urticaria to Omalizumab.
Picado C, Mascaró JJ, Vlagea A, Pascal M, Muñoz-Cano R, Bartra J
J Investig Allergol Clin Immunol 2022 Dec 15;32(6):504-506. Epub 2022 Feb 28 doi: 10.18176/jiaci.0796. PMID: 35225792
Primary immunodeficiencies of the B lymphocyte.
Moise A, Nedelcu FD, Toader MA, Sora SM, Tica A, Ferastraoaru DE, Constantinescu I
J Med Life 2010 Jan-Mar;3(1):60-3. PMID: 20302197Free PMC Article

Clinical prediction guides

Constant vigilance! Managing threats to the skin barrier.
Chu V, Ong PY
Ann Allergy Asthma Immunol 2024 Jun;132(6):678-685. Epub 2024 Feb 13 doi: 10.1016/j.anai.2024.02.004. PMID: 38360106
Who's your data? Primary immune deficiency differential diagnosis prediction via machine learning and data mining of the USIDNET registry.
Méndez Barrera JA, Rocha Guzmán S, Hierro Cascajares E, Garabedian EK, Fuleihan RL, Sullivan KE, Lugo Reyes SO
Clin Immunol 2023 Oct;255:109759. Epub 2023 Sep 9 doi: 10.1016/j.clim.2023.109759. PMID: 37678719
A narrative review on the role of magnesium in immune regulation, inflammation, infectious diseases, and cancer.
Ashique S, Kumar S, Hussain A, Mishra N, Garg A, Gowda BHJ, Farid A, Gupta G, Dua K, Taghizadeh-Hesary F
J Health Popul Nutr 2023 Jul 27;42(1):74. doi: 10.1186/s41043-023-00423-0. PMID: 37501216Free PMC Article
Impaired Response to Polysaccharide Vaccine in Selective IgE Deficiency.
Noonan E, Straesser MD, Makin T, Williams A, Al-Hazaymeh A, Routes JM, Verbsky J, Borish L, Lawrence MG
J Clin Immunol 2023 Aug;43(6):1448-1454. Epub 2023 May 12 doi: 10.1007/s10875-023-01501-y. PMID: 37169968
Acquired Angioedema in Selected Neoplastic Diseases.
Zając M, Bożek A, Kozłowska R, Grzanka A
Medicina (Kaunas) 2023 Mar 24;59(4) doi: 10.3390/medicina59040644. PMID: 37109602Free PMC Article

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