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Selective IgA deficiency disease

MedGen UID:: 883982
•Concept ID:: C4049006
•: Disease or Syndrome

Synonyms:	IgA, SELECTIVE DEFICIENCY OF; IMMUNOGLOBULIN A, SELECTIVE DEFICIENCY OF
SNOMED CT:	Selective IgA immunodeficiency (190979003); Selective immunoglobulin A deficiency (190979003)

Monarch Initiative:	MONDO:0001341
OMIM®:	137100

Definition

A dysgammaglobulinemia characterized by low or undetectable serum levels of immunoglobulin class A (IgA). It is the most common primary antibody deficiency. It may be inherited or the reversible sequela of infection or certain drugs. It may be caused by decreased or inefficient class-switching from progenitor B cells without any corresponding decreases in the other isotypes. Though affected persons may be asymptomatic, low levels of IgA will reduce the immune system''s ability to combat infection where IgA is normally secreted, at mucosal surfaces. Selective IgA deficiency is seen in greater proportion among patients with autoimmune disorders. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSelective IgA deficiency disease

Phenotypic abnormality
- Abnormality of the immune system
  - Abnormality of immune system physiology
    - Immunodeficiency
      - Humoral immune defect
        Selective immunoglobulin deficiency disease
        Selective IgA deficiency disease

Professional guidelines

PubMed

AGA Clinical Practice Update on the Evaluation and Management of Seronegative Enteropathies: Expert Review.

Leonard MM, Lebwohl B, Rubio-Tapia A, Biagi F
Gastroenterology 2021 Jan;160(1):437-444. Epub 2020 Oct 1 doi: 10.1053/j.gastro.2020.08.061. PMID: 33010252

Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.

Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681

Immunodeficiency in chronic sinusitis: recognition and treatment.

Stevens WW, Peters AT
Am J Rhinol Allergy 2015 Mar-Apr;29(2):115-8. doi: 10.2500/ajra.2015.29.4144. PMID: 25785751

See all (14)

Recent clinical studies

Etiology

IgA deficiency and autoimmune comorbidities in Juvenile Idiopathic Arthritis.

Torres-Fernandez D, Perez-Heras I, Fernandez-Manso B, Galán Del Río P, Gonzalez-Granado LI, de Inocencio J
An Pediatr (Engl Ed) 2022 Sep;97(3):172-178. Epub 2022 Apr 19 doi: 10.1016/j.anpede.2022.03.004. PMID: 35459637

Relationship between Selective IgA Deficiency and COVID-19 Prognosis.

Çölkesen F, Kandemir B, Arslan Ş, Çölkesen F, Yıldız E, Korkmaz C, Vatansev H, Evcen R, Aykan FS, Kılınç M, Aytekin G, Feyzioğlu B, Doğan M, Teke T
Jpn J Infect Dis 2022 May 24;75(3):228-233. Epub 2021 Sep 30 doi: 10.7883/yoken.JJID.2021.281. PMID: 34588364

Diagnosing and Monitoring Celiac Patients with Selective IgA Deficiency: Still an Open Issue.

Di Tola M, Bizzaro N, Gaudio M, Maida C, Villalta D, Alessio MG, Previtali G, Fabris M, Deleonardi G, Tampoia M, Brusca I, Pesce G, Porcelli B, Manfredi M, Infantino M; Study Group on Autoimmune Diseases of the Italian Society of Clinical Pathology and Laboratory Medicine
Dig Dis Sci 2021 Oct;66(10):3234-3241. Epub 2021 Aug 12 doi: 10.1007/s10620-021-07204-x. PMID: 34383199

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency.

Odineal DD, Gershwin ME
Clin Rev Allergy Immunol 2020 Feb;58(1):107-133. doi: 10.1007/s12016-019-08756-7. PMID: 31267472

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

Chapman KR, Burdon JG, Piitulainen E, Sandhaus RA, Seersholm N, Stocks JM, Stoel BC, Huang L, Yao Z, Edelman JM, McElvaney NG; RAPID Trial Study Group
Lancet 2015 Jul 25;386(9991):360-8. Epub 2015 May 27 doi: 10.1016/S0140-6736(15)60860-1. PMID: 26026936

See all (216)

Diagnosis

Diagnosing and Monitoring Celiac Patients with Selective IgA Deficiency: Still an Open Issue.

The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency.

Odineal DD, Gershwin ME
Clin Rev Allergy Immunol 2020 Feb;58(1):107-133. doi: 10.1007/s12016-019-08756-7. PMID: 31267472

Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.

Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681

Ataxia telangiectasia: a review.

Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM
Orphanet J Rare Dis 2016 Nov 25;11(1):159. doi: 10.1186/s13023-016-0543-7. PMID: 27884168 Free PMC Article

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

See all (224)

Therapy

Autoimmune Inner Ear Disease among Patients with Selective IgA Deficiency.

Magen E, Blum I, Waitman DA, Kahan N, Forer B
Audiol Neurootol 2021;26(2):127-134. Epub 2020 Dec 11 doi: 10.1159/000509577. PMID: 33311024

Limited Innovations After More Than 65 Years of Immunoglobulin Replacement Therapy: Potential of IgA- and IgM-Enriched Formulations to Prevent Bacterial Respiratory Tract Infections.

Langereis JD, van der Flier M, de Jonge MI
Front Immunol 2018;9:1925. Epub 2018 Aug 23 doi: 10.3389/fimmu.2018.01925. PMID: 30190722 Free PMC Article

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

Immunodeficiency in chronic sinusitis: recognition and treatment.

Stevens WW, Peters AT
Am J Rhinol Allergy 2015 Mar-Apr;29(2):115-8. doi: 10.2500/ajra.2015.29.4144. PMID: 25785751

Selective IgA deficiency.

Yel L
J Clin Immunol 2010 Jan;30(1):10-6. Epub 2010 Jan 26 doi: 10.1007/s10875-009-9357-x. PMID: 20101521 Free PMC Article

See all (96)

Prognosis

Relationship between Selective IgA Deficiency and COVID-19 Prognosis.

Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management.

Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A
Scand J Immunol 2017 Jan;85(1):3-12. doi: 10.1111/sji.12499. PMID: 27763681

Ataxia telangiectasia: a review.

Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM
Orphanet J Rare Dis 2016 Nov 25;11(1):159. doi: 10.1186/s13023-016-0543-7. PMID: 27884168 Free PMC Article

Seronegative Celiac Disease and Immunoglobulin Deficiency: Where to Look in the Submerged Iceberg?

Giorgio F, Principi M, Losurdo G, Piscitelli D, Iannone A, Barone M, Amoruso A, Ierardi E, Di Leo A
Nutrients 2015 Sep 8;7(9):7486-504. doi: 10.3390/nu7095350. PMID: 26371035 Free PMC Article

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

See all (95)

Clinical prediction guides

The Prevalence of Selective and Partial Immunoglobulin A Deficiency in Patients with Autoimmune Polyendocrinopathy.

Jamee M, Alaei MR, Mesdaghi M, Noorian S, Moosavian M, Dolatshahi E, Taghavi Kojidi H, Chavoshzadeh Z, Fallahi M, Parviz S, Aghamahdi F, Azizi G
Immunol Invest 2022 May;51(4):778-786. Epub 2021 Jan 12 doi: 10.1080/08820139.2021.1872615. PMID: 33432864

Seronegative Celiac Disease and Immunoglobulin Deficiency: Where to Look in the Submerged Iceberg?

Giorgio F, Principi M, Losurdo G, Piscitelli D, Iannone A, Barone M, Amoruso A, Ierardi E, Di Leo A
Nutrients 2015 Sep 8;7(9):7486-504. doi: 10.3390/nu7095350. PMID: 26371035 Free PMC Article

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial.

Selective IgA deficiency.

Yel L
J Clin Immunol 2010 Jan;30(1):10-6. Epub 2010 Jan 26 doi: 10.1007/s10875-009-9357-x. PMID: 20101521 Free PMC Article

Selective IgG1 deficiency.

Lacombe C, Aucouturier P, Preud'homme JL
Clin Immunol Immunopathol 1997 Aug;84(2):194-201. doi: 10.1006/clin.1997.4386. PMID: 9245552

See all (114)