Selective IgG subclass deficiency

MedGen UID:: 56446
•Concept ID:: C0162539
•: Disease or Syndrome

Synonyms:	Deficiencies, IgG; Deficiency, IgG; IgG Deficiencies; IgG Deficiency
SNOMED CT:	Selective immunoglobulin G deficiency (190981001); Selective IgG immunodeficiency (190981001); Immunoglobulin G deficiency (12631000119106)

Monarch Initiative:	MONDO:0001901

Definition

A broad classification of dysgammaglobulinemias characterized by low or undetectable serum levels of immunoglobulin class G (IgG). Deficiencies of IgG present variably according to subclass. IgG deficiencies are typically relative among subclasses and not absolute. Thus even with a given selective IgG subclass deficiency, total IgG levels may still fall within normal range. The clinical course and prognosis is dependent upon the severity of the deficiency and associated morbidity. [from NCI]

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

Treatment and management of primary antibody deficiency: German interdisciplinary evidence-based consensus guideline.

Hanitsch L, Baumann U, Boztug K, Burkhard-Meier U, Fasshauer M, Habermehl P, Hauck F, Klock G, Liese J, Meyer O, Müller R, Pachlopnik-Schmid J, Pfeiffer-Kascha D, Warnatz K, Wehr C, Wittke K, Niehues T, von Bernuth H
Eur J Immunol 2020 Oct;50(10):1432-1446. Epub 2020 Sep 9 doi: 10.1002/eji.202048713. PMID: 32845010

Immunoglobulin treatment in primary antibody deficiency.

Maarschalk-Ellerbroek LJ, Hoepelman IM, Ellerbroek PM
Int J Antimicrob Agents 2011 May;37(5):396-404. Epub 2011 Jan 26 doi: 10.1016/j.ijantimicag.2010.11.027. PMID: 21276714

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Recent clinical studies

Etiology

Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum.

Shin JJ, Liauw D, Siddiqui S, Lee J, Chung EJ, Steele R, Hsu FI, Price C, Kang I
J Clin Immunol 2020 May;40(4):592-601. Epub 2020 Apr 2 doi: 10.1007/s10875-020-00773-y. PMID: 32239366 Free PMC Article

Clinical and Immunological Features of 78 Adult Patients with Primary Selective IgG Subclass Deficiencies.

Khokar A, Gupta S
Arch Immunol Ther Exp (Warsz) 2019 Oct;67(5):325-334. Epub 2019 Jul 30 doi: 10.1007/s00005-019-00556-3. PMID: 31363786

Immunoglobulin subclass concentration in preterm infants treated prophylactically with different intravenous immunoglobins.

Amato M, Hüppi P, Imbach P, Llauto A, Bürgi W
Am J Perinatol 1995 Sep;12(5):306-9. doi: 10.1055/s-2007-994481. PMID: 8540928

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Diagnosis

Defining Clinical and Immunological Predictors of Poor Immune Responses to COVID-19 mRNA Vaccines in Patients with Primary Antibody Deficiency.

Shin JJ, Par-Young J, Unlu S, McNamara A, Park HJ, Shin MS, Gee RJ, Doyle H, Afinogenova Y, Zidan E, Kwah J, Russo A, Mamula M, Hsu FI, Catanzaro J, Racke M, Bucala R, Wilen C, Kang I
J Clin Immunol 2022 Aug;42(6):1137-1150. Epub 2022 Jun 17 doi: 10.1007/s10875-022-01296-4. PMID: 35713752 Free PMC Article

H syndrome: 5 new cases from the United States with novel features and responses to therapy.

Bloom JL, Lin C, Imundo L, Guthery S, Stepenaskie S, Galambos C, Lowichik A, Bohnsack JF
Pediatr Rheumatol Online J 2017 Oct 17;15(1):76. doi: 10.1186/s12969-017-0204-y. PMID: 29041934 Free PMC Article

HLA-A and -B alleles and haplotypes in 240 index patients with common variable immunodeficiency and selective IgG subclass deficiency in central Alabama.

Barton JC, Bertoli LF, Acton RT
BMC Med Genet 2003 Jun 12;4:3. doi: 10.1186/1471-2350-4-3. PMID: 12803653 Free PMC Article

Effector mechanisms activated by human IgG subclass antibodies: clinical and molecular aspects. Review article.

Jefferis R, Pound J, Lund J, Goodall M
Ann Biol Clin (Paris) 1994;52(1):57-65. PMID: 8210076

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Therapy

Clinical and Immunological Features of 78 Adult Patients with Primary Selective IgG Subclass Deficiencies.

Khokar A, Gupta S
Arch Immunol Ther Exp (Warsz) 2019 Oct;67(5):325-334. Epub 2019 Jul 30 doi: 10.1007/s00005-019-00556-3. PMID: 31363786

H syndrome: 5 new cases from the United States with novel features and responses to therapy.

Immunoglobulin subclass concentration in preterm infants treated prophylactically with different intravenous immunoglobins.

Amato M, Hüppi P, Imbach P, Llauto A, Bürgi W
Am J Perinatol 1995 Sep;12(5):306-9. doi: 10.1055/s-2007-994481. PMID: 8540928

Serum IgG subclass concentrations before and after administration of intravenous immunoglobulin in common variable immunodeficiency.

Ozawa T, Motoyoshi F, Kato Y, Kondo N, Orii T
J Clin Lab Immunol 1992;38(1):29-39. PMID: 1343346

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Prognosis

Defining Clinical and Immunological Predictors of Poor Immune Responses to COVID-19 mRNA Vaccines in Patients with Primary Antibody Deficiency.

Frequency of selective IgG subclass deficiency: a reappraisal.

Aucouturier P, Mariault M, Lacombe C, Preud'homme JL
Clin Immunol Immunopathol 1992 Jun;63(3):289-91. doi: 10.1016/0090-1229(92)90236-h. PMID: 1623649

Role of immunoglobulin subclasses and specific antibody determinations in the evaluation of recurrent infection in children.

Gross S, Blaiss MS, Herrod HG
J Pediatr 1992 Oct;121(4):516-22. doi: 10.1016/s0022-3476(05)81137-0. PMID: 1403382

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