MCID: SLC004
MIFTS: 38

Selective Igg Deficiency Disease

Categories: Immune diseases

Aliases & Classifications for Selective Igg Deficiency Disease

MalaCards integrated aliases for Selective Igg Deficiency Disease:

Name: Selective Igg Deficiency Disease 12 15
Igg Deficiency 44 70
Selective Immunoglobulin G Subclass Deficiency 12
Selective Immunoglobulin G Deficiency 12
Immunoglobin G Subclass Deficiency 12
Selective Igg Immunodeficiency 12
Selective Deficiency of Igg 12

Classifications:



External Ids:

Disease Ontology 12 DOID:14176
MeSH 44 D017099
NCIt 50 C27142
SNOMED-CT 67 190981001
ICD10 32 D80.3
UMLS 70 C0162539

Summaries for Selective Igg Deficiency Disease

Disease Ontology : 12 A dysgammaglobulinemia that is characterized by isolated deficiency of an IgG subclass, normal total IgG levels, and subsequent increased susceptibility to recurrent infections, atopic disease, and autoimmune conditions.

MalaCards based summary : Selective Igg Deficiency Disease, also known as igg deficiency, is related to herpes zoster and vasculitis. An important gene associated with Selective Igg Deficiency Disease is HLA-A (Major Histocompatibility Complex, Class I, A), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Levoleucovorin and Trimethoprim have been mentioned in the context of this disorder. Affiliated tissues include lung, bone marrow and spinal cord, and related phenotypes are Reduced mammosphere formation and hematopoietic system

Related Diseases for Selective Igg Deficiency Disease

Diseases related to Selective Igg Deficiency Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 109)
# Related Disease Score Top Affiliating Genes
1 herpes zoster 30.1 HLA-B HLA-A FCGR3A
2 vasculitis 29.5 MBL2 HLA-B FCGR3B
3 immune deficiency disease 29.2 TNFRSF13B MBL2 HLA-B HLA-A FCGR3B FCGR3A
4 common variable immunodeficiency 29.2 TNFRSF13C TNFRSF13B MBL2 HLA-B HLA-A HFE
5 systemic lupus erythematosus 28.4 TNFRSF13C TNFRSF13B MBL2 HLA-A FCGR3B FCGR3A
6 primary agammaglobulinemia 10.3 TNFRSF13C TNFRSF13B
7 postherpetic neuralgia 10.3 HLA-B HLA-A
8 frontal fibrosing alopecia 10.3 HLA-B HLA-A
9 stevens-johnson syndrome/toxic epidermal necrolysis 10.3 HLA-B HLA-A
10 htlv-1 associated myelopathy/tropical spastic paraparesis 10.3 HLA-B HLA-A
11 epstein-barr virus hepatitis 10.3 HLA-B HLA-A
12 nontuberculous mycobacterial lung disease 10.3 HLA-B HLA-A
13 colorectal cancer, hereditary nonpolyposis, type 8 10.2 HLA-B HLA-A
14 mental retardation, autosomal dominant 40 10.2 HLA-B HLA-A
15 pityriasis rosea 10.2 HLA-B HLA-A
16 pars planitis 10.2 HLA-B HLA-A
17 juvenile dermatitis herpetiformis 10.2 TNFRSF25 HLA-A
18 chronic pyelonephritis 10.2 HLA-B HLA-A
19 alveolar echinococcosis 10.2 HLA-B HLA-A
20 transient hypogammaglobulinemia 10.2 TNFRSF13B CD19
21 paraneoplastic pemphigus 10.2 HLA-B HLA-A
22 cytomegalovirus retinitis 10.2 HLA-B HLA-A
23 haemophilus meningitis 10.2 TNFRSF25 HLA-A
24 histoplasmosis 10.2 HLA-B HLA-A
25 pure red-cell aplasia 10.2 HLA-B HLA-A
26 orbital granuloma 10.2 TNFRSF25 TNFRSF13B
27 adie pupil 10.2 TOR1A TNFRSF25
28 47,xyy 10.2
29 autoimmune gastritis 10.1 HLA-B HLA-A
30 cryptococcal meningitis 10.1 TNFRSF13C TNFRSF13B MBL2
31 good syndrome 10.1 TNFRSF13B CD19
32 trachoma 10.1 MBL2 HLA-B
33 adrenal cortical hypofunction 10.1 TOR1A TNFRSF25
34 invasive malignant thymoma 10.1 TNFRSF25 CD19
35 porphyria cutanea tarda 10.0 HLA-B HLA-A HFE
36 transient hypogammaglobulinemia of infancy 10.0 TNFRSF13C TNFRSF13B CD19
37 immunodeficiency, common variable, 2 10.0 TNFRSF13C TNFRSF13B CD19
38 immunoglobulin a deficiency 1 10.0 TOR1A TNFRSF25 TNFRSF13B
39 agammaglobulinemia, x-linked 10.0 TNFRSF13C TNFRSF13B CD19
40 type 1 diabetes mellitus 2 10.0 TOR1A TNFRSF25
41 thrombocytopenia due to platelet alloimmunization 10.0 TNFRSF13B CD19
42 immunoglobulin g deficiency 10.0
43 microscopic polyangiitis 10.0 FCGR3B FCGR3A
44 idiopathic bronchiectasis 10.0 HLA-B HLA-A
45 transient neonatal neutropenia 10.0 FCGR3B FCGR3A
46 oxirane allergy 10.0 FCGR3B FCGR3A
47 herpetic whitlow 10.0 FCGR3B FCGR3A
48 immunodeficiency 20 10.0 FCGR3B FCGR3A
49 trichothiodystrophy 5, nonphotosensitive 9.9 FCGR3B FCGR3A
50 takayasu arteritis 9.9 HLA-B HLA-A FCGR3A

Graphical network of the top 20 diseases related to Selective Igg Deficiency Disease:



Diseases related to Selective Igg Deficiency Disease

Symptoms & Phenotypes for Selective Igg Deficiency Disease

GenomeRNAi Phenotypes related to Selective Igg Deficiency Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.02 HFE NCAM1 TNFRSF13B TNFRSF13C TOR1A

MGI Mouse Phenotypes related to Selective Igg Deficiency Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.61 CD19 FCGR3B HFE HLA-A MBL2 TNFRSF13B
2 immune system MP:0005387 9.28 CD19 FCGR3B HFE HLA-A MBL2 TNFRSF13B

Drugs & Therapeutics for Selective Igg Deficiency Disease

Drugs for Selective Igg Deficiency Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Levoleucovorin Approved, Investigational Phase 4 68538-85-2 149436
2
Trimethoprim Approved, Vet_approved Phase 4 738-70-5 5578
3
Sulfamethoxazole Approved Phase 4 723-46-6 5329
4
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
5 Immunoglobulins Phase 4
6 Vitamin B Complex Phase 4
7 Folate Phase 4
8 Anti-Bacterial Agents Phase 4
9 Antibiotics, Antitubercular Phase 4
10 Immunologic Factors Phase 4
11 Rho(D) Immune Globulin Phase 4
12 gamma-Globulins Phase 4
13 Anti-Infective Agents Phase 4
14 Vitamin B9 Phase 4
15 Trimethoprim, Sulfamethoxazole Drug Combination Phase 4
16 Immunoglobulins, Intravenous Phase 4
17 Antibodies Phase 4
18 Immunoglobulin G Phase 3
19 Vaccines Phase 2
20 Heptavalent Pneumococcal Conjugate Vaccine Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Treatment in Patients With Recurrent Infections and IgG Subclass Deficiency, and/or Deficient Anti-Polysaccharide Antibody Response Completed NCT00522821 Phase 4 intravenous immunoglobulins;antibiotics
2 IVIG Treatment for Asthmatic Patients With IgG Subclass Deficiency Unknown status NCT01992328 Phase 3 Immune Globulin
3 A Multicenter Extension Study on the Safety and Efficacy of IgPro10 in Patients With Primary Immunodeficiency (PID) Completed NCT00322556 Phase 3 Immunoglobulins Intravenous (Human)
4 A Multicenter Study on the Efficacy, Safety and Pharmacokinetics of IgPro10 in Patients With Primary Immunodeficiency (PID) Completed NCT00168025 Phase 3 Immunoglobulins Intravenous (Human)
5 An Open Study to Evaluate the Safety and Efficacy of IVIG-F10 in Patients With Primary Immunodeficiency Diseases (PID) Completed NCT00168012 Phase 3 Immunoglobulins Intravenous (Human)
6 Efficacy of IgIv in Patients With IgG Subclass Deficiency and Recurrent Infections Completed NCT00417573 Phase 2 IV Gamunex 10%
7 PNEUMOCELL - Conjugated Pneumococcal Vaccination in Patients With Immunoglobulin G-deficiency Completed NCT01847781 Phase 2

Search NIH Clinical Center for Selective Igg Deficiency Disease

Cochrane evidence based reviews: igg deficiency

Genetic Tests for Selective Igg Deficiency Disease

Anatomical Context for Selective Igg Deficiency Disease

MalaCards organs/tissues related to Selective Igg Deficiency Disease:

40
Lung, Bone Marrow, Spinal Cord, Skin, B Cells, Myeloid

Publications for Selective Igg Deficiency Disease

Articles related to Selective Igg Deficiency Disease:

(show top 50) (show all 89)
# Title Authors PMID Year
1
Immunoglobulin G Deficiency in Children with Recurrent Respiratory Infections with and Without History of Allergy. 61
32519307 2021
2
Immunoglobulin Deficiency as an Indicator of Disease Severity in Patients with COVID-19. 61
33237794 2020
3
Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum. 61
32239366 2020
4
Serum B-Cell Maturation Antigen (BCMA) Levels Differentiate Primary Antibody Deficiencies. 61
31430592 2020
5
De novo bronchiectasis in haematological malignancies: patient characteristics, risk factors and survival. 61
31720297 2019
6
Asthma as aetiology of bronchiectasis in Finland. 61
31128603 2019
7
Differentiation of Common Variable Immunodeficiency From IgG Deficiency. 61
30557717 2019
8
Gamma globulin replacement therapy in uncontrolled, severe asthma associated with humoral immunodeficiency: A series of five case reports. 61
29420096 2019
9
Recurrent Septic Shock and Syncope: An Unusual Combination. 61
31331154 2018
10
Vitamin D deficiency in children with recurrent respiratory infections, with or without immunoglobulin deficiency. 61
29128760 2018
11
Transient IgG deficiency with lesions in brain and spinal cord: a mimicker of common variable immunodeficiency syndrome. 61
27848135 2017
12
The Pneumocell-study: Vaccination of IgG1- and IgG2-deficient patients with Prevnar13. 61
28410816 2017
13
Agammaglobulinaemia despite terminal B-cell differentiation in a patient with a novel LRBA mutation. 61
28690850 2017
14
Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: A large patient cohort study. 61
27555459 2017
15
Adaptive Returns of Deficient Systemic Plasma Immunoglobulin G Levels as Rehabilitation Biomarker After Emergency Colectomy for Fulminant Ulcerative Colitis. 61
29276421 2017
16
Immunoglobulin G Deficiency-Associated Septic Arthritis Identified Following Corticosteroid Injection and Knee Arthroscopy: A Case Report. 61
29252646 2016
17
Secondary Antibody Deficiency in Glucocorticoid Therapy Clearly Differs from Primary Antibody Deficiency. 61
26980224 2016
18
Detection of the Dimorphic Phases of Mucor circinelloides in Blood Cultures from an Immunosuppressed Female. 61
27777804 2016
19
Immunoglobulin deficiency in patients with chronic rhinosinusitis: Systematic review of the literature and meta-analysis. 61
26329513 2015
20
Obligatory Role for B Cells in the Development of Angiotensin II-Dependent Hypertension. 61
26351030 2015
21
Recurrent C. difficile in a Patient with IgG Deficiency. 61
25945268 2015
22
[Classic Kaposi sarcoma associated with IgG deficiency]. 61
23122615 2013
23
Serum killing of Ureaplasma parvum shows serovar-determined susceptibility for normal individuals and common variable immuno-deficiency patients. 61
21802767 2012
24
IgG deficiency and expansion of CTG repeats in myotonic dystrophy. 61
21371814 2011
25
Immunoglobulin G deficiency in United kingdom children with invasive pneumococcal disease. 61
21487328 2011
26
Efficient immunoglobulin gene disruption and targeted replacement in rabbit using zinc finger nucleases. 61
21695153 2011
27
Immunoglobulin G subclass deficiency is the major phenotype of primary immunodeficiency in a Korean adult cohort. 61
20514300 2010
28
Down-Syndrome associated with MBL-deficiency, IgG-deficiency, vasculitis and mutated prothrombin. 61
19399328 2009
29
Results of endoscopic maxillary mega-antrostomy in recalcitrant maxillary sinusitis. 61
19178809 2008
30
Ruptured inflammatory aortic aneurysm with aortoenteric fistula and infected with Streptococcus pneumoniae: a review of the literature. 61
23133042 2008
31
[IgIV at home:experience of a center--economic aspects]. 61
17768832 2007
32
Collagenous duodeno-ileo-colitis with transient IgG deficiency preceded by Yersinia enterocolitica intestinal infection: case report and review of literature. 61
17408550 2007
33
Kinetics of FcRn-mediated recycling of IgG and albumin in human: pathophysiology and therapeutic implications using a simplified mechanism-based model. 61
17046328 2007
34
Recurrent erysipelas in myotonic dystrophy type 1 with IgG deficiency. 61
17389801 2007
35
[Economic evaluation of at home subcutaneous and intravenous immunoglobulin substitution]. 61
17011081 2006
36
Immunoglobulin induction therapy in renal transplant recipients: Effects on immunoglobulin and regulatory antibody levels. 61
17175311 2006
37
No evidence for prolonged excretion of polioviruses in persons with residual paralytic poliomyelitis in Ethiopia, Pakistan and Guatemala. 61
16682222 2006
38
Variability of immunodeficiency associated with ataxia telangiectasia and clinical evolution in 12 affected patients. 61
16238588 2005
39
Carbamazepine-induced IgG1 and IgG2 deficiency associated with B cell maturation defect. 61
15010058 2004
40
Search for poliovirus carriers among people with primary immune deficiency diseases in the United States, Mexico, Brazil, and the United Kingdom. 61
15106294 2004
41
The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. 61
14663287 2003
42
[The hyper-IgM syndrome: 13 observations]. 61
12714921 2003
43
Spinal epidural abscess associated with IgG4 deficiency. 61
12571493 2003
44
Evidence to support the cellular mechanism involved in serum IgG homeostasis in humans. 61
12578848 2003
45
[Henoch-Schoenlein syndrome in an 80-year-old patient]. 61
12075498 2002
46
Localization of extrapulmonary tuberculosis in the synovial membrane, skin, and meninges in a patient with systemic lupus erythematosus and IgG deficiency. 61
12120911 2002
47
[Pathogenesis and treatment of chronic purulent otitis media and its complications: immunological aspects]. 61
11510039 2001
48
[Middle lobectomy for bronchiectasis: clinical case and review of the literature]. 61
11280820 2001
49
Immunoglobulin deficiency in children with a sudden overwhelming infection. 61
11206240 2001
50
[Selective IgG deficiency]. 61
11212832 2000

Variations for Selective Igg Deficiency Disease

Expression for Selective Igg Deficiency Disease

Search GEO for disease gene expression data for Selective Igg Deficiency Disease.

Pathways for Selective Igg Deficiency Disease

Pathways related to Selective Igg Deficiency Disease according to GeneCards Suite gene sharing:

(show all 15)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.45 TNFRSF25 TNFRSF13C TNFRSF13B NCAM1 MBL2 HLA-B
2
Show member pathways
13.02 TNFRSF25 TNFRSF13C TNFRSF13B NCAM1 HLA-B HLA-A
3
Show member pathways
12.58 TNFRSF13C TNFRSF13B HLA-B HLA-A
4
Show member pathways
12.37 TNFRSF13C TNFRSF13B FCGR3B FCGR3A
5
Show member pathways
12.18 HLA-B HLA-A FCGR3B FCGR3A
6 12.06 TNFRSF13C HLA-B HLA-A
7 11.86 NCAM1 HLA-B HLA-A
8 11.69 HLA-B HLA-A FCGR3A CD19
9 11.64 MBL2 FCGR3B FCGR3A
10 11.61 NCAM1 HLA-A CD19
11
Show member pathways
11.46 TNFRSF25 TNFRSF13C TNFRSF13B
12 11.2 MBL2 HLA-B HLA-A FCGR3B FCGR3A
13 11.08 TNFRSF13C TNFRSF13B CD19
14 11.08 TNFRSF13C TNFRSF13B FCGR3B FCGR3A
15 10.92 HLA-B HLA-A

GO Terms for Selective Igg Deficiency Disease

Cellular components related to Selective Igg Deficiency Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.11 TOR1A TNFRSF25 TNFRSF13C TNFRSF13B NCAM1 HLA-B
2 integral component of membrane GO:0016021 10.02 TNFRSF25 TNFRSF13C TNFRSF13B NCAM1 HLA-B HLA-A
3 plasma membrane GO:0005886 9.96 TNFRSF25 TNFRSF13C TNFRSF13B NCAM1 HLA-B HLA-A
4 cell surface GO:0009986 9.73 NCAM1 MBL2 HLA-B HLA-A
5 integral component of plasma membrane GO:0005887 9.56 TNFRSF25 TNFRSF13B HLA-B HLA-A HFE FCGR3B
6 external side of plasma membrane GO:0009897 9.55 TNFRSF13C NCAM1 HFE FCGR3A CD19
7 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.4 HLA-B HLA-A
8 MHC class I protein complex GO:0042612 8.8 HLA-B HLA-A HFE

Biological processes related to Selective Igg Deficiency Disease according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.84 HLA-B HLA-A FCGR3B FCGR3A
2 cell surface receptor signaling pathway GO:0007166 9.8 TNFRSF25 TNFRSF13B FCGR3B FCGR3A
3 adaptive immune response GO:0002250 9.72 TNFRSF13C TNFRSF13B HLA-B HLA-A CD19
4 tumor necrosis factor-mediated signaling pathway GO:0033209 9.7 TNFRSF25 TNFRSF13C TNFRSF13B
5 immune system process GO:0002376 9.63 TNFRSF13C TNFRSF13B MBL2 HLA-B HLA-A CD19
6 interferon-gamma-mediated signaling pathway GO:0060333 9.58 NCAM1 HLA-B HLA-A
7 acute-phase response GO:0006953 9.56 MBL2 HFE
8 positive regulation of T cell mediated cytotoxicity GO:0001916 9.55 HLA-B HLA-A
9 detection of bacterium GO:0016045 9.51 HLA-B HLA-A
10 regulation of protein localization to cell surface GO:2000008 9.48 TOR1A HFE
11 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.46 HLA-B HLA-A
12 antigen processing and presentation of endogenous peptide antigen via MHC class I via ER pathway, TAP-independent GO:0002486 9.43 HLA-B HLA-A
13 antigen processing and presentation GO:0019882 9.43 HLA-B HLA-A HFE
14 protection from natural killer cell mediated cytotoxicity GO:0042270 9.37 HLA-B HLA-A
15 antigen processing and presentation of peptide antigen via MHC class I GO:0002474 9.13 HLA-B HLA-A HFE
16 regulation of immune response GO:0050776 9.02 HLA-B HLA-A FCGR3B FCGR3A CD19

Molecular functions related to Selective Igg Deficiency Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 9.62 MBL2 HLA-B HLA-A HFE
2 signaling receptor activity GO:0038023 9.54 TNFRSF25 TNFRSF13C TNFRSF13B
3 IgG binding GO:0019864 9.26 FCGR3B FCGR3A
4 beta-2-microglobulin binding GO:0030881 9.16 HLA-A HFE
5 TAP binding GO:0046977 8.96 HLA-B HLA-A
6 peptide antigen binding GO:0042605 8.8 HLA-B HLA-A HFE

Sources for Selective Igg Deficiency Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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