MCID: SLC004
MIFTS: 28

Selective Igg Deficiency Disease

Categories: Immune diseases
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Aliases & Classifications for Selective Igg Deficiency Disease

MalaCards integrated aliases for Selective Igg Deficiency Disease:

Name: Selective Igg Deficiency Disease 11 14
Igg Deficiency 43 71
Selective Immunoglobulin G Subclass Deficiency 11
Selective Immunoglobulin G Deficiency 11
Immunoglobin G Subclass Deficiency 11
Selective Igg Immunodeficiency 11
Selective Deficiency of Igg 11

Classifications:



External Ids:

Disease Ontology 11 DOID:14176
MeSH 43 D017099
NCIt 49 C27142
SNOMED-CT 68 190981001
ICD10 31 D80.3
UMLS 71 C0162539

Summaries for Selective Igg Deficiency Disease

Disease Ontology: 11 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 common variable immunodeficiency and camptodactyly-arthropathy-coxa vara-pericarditis syndrome. An important gene associated with Selective Igg Deficiency Disease is PRDM1 (PR/SET Domain 1), and among its related pathways/superpathways are BAFF in B-Cell Signaling and NF-kappaB Signaling. The drugs Sulfamethoxazole and Levoleucovorin have been mentioned in the context of this disorder. Related phenotypes are neoplasm and homeostasis/metabolism

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 82)
# Related Disease Score Top Affiliating Genes
1 common variable immunodeficiency 29.2 TNFRSF13C TNFRSF13B PRDM1 IRF4
2 camptodactyly-arthropathy-coxa vara-pericarditis syndrome 10.2
3 immune deficiency disease 10.2
4 herpes zoster 10.2
5 47 xxx syndrome 10.2
6 monoclonal paraproteinemia 10.1 PRDM1 PAX5
7 immunoglobulin g deficiency 10.0
8 myotonic dystrophy 1 10.0
9 lung lymphoma 10.0 PAX5 IRF4
10 bone lymphoma 10.0 PAX5 IRF4
11 breast lymphoma 10.0 PAX5 IRF4
12 childhood lymphoma 9.9 PAX5 IRF4
13 extramedullary plasmacytoma 9.9 PAX5 IRF4
14 multicentric castleman disease 9.9 PAX5 IRF4
15 lymphoplasmacytic lymphoma 9.9 PAX5 IRF4
16 testicular lymphoma 9.9 PDCD1LG2 IRF4
17 angioimmunoblastic t-cell lymphoma 9.9 PAX5 IRF4
18 primary mediastinal b-cell lymphoma 9.9 PDCD1LG2 IRF4
19 systemic lupus erythematosus 9.9
20 systemic lupus erythematosus 1 9.9
21 collagenous colitis 9.9
22 bronchiectasis 9.9
23 marginal zone b-cell lymphoma 9.9 PAX5 IRF4
24 primary agammaglobulinemia 9.8 TNFRSF13C TNFRSF13B
25 immunodeficiency, common variable, 2 9.8 TNFRSF13C TNFRSF13B
26 waldenstroem's macroglobulinemia 9.8 PAX5 IRF4
27 transient hypogammaglobulinemia of infancy 9.8 TNFRSF13C TNFRSF13B
28 transient hypogammaglobulinemia 9.8 TNFRSF13C TNFRSF13B
29 good syndrome 9.8 TNFRSF13C TNFRSF13B
30 immunodeficiency with hyper-igm, type 3 9.8 TNFRSF13C TNFRSF13B
31 lymphoma 9.8 PRDM1 PAX5 IRF4
32 adult lymphoma 9.8 PRDM1 PAX5 IRF4
33 plasmablastic lymphoma 9.8 PRDM1 PAX5 IRF4
34 prion disease 9.8 PAX5 IRF4
35 mature b-cell neoplasm 9.8 PRDM1 PAX5 IRF4
36 plasmacytoma 9.8 PRDM1 PAX5 IRF4
37 immunoglobulin alpha deficiency 9.8 TNFRSF13C TNFRSF13B
38 immunodeficiency with hyper-igm, type 1 9.7 TNFRSF13C TNFRSF13B
39 cd40 ligand deficiency 9.7 TNFRSF13C TNFRSF13B
40 autoimmune disease 9.7
41 ige responsiveness, atopic 9.7
42 kaposi sarcoma 9.7
43 pernicious anemia 9.7
44 thrombophilia due to thrombin defect 9.7
45 down syndrome 9.7
46 hydrops fetalis, nonimmune 9.7
47 immunodeficiency-centromeric instability-facial anomalies syndrome 1 9.7
48 pulmonary disease, chronic obstructive 9.7
49 mannose-binding lectin deficiency 9.7
50 twin-to-twin transfusion syndrome 9.7

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

MGI Mouse Phenotypes related to Selective Igg Deficiency Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 9.65 IRF4 PAX5 PDCD1LG2 PRDM1 TNFRSF13B
2 homeostasis/metabolism MP:0005376 9.63 IRF4 PAX5 PDCD1LG2 PRDM1 TNFRSF13B TNFRSF13C
3 immune system MP:0005387 9.43 IRF4 PAX5 PDCD1LG2 PRDM1 TNFRSF13B TNFRSF13C
4 hematopoietic system MP:0005397 9.1 IRF4 PAX5 PDCD1LG2 PRDM1 TNFRSF13B TNFRSF13C

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
Sulfamethoxazole Approved Phase 4 723-46-6 5329
2
Levoleucovorin Approved, Experimental, Investigational Phase 4 68538-85-2, 58-05-9, 73951-54-9 149436 6006
3
Trimethoprim Approved, Vet_approved Phase 4 738-70-5 5578
4
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
5 gamma-Globulins Phase 4
6 Antibodies Phase 4
7 Rho(D) Immune Globulin Phase 4
8 Immunologic Factors Phase 4
9 Immunoglobulins, Intravenous Phase 4
10 Immunoglobulins Phase 4
11 Anti-Infective Agents Phase 4
12 Folate Phase 4
13 Vitamin B9 Phase 4
14 Antibiotics, Antitubercular Phase 4
15 Vitamin B Complex Phase 4
16 Anti-Bacterial Agents Phase 4
17 Trimethoprim, Sulfamethoxazole Drug Combination 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 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)
4 A Multicenter Extension Study on the Safety and Efficacy of IgPro10 in Patients With Primary Immunodeficiency (PID) Completed NCT00322556 Phase 3 Immunoglobulins Intravenous (Human)
5 A Multicenter Study on the Efficacy, Safety and Pharmacokinetics of IgPro10 in Patients With Primary Immunodeficiency (PID) Completed NCT00168025 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

Publications for Selective Igg Deficiency Disease

Articles related to Selective Igg Deficiency Disease:

# Title Authors PMID Year
1
Predictors of shingles reports at diagnosis of common variable immunodeficiency and selective immunoglobulin G subclass deficiency in 212 Alabama adults. 62
24470948 2012
2
[Immunologic complications of visceral leishmaniasis]. 62
1775144 1991
3
Identification and rĂ´le of immunoglobulins in respiratory secretions. 62
3322854 1987
4
A case of selective immunoglobulin G-deficiency: analysis of coculture with normal lymphocytes in vitro. 62
7460398 1981
5
Selective deficiency of IgG and IgA as a first stage defect in B cell differentiation. 62
300257 1977

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.55 TNFRSF13C TNFRSF13B
2 11.44 PRDM1 PDCD1LG2 PAX5 IRF4
3 10.98 PRDM1 PAX5 IRF4
4 10.66 PAX5 IRF4

GO Terms for Selective Igg Deficiency Disease

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of T cell proliferation GO:0042102 9.56 TNFRSF13C PDCD1LG2
2 adaptive immune response GO:0002250 9.56 TNFRSF13C TNFRSF13B PRDM1 PDCD1LG2
3 T cell costimulation GO:0031295 9.46 TNFRSF13C PDCD1LG2
4 immune system process GO:0002376 9.1 TNFRSF13C TNFRSF13B PRDM1 PDCD1LG2 IRF4

Sources for Selective Igg Deficiency Disease

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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