SIGMD
MCID: SLC003
MIFTS: 21

Selective Igm Deficiency Disease (SIGMD)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Selective Igm Deficiency Disease

MalaCards integrated aliases for Selective Igm Deficiency Disease:

Name: Selective Igm Deficiency Disease 12
Selective Immunoglobulin M Deficiency 53 59 72
Selective Igm Deficiency 53 59
Sigmd 53

Classifications:

Orphanet: 59  
Rare immunological diseases


External Ids:

Disease Ontology 12 DOID:0050222
ICD10 33 D80.4
ICD10 via Orphanet 34 D80.4
Orphanet 59 ORPHA331235
UMLS 72 C0154275

Summaries for Selective Igm Deficiency Disease

NIH Rare Diseases : 53 Selective IgM deficiency (SIgMD) is a rare immune disorder in which a person has no immunoglobulin M (IgM) antibodies, or too little IgM, with normal levels of IgG and IgA antibodies. IgM is the first antibody the immune system makes to fight a new infection. Therefore, when a person does not have enough IgM, the body may have difficulty fighting infections. SIgMD can occur in infants, children, or adults. The disorder may occur as a primary disorder (on its own) or more commonly, as a secondary disorder (associated with another underlying disease or condition). SIgMD may occur in association with some cancers, autoimmune diseases, allergic diseases, and gastrointestinal diseases. Symptoms of SIgMD may include repeated viral, bacterial, or fungal infections, such as ear infections, bronchitis, sinusitis, and pneumonia. Infections may be life-threatening. Repeated infections are common in infants with SIgMD. In some people, diarrhea or a skin rash is the first symptom. Others do not have symptoms specific to SIgMD and are diagnosed when being evaluated for another condition. The diagnosis is made by blood tests showing low or absent IgM and normal levels of other antibodies, as well as diagnosing or ruling out underlying conditions that can cause low levels of IgM. The cause of SIgMD is still unclear. SIgMD usually occurs in only one person in a family, but familial cases have occasionally been reported. It may occur in some people with chromosome disorders such as 22q11.2 deletion syndrome. There are no official treatment recommendations since information about SIgMD is limited. Treatment options depend on the severity of symptoms and whether a person has an associated underlying disease. Treatment may include prompt treatment of infections, taking measures to prevent infections, prophylactic antibiotics, and immune globulin therapy.

MalaCards based summary : Selective Igm Deficiency Disease, also known as selective immunoglobulin m deficiency, is related to autoimmune disease and immune deficiency disease. Affiliated tissues include skin, testes and liver.

Disease Ontology : 12 A selective immunoglobulin deficiency disease thatis a dysgammaglobulinemia resulting from decreased levels of immunoglobulin M (IgM) production to roduction of other antibodies.

Related Diseases for Selective Igm Deficiency Disease

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

(show top 50) (show all 67)
# Related Disease Score Top Affiliating Genes
1 autoimmune disease 10.2
2 immune deficiency disease 10.2
3 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
4 autoimmune hepatitis 10.0
5 systemic lupus erythematosus 10.0
6 immunoglobulin alpha deficiency 10.0
7 lupus erythematosus 10.0
8 digeorge syndrome 10.0
9 diarrhea 10.0
10 ige responsiveness, atopic 9.9
11 chronic recurrent multifocal osteomyelitis 9.9
12 dermatitis, atopic 9.9
13 allergic rhinitis 9.9
14 aspergillosis 9.9
15 autoimmune glomerulonephritis 9.9
16 invasive aspergillosis 9.9
17 osteomyelitis 9.9
18 bacterial infectious disease 9.9
19 giardiasis 9.9
20 dysgammaglobulinemia 9.9
21 hepatitis a 9.9
22 acute cystitis 9.9
23 toxic shock syndrome 9.9
24 histoplasmosis 9.9
25 glomerulonephritis 9.9
26 food allergy 9.9
27 rhinitis 9.9
28 proliferative glomerulonephritis 9.9
29 liver cirrhosis 9.9
30 hemolytic anemia 9.9
31 impetigo 9.9
32 molluscum contagiosum 9.9
33 miliary tuberculosis 9.9
34 neurofibromatosis, type ii 9.8
35 immunoglobulin a deficiency 1 9.8
36 otitis media 9.8
37 palatopharyngeal incompetence 9.8
38 anaplastic large cell lymphoma 9.8
39 lymphoproliferative syndrome 9.8
40 gastric leiomyoma 9.8
41 sarcoma 9.8
42 t cell deficiency 9.8
43 tetanus 9.8
44 patau syndrome 9.8
45 common variable immunodeficiency 9.8
46 leiomyoma 9.8
47 adult-onset still's disease 9.8
48 ventricular septal defect 9.8
49 agammaglobulinemia 9.8
50 clear cell sarcoma 9.8

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



Diseases related to Selective Igm Deficiency Disease

Symptoms & Phenotypes for Selective Igm Deficiency Disease

Drugs & Therapeutics for Selective Igm Deficiency Disease

Search Clinical Trials , NIH Clinical Center for Selective Igm Deficiency Disease

Genetic Tests for Selective Igm Deficiency Disease

Anatomical Context for Selective Igm Deficiency Disease

MalaCards organs/tissues related to Selective Igm Deficiency Disease:

41
Skin, Testes, Liver, B Cells

Publications for Selective Igm Deficiency Disease

Articles related to Selective Igm Deficiency Disease:

(show all 22)
# Title Authors PMID Year
1
Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature. 6
28730517 2017
2
Acute-onset Autoimmune Hepatitis in a Patient with Selective Immunoglobulin M Deficiency. 38
30996186 2019
3
Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency. 38
30610030 2019
4
Chronic Pulmonary Histoplasmosis Identified in a Young Patient with Selective Immunoglobulin M Deficiency. 38
29888016 2018
5
Hypomorphic Mutations in the BCR Signalosome Lead to Selective Immunoglobulin M Deficiency and Impaired B-cell Homeostasis. 38
30619340 2018
6
Symptomatic Primary Selective IgM Immunodeficiency - B Lymphoid Cell Defect in Adult Man with Secondary HLH Syndrome. 38
27759358 2016
7
Selective immunoglobulin M deficiency in an adult with miliary tuberculosis: A clinically interesting coexistence. A case report and review of the literature. 38
26928000 2016
8
Symptomatic Primary Selective Immunoglobulin M Deficiency with Nonprotective Pneumococcal Titers Responsive to Subcutaneous Immunoglobulin Treatment. 38
27505292 2016
9
The prevalence of Selective Immunoglobulin M Deficiency (SIgMD) in Iranian volunteer blood donors. 38
26429316 2016
10
Selective Immunoglobulin M Deficiency Among Clozapine-Treated Patients: A Nested Case-Control Study. 38
26693046 2015
11
Atypical X-linked agammaglobulinaemia caused by a novel BTK mutation in a selective immunoglobulin M deficiency patient. 38
24074005 2013
12
A novel type of selective immunoglobulin m deficiency in a patient with autoimmune liver cirrhosis with recurrent hepatocellular carcinoma: a case report and review of the literature. 38
23257944 2013
13
Selective IgM deficiency in CD30+ cutaneous lymphoproliferative disorder. 38
21516014 2011
14
Selective immunoglobulin M deficiency in a patient with refractory giardiasis. 38
20815320 2010
15
Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency. 38
19034453 2009
16
Selective immunoglobulin M deficiency presenting with recurrent impetigo: a case report and review of the literature. 38
19218822 2009
17
A case of selective immunoglobulin M deficiency and autoimmune glomerulonephritis. 38
18365138 2008
18
Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis. 38
18564634 2008
19
Selective immunoglobulin M deficiency associated with disseminated molluscum contagiosum. 38
3089801 1986
20
Selective immunoglobulin M deficiency in an adult: assessment of immunoglobulin production by peripheral blood lymphocytes in vitro. 38
6891628 1982
21
Evaluation for immune system failures in horses and ponies. 38
6893592 1980
22
Selective immunoglobulin M deficiency in foals. 38
833046 1977

Variations for Selective Igm Deficiency Disease

Expression for Selective Igm Deficiency Disease

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

Pathways for Selective Igm Deficiency Disease

GO Terms for Selective Igm Deficiency Disease

Sources for Selective Igm Deficiency Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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