MCID: ATM074
MIFTS: 23

Autoimmune Autonomic Ganglionopathy

Categories: Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Autoimmune Autonomic Ganglionopathy

MalaCards integrated aliases for Autoimmune Autonomic Ganglionopathy:

Name: Autoimmune Autonomic Ganglionopathy 54
Autoimmune Autonomic Neuropathy 54

Classifications:



Summaries for Autoimmune Autonomic Ganglionopathy

NIH Rare Diseases : 54 Autoimmune autonomic ganglionopathy (AAG) is a rare autoimmune disorder in which the body's immune system mistakenly attacks and damages certain parts of the autonomic nervous system. Signs and symptoms of the condition vary but may include severe orthostatic hypotension (low blood pressure upon standing); fainting; constipation; fixed and dilated pupils; urinary retention; and/or dry mouth and eyes. The exact underlying cause of AAG is poorly understood. Treatment depends on many factors including the severity of the condition and the signs and symptoms present in each person. Due to the rarity of AAG, there are no standard treatment protocols; however, treatment with plasmapheresis, intravenous (IV) immunoglobulin, corticosteroids or immunosuppressive drugs has been reported with variable success. Approximately one third of affected people may improve spontaneously without treatment, but the recovery is often incomplete.

MalaCards based summary : Autoimmune Autonomic Ganglionopathy, also known as autoimmune autonomic neuropathy, is related to autonomic nervous system disease and dysautonomia. An important gene associated with Autoimmune Autonomic Ganglionopathy is CACNA1B (Calcium Voltage-Gated Channel Subunit Alpha1 B), and among its related pathways/superpathways is Beta-Adrenergic Signaling. The drugs Immunologic Factors and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include eye and lung.

Wikipedia : 77 Autoimmune autonomic ganglionopathy (AAG) is an rare form of dysautonomia in which the patients immune... more...

Related Diseases for Autoimmune Autonomic Ganglionopathy

Diseases related to Autoimmune Autonomic Ganglionopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 22)
# Related Disease Score Top Affiliating Genes
1 autonomic nervous system disease 11.4
2 dysautonomia 11.4
3 sjogren syndrome 10.2
4 anhidrosis 10.2
5 neuropathy 10.2
6 small cell cancer of the lung 10.1
7 lung cancer 10.1
8 myasthenia gravis 10.1
9 myeloma, multiple 10.1
10 lymphoma 10.1
11 autonomic neuropathy 10.1
12 intestinal pseudo-obstruction 10.1
13 monoclonal gammopathy of uncertain significance 10.1
14 subacute delirium 10.1
15 encephalitis 10.1
16 baroreflex failure 10.1
17 harlequin syndrome 10.1
18 myasthenia gravis congenital 10.1
19 opsoclonus-myoclonus syndrome 10.1
20 pure autonomic failure 10.1
21 myoclonus 10.1
22 encephalopathy 10.1

Graphical network of the top 20 diseases related to Autoimmune Autonomic Ganglionopathy:



Diseases related to Autoimmune Autonomic Ganglionopathy

Symptoms & Phenotypes for Autoimmune Autonomic Ganglionopathy

Drugs & Therapeutics for Autoimmune Autonomic Ganglionopathy

Drugs for Autoimmune Autonomic Ganglionopathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 6)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunologic Factors Phase 2, Phase 3
2 Immunoglobulins Phase 2, Phase 3
3 Antibodies Phase 2, Phase 3
4 Rho(D) Immune Globulin Phase 2, Phase 3
5 Immunoglobulins, Intravenous Phase 2, Phase 3
6 gamma-Globulins Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Evaluating the Effectiveness of Intravenous Immunoglobulin Therapy in Autoimmune Autonomic Ganglionopathy Completed NCT01522235 Phase 2, Phase 3 Double blinded IVIg
2 Concentration and Attentional Deficits in POTS and Other Autonomic Neuropathies Recruiting NCT03681080 Not Applicable
3 Evaluation of Primary Chronic Autonomic Failure Terminated NCT00059033

Search NIH Clinical Center for Autoimmune Autonomic Ganglionopathy

Genetic Tests for Autoimmune Autonomic Ganglionopathy

Anatomical Context for Autoimmune Autonomic Ganglionopathy

MalaCards organs/tissues related to Autoimmune Autonomic Ganglionopathy:

42
Eye, Lung

Publications for Autoimmune Autonomic Ganglionopathy

Articles related to Autoimmune Autonomic Ganglionopathy:

(show top 50) (show all 57)
# Title Authors Year
1
Autoimmune autonomic ganglionopathy associated with monoclonal gammopathy of undetermined significance: a case report. ( 30685800 )
2019
2
Autoimmune autonomic neuropathies: time to look beyond autoimmune autonomic ganglionopathy. ( 29305816 )
2018
3
Seronegative and seropositive autoimmune autonomic ganglionopathy (AAG): Same clinical picture, same response to immunotherapy. ( 29685292 )
2018
4
Diagnostic treatment dilemma: baroreflex failure or autoimmune autonomic ganglionopathy? ( 30151691 )
2018
5
Autoimmune autonomic ganglionopathy associated with Sjögren's syndrome presenting with recurrent abdominal distension. ( 30279247 )
2018
6
Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment. ( 30352532 )
2018
7
A case of autoimmune autonomic ganglionopathy with prolonged delirium. ( 30466057 )
2018
8
Rituximab treatment in seronegative autoimmune autonomic neuropathy and autoimmune autonomic ganglionopathy: Case-report and literature review. ( 30468952 )
2018
9
Seronegative autoimmune autonomic neuropathy: a distinct clinical entity. ( 29280036 )
2018
10
Acquired anhidrosis in a case of autoimmune autonomic ganglionopathy. ( 27774700 )
2017
11
Case of autoimmune autonomic ganglionopathy manifesting anhidrosis. ( 28620980 )
2017
12
Extra-autonomic manifestations in autoimmune autonomic ganglionopathy: a Japanese survey. ( 27837100 )
2017
13
A case of seropositive autoimmune autonomic ganglionopathy with diffuse esophageal spasm. ( 28214088 )
2017
14
Long-term cardiovascular autonomic and clinical changes after immunoglobulin G immunoadsorption therapy in autoimmune autonomic ganglionopathy. ( 28319594 )
2017
15
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy. ( 28740856 )
2017
16
Multiple Myeloma Presenting with Autoimmune Autonomic Ganglionopathy. ( 29021436 )
2017
17
Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. ( 26704065 )
2016
18
Autoimmune autonomic ganglionopathy in a pediatric patient presenting with acute encephalitis. ( 26740077 )
2016
19
Autoimmune autonomic ganglionopathy manifesting as acute-onset orthostatic hypotension in a patient undergoing peritoneal dialysis. ( 28509159 )
2016
20
Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients. ( 25790156 )
2015
21
A novel use of N-acetylcystine for the management of recurrent small bowel pseudo-obstruction in a patient with autoimmune autonomic ganglionopathy. ( 25975318 )
2015
22
Rituximab-based therapy and long-term control of autoimmune autonomic ganglionopathy. ( 26245264 )
2015
23
[A case report of chronic intestinal pseudo-obstruction with autoimmune autonomic ganglionopathy suspected from seropositive results for anti-ganglionic acetylcholine receptor antibody]. ( 25744921 )
2015
24
Transient neonatal autoimmune autonomic ganglionopathy. ( 25340087 )
2014
25
Sudomotor dysfunction in autoimmune autonomic ganglionopathy: a follow-up study. ( 22127477 )
2012
26
Autoimmune autonomic ganglionopathy with reversible cognitive impairment. ( 22158721 )
2012
27
A Unique Manifestation of Pupillary Fatigue in Autoimmune Autonomic Ganglionopathy. ( 22232207 )
2012
28
Long-term treatment with rituximab of autoimmune autonomic ganglionopathy in a patient with lymphoma. ( 21059985 )
2011
29
Autoimmune autonomic ganglionopathy after a CNS infection. ( 21210293 )
2011
30
Autoimmune autonomic ganglionopathy: a possible postganglionic neuropathy. ( 21482929 )
2011
31
Autonomic ganglia neuronal density and synaptic structure in chronic experimental autoimmune autonomic ganglionopathy. ( 21482937 )
2011
32
Seropositive myasthenia and autoimmune autonomic ganglionopathy: cross reactivity or subclinical disease? ( 21745762 )
2011
33
Coexistent autoimmune autonomic ganglionopathy and myasthenia gravis associated with non-small-cell lung cancer. ( 19882640 )
2010
34
Recent advances in autoimmune autonomic ganglionopathy. ( 20634694 )
2010
35
Isolated sympathetic failure with autoimmune autonomic ganglionopathy. ( 20837310 )
2010
36
Autoimmune autonomic ganglionopathy: treatment by plasma exchanges and rituximab. ( 19399547 )
2009
37
Efficacy of immunotherapy in seropositive and seronegative putative autoimmune autonomic ganglionopathy. ( 19506222 )
2009
38
Association of N-type calcium channel autoimmunity in patients with autoimmune autonomic ganglionopathy. ( 19541551 )
2009
39
Sudomotor dysfunction in autoimmune autonomic ganglionopathy. ( 19884578 )
2009
40
Autoimmune autonomic ganglionopathy with late-onset encephalopathy. ( 19028442 )
2009
41
Immunotherapy for autoimmune autonomic ganglionopathy. ( 19056323 )
2009
42
Autoimmune autonomic ganglionopathy with Sjögren's syndrome: significance of ganglionic acetylcholine receptor antibody and therapeutic approach. ( 19097826 )
2009
43
Antibody titers predict clinical features of autoimmune autonomic ganglionopathy. ( 19144572 )
2009
44
Clinical laboratory evaluation of autoimmune autonomic ganglionopathy: Preliminary observations. ( 19155193 )
2009
45
A Japanese case of autoimmune autonomic ganglionopathy (AAG) and a review of AAG cases in Japan. ( 19195935 )
2009
46
Special section on autoimmune autonomic ganglionopathy. Editorial. ( 19217830 )
2009
47
Autoimmune autonomic ganglionopathy. ( 19349706 )
2009
48
Combined immunomodulatory therapy in autoimmune autonomic ganglionopathy. ( 18268189 )
2008
49
Autoimmune autonomic ganglionopathy: IgG effects on ganglionic acetylcholine receptor current. ( 17536048 )
2007
50
Dysfunction of the pupillary light reflex in experimental autoimmune autonomic ganglionopathy. ( 17613283 )
2007

Variations for Autoimmune Autonomic Ganglionopathy

Expression for Autoimmune Autonomic Ganglionopathy

Search GEO for disease gene expression data for Autoimmune Autonomic Ganglionopathy.

Pathways for Autoimmune Autonomic Ganglionopathy

Pathways related to Autoimmune Autonomic Ganglionopathy according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.32 CACNA1B EPO

GO Terms for Autoimmune Autonomic Ganglionopathy

Sources for Autoimmune Autonomic Ganglionopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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