MCID: ATM074
MIFTS: 22

Autoimmune Autonomic Ganglionopathy

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Autoimmune Autonomic Ganglionopathy

MalaCards integrated aliases for Autoimmune Autonomic Ganglionopathy:

Name: Autoimmune Autonomic Ganglionopathy 53
Autoimmune Autonomic Neuropathy 53

Classifications:



Summaries for Autoimmune Autonomic Ganglionopathy

NIH Rare Diseases : 53 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 anhidrosis and neuropathy. 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 Rho(D) Immune Globulin and gamma-Globulins have been mentioned in the context of this disorder. Affiliated tissues include eye and lung.

Wikipedia : 76 Autoimmune autonomic ganglionopathy (AAG) is an extremely rare form of dysautonomia in which the... 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 18)
# Related Disease Score Top Affiliating Genes
1 anhidrosis 10.1
2 neuropathy 10.1
3 small cell cancer of the lung 9.9
4 lung cancer 9.9
5 myasthenia gravis 9.9
6 myeloma, multiple 9.9
7 lymphoma 9.9
8 autonomic neuropathy 9.9
9 esophagitis 9.9
10 intestinal pseudo-obstruction 9.9
11 neuronitis 9.9
12 peritonitis 9.9
13 encephalitis 9.9
14 harlequin syndrome 9.9
15 myasthenia gravis congenital 9.9
16 opsoclonus-myoclonus syndrome 9.9
17 encephalopathy 9.9
18 myoclonus 9.9

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):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Rho(D) Immune Globulin Phase 2, Phase 3
2 gamma-Globulins Phase 2, Phase 3
3 Antibodies Phase 2, Phase 3
4 Immunoglobulins Phase 2, Phase 3
5 Immunoglobulins, Intravenous 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 Evaluation of Primary Chronic Autonomic Failure Recruiting 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:

41
Eye, Lung

Publications for Autoimmune Autonomic Ganglionopathy

Articles related to Autoimmune Autonomic Ganglionopathy:

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