MCID: ATM074
MIFTS: 19

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 20
Autoimmune Autonomic Neuropathy 20

Classifications:



Summaries for Autoimmune Autonomic Ganglionopathy

GARD : 20 Autoimmune autonomic ganglionopathy (AAG) is a condition in which the body's immune system mistakenly attacks and damages certain parts of the autonomic nervous system. AAG may be divided into two different types based on the presence of specific types of cells in the blood that normally fight infection ( antibodies ). Signs and symptoms of AAG usually begin in adulthood and are different from person to person. Symptoms may include severe orthostatic hypotension (low blood pressure upon standing), fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes. The underlying cause of AAG is poorly understood. Diagnosis is based on the symptoms, clinical exam, and specialized laboratory tests. 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 is no standard treatment. Treatment options include plasmapheresis, intravenous (IV) immunoglobulin, corticosteroids, or immunosuppressive drugs. Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete.

MalaCards based summary : Autoimmune Autonomic Ganglionopathy, also known as autoimmune autonomic neuropathy, is related to autonomic dysfunction 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 Rho(D) Immune Globulin and Immunologic Factors have been mentioned in the context of this disorder.

Wikipedia : 73 Autoimmune autonomic ganglionopathy (AAG) is a rare form of dysautonomia in which the patient's immune... more...

Related Diseases for Autoimmune Autonomic Ganglionopathy

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 Rho(D) Immune Globulin Phase 2, Phase 3
2 Immunologic Factors Phase 2, Phase 3
3 Immunoglobulins, Intravenous Phase 2, Phase 3
4 gamma-Globulins Phase 2, Phase 3
5 Immunoglobulins
6 Antibodies

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Double-blind, Randomized, Placebo-controlled Trial to Evaluate the Efficacy of Intravenous Immunoglobulin Therapy in Autoimmune Autonomic Ganglionopathy. Completed NCT01522235 Phase 2, Phase 3 Double blinded IVIg
2 Autonomic Neuropathy and Its Pathophysiology in Autoimmune Autonomic Neuropathies, Postural Orthostatic Tachycardia Syndrome and Ehlers Danlos Syndromes: Peripheral Autonomic Small Fiber Neuropathy or Central Autonomic Failure? Recruiting NCT04310644

Search NIH Clinical Center for Autoimmune Autonomic Ganglionopathy

Genetic Tests for Autoimmune Autonomic Ganglionopathy

Anatomical Context for Autoimmune Autonomic Ganglionopathy

Publications for Autoimmune Autonomic Ganglionopathy

Articles related to Autoimmune Autonomic Ganglionopathy:

(show top 50) (show all 118)
# Title Authors PMID Year
1
Multimodal Biomarkers Quantify Recovery in Autoimmune Autonomic Ganglionopathy. 61
33438240 2021
2
Role of Plasma Exchange for Autoimmune Autonomic Ganglionopathy (AAG): A Case Report. 61
33686692 2021
3
Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies. 61
33583893 2021
4
Autonomic neuropathies. 61
32926436 2021
5
Sudomotor Dysfunction. 61
32906168 2020
6
Chronic autoimmune autonomic ganglionopathy. A rare case of dysautonomia. 61
32900526 2020
7
A comprehensive analysis of the clinical characteristics and laboratory features in 179 patients with autoimmune autonomic ganglionopathy. 61
31924415 2020
8
Autoimmune Autonomic Disorders. 61
31996621 2020
9
Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases. 61
32079137 2020
10
Stiff Person Syndrome and Acetylcholine Receptor Ganglionic Neuronal Antibodies. 61
32095128 2020
11
[Autoimmune autonomic ganglionopathy]. 61
31761837 2019
12
Overnight loss of pigmented hair in autoimmune autonomic neuropathy treated with IVIg. 61
31554670 2019
13
Seronegative autoimmune autonomic ganglionopathy from dual immune checkpoint inhibition in a patient with metastatic melanoma. 61
31623673 2019
14
Autoimmune autonomic ganglionopathy associated with monoclonal gammopathy of undetermined significance: a case report. 61
30685800 2019
15
Autoimmune autonomic neuropathies and ganglionopathies: epidemiology, pathophysiology, and therapeutic advances. 61
31093868 2019
16
Rituximab treatment in seronegative autoimmune autonomic neuropathy and autoimmune autonomic ganglionopathy: Case-report and literature review. 61
30468952 2019
17
A case of autoimmune autonomic ganglionopathy with prolonged delirium. 61
30466057 2019
18
Diagnostic treatment dilemma: baroreflex failure or autoimmune autonomic ganglionopathy? 61
30151691 2018
19
Anti-ganglionic AChR antibodies in Japanese patients with motility disorders. 61
29766276 2018
20
Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment. 61
30352532 2018
21
Ganglionic Antibody Level as a Predictor of Severity of Autonomic Failure. 61
30170741 2018
22
Autoimmune autonomic ganglionopathy associated with Sjögren's syndrome presenting with recurrent abdominal distension. 61
30279247 2018
23
Recurrent autonomic and sensory neuropathy in a patient with anti-ganglionic acetylcholine receptor antibodies. 61
30211328 2018
24
Seronegative and seropositive autoimmune autonomic ganglionopathy (AAG): Same clinical picture, same response to immunotherapy. 61
29685292 2018
25
[Anti-Ganglionic Acetylcholine Receptor Antibodies, Autoimmune Autonomic Ganglionopathy, and Related Disorders]. 61
29632286 2018
26
Seronegative autoimmune autonomic neuropathy: a distinct clinical entity. 61
29280036 2018
27
Autoimmune autonomic neuropathies: time to look beyond autoimmune autonomic ganglionopathy. 61
29305816 2018
28
Clinical Problem Solving: A Tobacco Merchant Who Can't Spit. 61
29235746 2017
29
Multiple Myeloma Presenting with Autoimmune Autonomic Ganglionopathy. 61
29021436 2017
30
Case of autoimmune autonomic ganglionopathy manifesting anhidrosis. 61
28620980 2017
31
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy. 61
28740856 2017
32
Long-term cardiovascular autonomic and clinical changes after immunoglobulin G immunoadsorption therapy in autoimmune autonomic ganglionopathy. 61
28319594 2017
33
A case of seropositive autoimmune autonomic ganglionopathy with diffuse esophageal spasm. 61
28214088 2017
34
Extra-autonomic manifestations in autoimmune autonomic ganglionopathy: a Japanese survey. 61
27837100 2017
35
Acquired anhidrosis in a case of autoimmune autonomic ganglionopathy. 61
27774700 2017
36
[The interface between the immune system and autonomic nervous system]. 61
29238017 2017
37
Pediatric Anti-ganglionic Antibody Positive Autonomic Neuropathy: Clinical Presentation and Response to Treatment. 61
27569256 2016
38
Autoimmune autonomic ganglionopathy in a pediatric patient presenting with acute encephalitis. 61
26740077 2016
39
Autoimmune autonomic ganglionopathy manifesting as acute-onset orthostatic hypotension in a patient undergoing peritoneal dialysis. 61
28509159 2016
40
Autoimmune autonomic disorders. 61
27112689 2016
41
Association between Anti-Ganglionic Nicotinic Acetylcholine Receptor (gAChR) Antibodies and HLA-DRB1 Alleles in the Japanese Population. 61
26807576 2016
42
Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. 61
26704065 2016
43
[Acute Sensory Neuropathies and Acute Autonomic Neuropathies]. 61
26560953 2015
44
Clinical experience of seropositive ganglionic acetylcholine receptor antibody in a tertiary neurology referral center. 61
25557122 2015
45
[(Autoimmune autonomic ganglionopathy: AAG)]. 61
26480742 2015
46
Rituximab-based therapy and long-term control of autoimmune autonomic ganglionopathy. 61
26245264 2015
47
A novel use of N-acetylcystine for the management of recurrent small bowel pseudo-obstruction in a patient with autoimmune autonomic ganglionopathy. 61
25975318 2015
48
[A case report of chronic intestinal pseudo-obstruction with autoimmune autonomic ganglionopathy suspected from seropositive results for anti-ganglionic acetylcholine receptor antibody]. 61
25744921 2015
49
Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients. 61
25790156 2015
50
Transient neonatal autoimmune autonomic ganglionopathy. 61
25340087 2014

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 EPO CACNA1B

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
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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