MCID: ATM024
MIFTS: 46

Autoimmune Pancreatitis

Categories: Rare diseases, Gastrointestinal diseases, Bone diseases, Immune diseases, Endocrine diseases, Genetic diseases

Aliases & Classifications for Autoimmune Pancreatitis

MalaCards integrated aliases for Autoimmune Pancreatitis:

Name: Autoimmune Pancreatitis 50 56 69
Lymphoplasmocytic Sclerosing Pancreatitis 50
Aip 56

Characteristics:

Orphanet epidemiological data:

56
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 56 ORPHA103919
UMLS via Orphanet 70 C2609129
ICD10 via Orphanet 34 K86.1

Summaries for Autoimmune Pancreatitis

NIH Rare Diseases : 50 autoimmune pancreatitis affects the pancreas, a gland behind the stomach and in front of the spine, and can also affect the bile ducts, salivary glands, kidneys, and lymph nodes. it is thought to occur when the immune system mistakenly begins to attack these healthy body tissues, glands, and organs. common signs and symptoms include painless jaundice, weight loss, and noncancerous masses in the pancreas and other organs. treatment often involves corticosteroids. the condition may recur following treatment, and require additional therapy. last updated: 2/6/2012

MalaCards based summary : Autoimmune Pancreatitis, also known as lymphoplasmocytic sclerosing pancreatitis, is related to gastric ulcer and rheumatoid arthritis. An important gene associated with Autoimmune Pancreatitis is CA2 (Carbonic Anhydrase 2), and among its related pathways/superpathways are Allograft rejection and Immune response NFAT in immune response. The drugs Secretin and Gastrointestinal Agents have been mentioned in the context of this disorder. Affiliated tissues include pancreas, salivary gland and kidney, and related phenotypes are digestive/alimentary and endocrine/exocrine gland

Wikipedia : 72 Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be... more...

Related Diseases for Autoimmune Pancreatitis

Diseases in the Pancreatitis family:

Pancreatitis, Hereditary Acute Pancreatitis
Prss1-Related Hereditary Pancreatitis Autoimmune Pancreatitis
Cftr-Related Hereditary Pancreatitis Ctrc-Related Hereditary Pancreatitis
Spink1-Related Hereditary Pancreatitis Autoimmune Pancreatitis Type 2
Autoimmune Pancreatitis Type 1 Recurrent Acute Pancreatitis

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

(show top 50) (show all 190)
id Related Disease Score Top Affiliating Genes
1 gastric ulcer 28.6 CTLA4 TNF
2 rheumatoid arthritis 27.9 CTLA4 HLA-DQB1 HLA-DRB1 TNF
3 asthma 27.8 CFTR HLA-DQB1 HLA-DRB1 IL2RA TNF
4 aip-related familial isolated pituitary adenomas 12.2
5 autoimmune pancreatitis type 1 12.0
6 autoimmune pancreatitis type 2 11.8
7 acromegaly 11.8
8 porphyria, acute intermittent 11.7
9 acute interstitial pneumonia 11.7
10 pituitary adenoma, prolactin-secreting 11.5
11 pituitary adenoma, growth hormone-secreting 11.5
12 igg4-related disease 11.1
13 mikulicz disease 11.1
14 pancreatitis 10.9
15 fruit allergy 10.5 HLA-DQB1 HLA-DRB1
16 breast hemangiopericytoma 10.5 HLA-DQB1 HLA-DRB1
17 light chain deposition disease 10.5 HLA-DQB1 HLA-DRB1
18 juvenile sialidosis type 2 10.5 HLA-DQB1 HLA-DRB1
19 idiopathic subglottic tracheal stenosis 10.5 HLA-DQB1 HLA-DRB1
20 reese retinal dysplasia 10.5 HLA-DQB1 HLA-DRB1
21 progressive myoclonus epilepsy 10.5 HLA-DQB1 HLA-DRB1
22 transient retinal arterial occlusion 10.4 HLA-DQB1 HLA-DRB1
23 ovarian surface papilloma 10.4 HLA-DQB1 HLA-DRB1
24 pellagra like syndrome 10.4 HLA-DQB1 HLA-DRB1
25 clear cell adenocarcinoma of ovary 10.4 HLA-DQB1 HLA-DRB1
26 papillary squamous carcinoma 10.4 HLA-DQB1 HLA-DRB1
27 reproductive system disease 10.4 HLA-DQB1 HLA-DRB1
28 mycobacterium kansasii 10.4 CFTR TNF
29 oculogyric crisis 10.4 HLA-DQB1 HLA-DRB1
30 idiopathic dilatation of the pulmonary artery 10.4 HLA-DQB1 TNF
31 migraine with brainstem aura 10.4 HLA-DRB1 TNF
32 alport syndrome-intellectual disability-midface hypoplasia-elliptocytosis syndrome 10.4 HLA-DRB1 IL2RA
33 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 10.4 HLA-DQB1 TNF
34 infantile hypotonia 10.3 HLA-DQB1 HLA-DRB1
35 epstein-barr virus hepatitis 10.3 HLA-DRB1 TNF
36 endocarditis 10.3 HLA-DQB1 TNF
37 moved to 244450 10.3 HLA-DRB1 TNF
38 riboflavin transporter deficiency 10.3 HLA-DRB1 TNF
39 central nervous system leiomyosarcoma 10.3 HLA-DRB1 TNF
40 cirrhotic cardiomyopathy 10.3 CFTR PRSS1
41 pancreatic cancer 10.3
42 aseptic meningitis 10.3 HLA-DRB1 TNF
43 ampulla of vater neoplasm 10.3 HLA-DQB1 TNF
44 cerebral sarcoidosis 10.3 HLA-DRB1 IL2RA
45 epidermolysis bullosa simplex superficialis 10.3 HLA-DQB1 HLA-DRB1
46 crohn's colitis 10.3 IL2RA TNF
47 hepadnavirus infection 10.3 HLA-DRB1 TNF
48 classic hairy cell leukemia 10.3 HLA-DQB1 HLA-DRB1
49 cholangitis 10.3
50 human monocytic ehrlichiosis 10.3 HLA-DQB1 TNF

Graphical network of the top 20 diseases related to Autoimmune Pancreatitis:



Diseases related to Autoimmune Pancreatitis

Symptoms & Phenotypes for Autoimmune Pancreatitis

MGI Mouse Phenotypes related to Autoimmune Pancreatitis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.63 CA2 CFTR CTLA4 HLA-DQB1 IL2RA TNF
2 endocrine/exocrine gland MP:0005379 9.43 CA2 CFTR CTLA4 HLA-DQB1 IL2RA TNF
3 respiratory system MP:0005388 9.02 CFTR CTLA4 HLA-DQB1 IL2RA TNF

Drugs & Therapeutics for Autoimmune Pancreatitis

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

(show all 19)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Secretin Approved, Investigational Phase 4 108153-74-8
2 Gastrointestinal Agents Phase 4
3 Hormone Antagonists Phase 4
4 Hormones Phase 4
5 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
6
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
7 Antibodies Phase 1, Phase 2
8 Antirheumatic Agents Phase 1, Phase 2
9 Immunoglobulins Phase 1, Phase 2
10
Lenalidomide Approved Phase 1 191732-72-6 216326
11 Angiogenesis Inhibitors Phase 1
12 Angiogenesis Modulating Agents Phase 1
13 Anti-Bacterial Agents Phase 1
14 Anti-Infective Agents Phase 1
15 Immunosuppressive Agents Phase 1
16
Pancrelipase Approved 53608-75-6
17
Prednisone Approved, Vet_approved 53-03-2 5865
18 Liver Extracts
19 pancreatin

Interventional clinical trials:

(show all 13)

id Name Status NCT ID Phase Drugs
1 Efficacy of Secretin MRCP in the Diagnosis and Follow up of Auto Immune Pancreatitis Unknown status NCT01845467 Phase 4
2 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
3 Evaluation of a New EUS Guided Biopsy Needle (SharkCore) Comparing to Standard EUS Needle (ProCore) Withdrawn NCT02766842 Phase 2
4 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
5 EUS-guided Procore-biopsy in Diagnosing Autoimmune Pancreatitis Unknown status NCT01774513
6 Follow-up Study of Complications of Acute Pancreatitis Unknown status NCT02305914
7 Pancreatic Duct Evaluation in Autoimmune Pancreatitis: MR Pancreatography Completed NCT01773031
8 Evaluation of EUS-guided FNA for Diagnosing Autoimmune Pancreatitis Completed NCT03241797
9 Genetic Analysis for Predicting of Relapse During Steroid Treatment for Autoimmune Pancreatitis (AIP) Completed NCT00444444
10 CT Pancreas Perfusion Completed NCT00588367
11 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Recruiting NCT02797665 corticosteroids
12 A Randomized Prospective Comparison of the New EUS Guided Acquire TM Needle Biopsy vs EUS Guided Fine Needle Aspiration for Suspected Solid Gastrointestinal Lesions Recruiting NCT03109639
13 Searching the Best Prognostic Factor in Out Come Evaluation in Patients With Acute Pancreatitis Admitted at Assiut University Hospitals Not yet recruiting NCT03259880

Search NIH Clinical Center for Autoimmune Pancreatitis

Genetic Tests for Autoimmune Pancreatitis

Anatomical Context for Autoimmune Pancreatitis

MalaCards organs/tissues related to Autoimmune Pancreatitis:

39
Pancreas, Salivary Gland, Kidney, Lymph Node, T Cells, Liver, Bone

Publications for Autoimmune Pancreatitis

Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 668)
id Title Authors Year
1
Autoimmune Pancreatitis Complicated With Pancreatic Ascites, Pancreatic Ductal Leakage, and Multiple Pseudocyst. ( 27977636 )
2017
2
A case of pancreatic intraepithelial neoplasia-3 with autoimmune pancreatitis. ( 28579586 )
2017
3
A Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD). ( 28736430 )
2017
4
Endoscopic Ultrasonography-Guided Fine Needle Aspiration Can Be Used to Rule Out Malignancy in Autoimmune Pancreatitis Patients. ( 28670760 )
2017
5
Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. ( 28601845 )
2017
6
A Case of Autoimmune Pancreatitis Presenting as a Deterioration in Glycaemic Control in a Patient with Pre-Existing Type 2 Diabetes. ( 28737310 )
2017
7
Autoimmune pancreatitis: Imaging features. ( 28836516 )
2017
8
Diagnosis and Management of Autoimmune Pancreatitis. ( 28842855 )
2017
9
Utility of contrast-enhanced harmonic EUS for evaluating theA effects of steroid therapy in a case of immunoglobulin G4-negative focal autoimmune pancreatitis. ( 28673642 )
2017
10
Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. ( 28921377 )
2017
11
IL-8 Expression in Granulocytic Epithelial Lesions of Idiopathic Duct-centric Pancreatitis (Type 2 Autoimmune Pancreatitis). ( 28614208 )
2017
12
Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease. ( 28634132 )
2017
13
Pancreatic pseudocyst localized in the gastric wall after EUS-guided FNA of type 1 autoimmune pancreatitis. ( 28583768 )
2017
14
Maintenance therapy in autoimmune pancreatitis: a weak light into the darkness. ( 28936461 )
2017
15
Microscopic findings in EUS-guided fine needle (SharkCore) biopsies with type 1 and type 2 autoimmune pancreatitis. ( 28868649 )
2017
16
Development of autoimmune pancreatitis is independent of CDKN1A/p21-mediated pancreatic inflammation. ( 28774888 )
2017
17
Immunoglobulin G4-related Coronary Periarteritis and Luminal Stenosis in a Patient with a History of Autoimmune Pancreatitis. ( 28824056 )
2017
18
The Role of Endoscopic Ultrasound in the Diagnosis of Autoimmune Pancreatitis. ( 28918803 )
2017
19
Fifteen-Year-Old Male with Type 2 Autoimmune Pancreatitis: An Argument for Endoscopy. ( 28626379 )
2017
20
Pancreatic Duct in Autoimmune Pancreatitis: Intraindividual Comparison of Magnetic Resonance Pancreatography at 1.5 T and 3.0 T. ( 28697133 )
2017
21
Features of Autoimmune Pancreatitis Associated With Inflammatory Bowel Diseases. ( 28782667 )
2017
22
Low-dose maintenance steroid treatment could reduce the relapse rate in patients with type 1 autoimmune pancreatitis: a long-term Japanese multicenter analysis of 510 patients. ( 28062947 )
2017
23
Autoimmune Pancreatitis:Typing,Diagnosis,and Treatment. ( 28065243 )
2016
24
Eosinophilic annular erythema in a patient with autoimmune pancreatitis: Nicotinamide therapy may be beneficial for achieving remission. ( 27129768 )
2016
25
Prospective multicenter study on the usefulness of EUS-guided FNAA biopsy for the diagnosis of autoimmune pancreatitis. ( 26777565 )
2016
26
Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting. ( 27107633 )
2016
27
Identification of Novel Serum Autoantibodies for Differential Diagnosis of Autoimmune Pancreatitis and Pancreatic Ductal Adenocarcinoma. ( 27623556 )
2016
28
Clinical and pathophysiological issues associated with type 1 autoimmune pancreatitis. ( 26864047 )
2016
29
Autoimmune Pancreatitis: An Update on Diagnosis and Management. ( 26895679 )
2016
30
Role of contrast harmonic endoscopic ultrasonography in other pancreatic solid lesions: Neuroendocrine tumors, autoimmune pancreatitis and metastases. ( 28000628 )
2016
31
Dilemmas in autoimmune pancreatitis. Surgical resection or not? ( 27546699 )
2016
32
Diagnosis of autoimmune pancreatitis by endoscopic ultrasonography -guided fine-needle aspiration using a 22-gauge needle: a prospective multicenter study. ( 27068878 )
2016
33
Nonoverlapping roles of PD-1 and FoxP3 in maintaining immune tolerance in a novel autoimmune pancreatitis mouse model. ( 27410049 )
2016
34
Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations. ( 27141136 )
2016
35
Specific MAPK-Associated MicroRNAs in Serum Differentiate Pancreatic Cancer from Autoimmune Pancreatitis. ( 27380024 )
2016
36
Why is one arm stronger than two arms? IgG4 antibodies in IgG4-related autoimmune pancreatitis. ( 27196569 )
2016
37
Autoimmune Pancreatitis and IgG4 Related Disease in Three Children. ( 27622194 )
2016
38
Preventing disease relapses in autoimmune pancreatitis with maintenance steroids: are we there yet? ( 27707776 )
2016
39
Is autoimmune pancreatitis a subclass of chronic pancreatitis? ( 28034552 )
2016
40
Relapsing sepsis episodes of Escherichia coli with CTX-M ESBL or derepressed ampC genes in a patient with chronic autoimmune pancreatitis complicated by IgG4 hypergammaglobulinaemia. ( 26862435 )
2016
41
In vitro studies implicate an imbalanced activation of dendritic cells in the pathogenesis of murine autoimmune pancreatitis. ( 27356751 )
2016
42
Correction: Investigation of Susceptibility Genes Triggering Lachrymal/Salivary Gland Lesion Complications in Japanese Patients with Type 1 Autoimmune Pancreatitis. ( 26731729 )
2016
43
Type 1 autoimmune pancreatitis: case scenario and review of the disease. ( 27716763 )
2016
44
Mass-forming type 1 autoimmune pancreatitis mimicking pancreatic cancer. ( 27121246 )
2016
45
Epidemiology, clinical presentation, diagnosis and treatment of autoimmune pancreatitis: A retrospective analysis of 53 patients. ( 26694138 )
2016
46
A Pitfall to Avoid: Hyperlipasemia, Azathioprine, and Autoimmune Pancreatitis. ( 27385401 )
2016
47
An Unusual Presentation of Pediatric Autoimmune Pancreatitis. ( 26752256 )
2016
48
Sequential Evaluation of Pancreato-Biliary Findings in a Case with IgG4-Associated Cholangiopathy and Autoimmune Pancreatitis during Corticosteroid Treatment. ( 27606144 )
2016
49
Autoimmune pancreatitis or pancreatic cancer? ( 27048909 )
2016
50
Localized (18)F-fluorodeoxyglucose uptake at the pancreatic head during remission phase of autoimmune pancreatitis: A case report. ( 27602112 )
2016

Variations for Autoimmune Pancreatitis

Expression for Autoimmune Pancreatitis

Search GEO for disease gene expression data for Autoimmune Pancreatitis.

Pathways for Autoimmune Pancreatitis

Pathways related to Autoimmune Pancreatitis according to GeneCards Suite gene sharing:

(show all 23)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.4 CTLA4 HLA-DQB1 HLA-DRB1 IL2RA TNF
2
Show member pathways
12.37 HLA-DQB1 HLA-DRB1 IL2RA TNF
3
Show member pathways
12.32 HLA-DQB1 HLA-DRB1 IL2RA TNF
4 12.28 CTLA4 IL2RA TNF
5
Show member pathways
12.25 HLA-DQB1 HLA-DRB1 IL2RA PRSS1 TNF
6 12.08 HLA-DQB1 HLA-DRB1 IL2RA TNF
7
Show member pathways
12.01 HLA-DQB1 HLA-DRB1 TNF
8
Show member pathways
12 CTLA4 HLA-DQB1 HLA-DRB1 IL2RA
9 11.98 HLA-DQB1 HLA-DRB1 TNF
10
Show member pathways
11.95 CTLA4 HLA-DQB1 HLA-DRB1
11 11.86 CTLA4 HLA-DQB1 HLA-DRB1
12 11.65 CA2 CFTR PRSS1
13
Show member pathways
11.55 HLA-DQB1 HLA-DRB1
14
Show member pathways
11.51 CTLA4 IL2RA TNF
15 11.46 CTLA4 HLA-DQB1 HLA-DRB1
16 11.43 CTLA4 IL2RA TNF
17 11.36 HLA-DQB1 HLA-DRB1
18 11.25 CA2 TNF
19 11.24 HLA-DQB1 HLA-DRB1 IL2RA TNF
20
Show member pathways
11.19 IL2RA TNF
21 11.05 HLA-DRB1 TNF
22 11.01 HLA-DRB1 IL2RA
23 10.88 CTLA4 HLA-DQB1 HLA-DRB1 TNF

GO Terms for Autoimmune Pancreatitis

Cellular components related to Autoimmune Pancreatitis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.95 CA2 CFTR CTLA4 HLA-DQB1 HLA-DRB1 IL2RA
2 lysosomal membrane GO:0005765 9.61 CFTR HLA-DQB1 HLA-DRB1
3 ER to Golgi transport vesicle membrane GO:0012507 9.48 HLA-DQB1 HLA-DRB1
4 transport vesicle membrane GO:0030658 9.46 HLA-DQB1 HLA-DRB1
5 cell surface GO:0009986 9.46 CFTR HLA-DRB1 IL2RA TNF
6 endosome membrane GO:0010008 9.43 CFTR HLA-DQB1 HLA-DRB1
7 clathrin-coated endocytic vesicle membrane GO:0030669 9.37 HLA-DQB1 HLA-DRB1
8 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.32 HLA-DQB1 HLA-DRB1
9 MHC class II protein complex GO:0042613 8.96 HLA-DQB1 HLA-DRB1
10 external side of plasma membrane GO:0009897 8.92 CTLA4 HLA-DRB1 IL2RA TNF

Biological processes related to Autoimmune Pancreatitis according to GeneCards Suite gene sharing:

(show all 12)
id Name GO ID Score Top Affiliating Genes
1 bicarbonate transport GO:0015701 9.51 CA2 CFTR
2 T cell costimulation GO:0031295 9.5 CTLA4 HLA-DQB1 HLA-DRB1
3 antigen processing and presentation GO:0019882 9.49 HLA-DQB1 HLA-DRB1
4 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.48 CFTR HLA-DRB1
5 positive regulation of osteoclast differentiation GO:0045672 9.46 CA2 TNF
6 negative regulation of immune response GO:0050777 9.43 CTLA4 IL2RA
7 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.4 HLA-DQB1 HLA-DRB1
8 inflammatory response to antigenic stimulus GO:0002437 9.37 HLA-DRB1 IL2RA
9 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.26 HLA-DQB1 HLA-DRB1
10 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 9.16 HLA-DQB1 HLA-DRB1
11 negative regulation of T cell proliferation GO:0042130 9.13 CTLA4 HLA-DRB1 IL2RA
12 immune response GO:0006955 9.02 CTLA4 HLA-DQB1 HLA-DRB1 IL2RA TNF

Molecular functions related to Autoimmune Pancreatitis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-DQB1 HLA-DRB1
2 MHC class II receptor activity GO:0032395 8.62 HLA-DQB1 HLA-DRB1

Sources for Autoimmune Pancreatitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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