MCID: ATM024
MIFTS: 49

Autoimmune Pancreatitis

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

Aliases & Classifications for Autoimmune Pancreatitis

MalaCards integrated aliases for Autoimmune Pancreatitis:

Name: Autoimmune Pancreatitis 49 55 69
Lymphoplasmocytic Sclerosing Pancreatitis 49
Aip 55

Characteristics:

Orphanet epidemiological data:

55
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 55 ORPHA103919
UMLS via Orphanet 70 C2609129
ICD10 via Orphanet 33 K86.1
UMLS 69 C2609129

Summaries for Autoimmune Pancreatitis

NIH Rare Diseases : 49 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 sclerosing cholangitis and cholangitis. An important gene associated with Autoimmune Pancreatitis is CA2 (Carbonic Anhydrase 2), and among its related pathways/superpathways are Allograft rejection and Influenza A. The drugs rituximab and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include pancreas, salivary gland and kidney, and related phenotypes are digestive/alimentary and respiratory system

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
Autoimmune 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 188)
# Related Disease Score Top Affiliating Genes
1 sclerosing cholangitis 30.4 CFTR HLA-DRB1 TNF
2 cholangitis 30.1 CA2 CFTR HLA-DRB1 TNF
3 sialadenitis 29.9 HLA-DQB1 TNF
4 primary biliary cirrhosis 29.4 CA2 CTLA4 HLA-DRB1
5 autoimmune hepatitis 29.3 CTLA4 HLA-DQB1 HLA-DRB1
6 celiac disease 1 29.2 CTLA4 HLA-DQB1 TNF
7 inflammatory bowel disease 29.0 CTLA4 HLA-DQB1 HLA-DRB1 TNF
8 diabetes mellitus 28.9 CTLA4 HLA-DQB1 HLA-DRB1 TNF
9 asthma 28.7 CFTR HLA-DQB1 HLA-DRB1 TNF
10 arthritis 28.6 FCRL3 HLA-DQB1 HLA-DRB1 TNF
11 autoimmune disease 28.3 CTLA4 FCRL3 HLA-DQB1 HLA-DRB1 TNF
12 rheumatoid arthritis 27.9 CTLA4 FCRL3 HLA-DQB1 HLA-DRB1 TNF
13 aip-related familial isolated pituitary adenomas 12.1
14 autoimmune pancreatitis type 1 12.1
15 autoimmune pancreatitis type 2 12.1
16 porphyria, acute intermittent 12.0
17 acromegaly 11.9
18 pituitary adenoma 1, multiple types 11.8
19 acute interstitial pneumonia 11.8
20 pituitary adenoma, prolactin-secreting 11.8
21 igg4-related disease 11.2
22 igg4-related dacryoadenitis and sialadenitis 11.2
23 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 11.0
24 pancreatitis 11.0
25 pancreatic cancer 10.4
26 limbic encephalitis with lgi1 antibodies 10.3 HLA-DQB1 HLA-DRB1
27 metal allergy 10.3 HLA-DQB1 HLA-DRB1
28 focal epithelial hyperplasia 10.3 HLA-DQB1 HLA-DRB1
29 lichen planopilaris 10.3 HLA-DQB1 HLA-DRB1
30 type ii mixed cryoglobulinemia 10.3 HLA-DQB1 HLA-DRB1
31 idiopathic inflammatory myopathy 10.3 HLA-DQB1 HLA-DRB1
32 recurrent respiratory papillomatosis 10.3 HLA-DQB1 HLA-DRB1
33 pityriasis rosea 10.3 HLA-DQB1 HLA-DRB1
34 whipple disease 10.3 HLA-DQB1 HLA-DRB1
35 acute disseminated encephalomyelitis 10.3 HLA-DQB1 HLA-DRB1
36 pediatric multiple sclerosis 10.3 HLA-DQB1 HLA-DRB1
37 osteonecrosis of the jaw 10.3 HLA-DQB1 HLA-DRB1
38 cystic echinococcosis 10.2 HLA-DQB1 HLA-DRB1
39 microscopic polyangiitis 10.2 HLA-DRB1 TNF
40 peanut allergy 10.2 HLA-DQB1 HLA-DRB1
41 mycobacterium abscessus 10.2 CFTR TNF
42 hypersomnia 10.2 HLA-DQB1 HLA-DRB1
43 actinic prurigo 10.2 HLA-DRB1 TNF
44 central nervous system vasculitis 10.2 HLA-DRB1 TNF
45 rheumatoid vasculitis 10.2 HLA-DRB1 TNF
46 alveolar echinococcosis 10.2 HLA-DRB1 TNF
47 idiopathic achalasia 10.2 HLA-DQB1 TNF
48 ocular cicatricial pemphigoid 10.2 HLA-DQB1 HLA-DRB1
49 hemorrhagic fever 10.2 HLA-DRB1 TNF
50 paraneoplastic pemphigus 10.2 HLA-DQB1 HLA-DRB1

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.35 CA2 CFTR CTLA4 HLA-DQB1 TNF
2 respiratory system MP:0005388 9.02 CA2 CFTR CTLA4 HLA-DQB1 TNF

Drugs & Therapeutics for Autoimmune Pancreatitis

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

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

Interventional clinical trials:

(show all 12)

# Name Status NCT ID Phase Drugs
1 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
2 Evaluation of a New EUS Guided Biopsy Needle (SharkCore) Comparing to Standard EUS Needle (ProCore) Withdrawn NCT02766842 Phase 2
3 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
4 EUS-guided Procore-biopsy in Diagnosing Autoimmune Pancreatitis Unknown status NCT01774513
5 Follow-up Study of Complications of Acute Pancreatitis Unknown status NCT02305914
6 Pancreatic Duct Evaluation in Autoimmune Pancreatitis: MR Pancreatography Completed NCT01773031
7 Evaluation of EUS-guided FNA for Diagnosing Autoimmune Pancreatitis Completed NCT03241797
8 Genetic Analysis for Predicting of Relapse During Steroid Treatment for Autoimmune Pancreatitis (AIP) Completed NCT00444444
9 CT Pancreas Perfusion Completed NCT00588367
10 A Randomized Prospective Comparison of the New EUS Guided Acquire TM Needle Biopsy vs EUS Guided Fine Needle Aspiration for Suspected Solid Gastrointestinal Lesions Completed NCT03109639
11 Efficacy of Secretin MRCP in the Diagnosis and Follow up of Auto Immune Pancreatitis Recruiting NCT01845467
12 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Recruiting NCT02797665 corticosteroids

Search NIH Clinical Center for Autoimmune Pancreatitis

Genetic Tests for Autoimmune Pancreatitis

Anatomical Context for Autoimmune Pancreatitis

MalaCards organs/tissues related to Autoimmune Pancreatitis:

38
Pancreas, Salivary Gland, Kidney, Lymph Node, T Cells, Liver, Bone

Publications for Autoimmune Pancreatitis

Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 689)
# Title Authors Year
1
Focal Autoimmune Pancreatitis Mimicking Pancreatic Cancer on FDG PET/CT Imaging. ( 29189377 )
2018
2
Endoscopic ultrasound guided 22 gauge core needle biopsy for the diagnosis of Autoimmune pancreatitis. ( 29338920 )
2018
3
Differences in Gut Microbiota Profiles between Autoimmune Pancreatitis and Chronic Pancreatitis. ( 29434076 )
2018
4
Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review. ( 29332510 )
2018
5
The Role of Endoscopic Ultrasound-guided Drainage for Autoimmune Pancreatitis-associated Pancreatic Cysts: A Report of Five Cases and a Literature Review. ( 29434161 )
2018
6
Serum Immunoglobulin G4 in Discriminating Autoimmune Pancreatitis From Pancreatic Cancer: A Diagnostic Meta-analysis. ( 29351121 )
2018
7
Monitoring and predicting disease activity in autoimmune pancreatitis with the M-ANNHEIM-AiP-Activity-Score. ( 29158146 )
2018
8
Adoptive transfer of CD3<sup>+</sup>T cells and CD4<sup>+</sup>CD44<sup>high</sup>memory T cells induces autoimmune pancreatitis in MRL/MpJ mice. ( 29383850 )
2018
9
Clinical course of type 1 autoimmune pancreatitis patients without steroid treatment: a Japanese multicenter study of 97 patients. ( 29430861 )
2018
10
Relapse rate and predictors of relapse in a large single center cohort of type 1 autoimmune pancreatitis: long-term follow-up results after steroid therapy with short-duration maintenance treatment. ( 29362937 )
2018
11
The clinical utility of immunoglobulin G4 in the evaluation of autoimmune pancreatitis and pancreatic adenocarcinoma. ( 29033025 )
2018
12
Endoscopic Ultrasonography-Guided Fine Needle Aspiration Can Be Used to Rule Out Malignancy in Autoimmune Pancreatitis Patients. ( 28670760 )
2017
13
Autoimmune pancreatitis: Imaging features. ( 28836516 )
2017
14
Immunoglobulin G4-related Coronary Periarteritis and Luminal Stenosis in a Patient with a History of Autoimmune Pancreatitis. ( 28824056 )
2017
15
IL-8 Expression in Granulocytic Epithelial Lesions of Idiopathic Duct-centric Pancreatitis (Type 2 Autoimmune Pancreatitis). ( 28614208 )
2017
16
Features of Autoimmune Pancreatitis Associated With Inflammatory Bowel Diseases. ( 28782667 )
2017
17
Prognosis of type 1 autoimmune pancreatitis after corticosteroid therapy-induced remission in terms of relapse and diabetes mellitus. ( 29166415 )
2017
18
Pancreatic pseudocyst localized in the gastric wall after EUS-guided FNA of type 1 autoimmune pancreatitis. ( 28583768 )
2017
19
Fifteen-Year-Old Male with Type 2 Autoimmune Pancreatitis: An Argument for Endoscopy. ( 28626379 )
2017
20
Type 2 Autoimmune Pancreatitis: A Challenge in the Differential Diagnosis of a Pancreatic Mass. ( 29255773 )
2017
21
Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. ( 28601845 )
2017
22
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
23
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
24
Chronic Fibro-Inflammatory Responses in Autoimmune Pancreatitis Depend on IFN-I+ and IL-33 Produced by Plasmacytoid Dendritic Cells. ( 28373582 )
2017
25
A case of pancreatic intraepithelial neoplasia-3 with autoimmune pancreatitis. ( 28579586 )
2017
26
Multifocal Mass Lesions in Autoimmune Pancreatitis. ( 29282391 )
2017
27
Development of autoimmune pancreatitis is independent of CDKN1A/p21-mediated pancreatic inflammation. ( 28774888 )
2017
28
Autoimmune Hemolytic Anemia Obscured by the Obstructive Jaundice Associated with IgG4-related Sclerosing Cholangitis in a Patient with Type 1 Autoimmune Pancreatitis: A Case Report and Review of the Literature. ( 29269682 )
2017
29
Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. ( 28921377 )
2017
30
Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease. ( 28634132 )
2017
31
IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. ( 29196804 )
2017
32
Autoimmune Pancreatitis Type II: First Report from India. ( 29207780 )
2017
33
Microscopic findings in EUS-guided fine needle (SharkCore) biopsies with type 1 and type 2 autoimmune pancreatitis. ( 28868649 )
2017
34
Diagnosis and Management of Autoimmune Pancreatitis. ( 28842855 )
2017
35
<sup>18</sup> F- FDG PET/CT helps differentiate autoimmune pancreatitis from pancreatic cancer. ( 29061130 )
2017
36
The Role of Endoscopic Ultrasound in the Diagnosis of Autoimmune Pancreatitis. ( 28918803 )
2017
37
A Case of Autoimmune Pancreatitis Presenting as a Deterioration in Glycaemic Control in a Patient with Pre-Existing Type 2 Diabetes. ( 28737310 )
2017
38
Autoimmune Pancreatitis Complicated With Pancreatic Ascites, Pancreatic Ductal Leakage, and Multiple Pseudocyst. ( 27977636 )
2017
39
Pancreatic Duct in Autoimmune Pancreatitis: Intraindividual Comparison of Magnetic Resonance Pancreatography at 1.5 T and 3.0 T. ( 28697133 )
2017
40
Maintenance therapy in autoimmune pancreatitis: a weak light into the darkness. ( 28936461 )
2017
41
Autoimmune Pancreatitis Type 2: Case Report. ( 29051892 )
2017
42
A Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD). ( 28736430 )
2017
43
Simultaneous Immunoglobulin G4-associated Autoimmune Hepatitis and Autoimmune Pancreatitis. ( 29201784 )
2017
44
A Pitfall to Avoid: Hyperlipasemia, Azathioprine, and Autoimmune Pancreatitis. ( 27385401 )
2016
45
Autoimmune Pancreatitis: An Update on Diagnosis and Management. ( 26895679 )
2016
46
Diagnosis of autoimmune pancreatitis by endoscopic ultrasonography -guided fine-needle aspiration using a 22-gauge needle: a prospective multicenter study. ( 27068878 )
2016
47
Assessment of the Rate of Decrease in Serum IgG4 Level of Autoimmune Pancreatitis Patients in Response to Initial Steroid Therapy as a Predictor of Subsequent Relapse. ( 27171510 )
2016
48
Sensitization to and Challenge with Gliadin Induce Pancreatitis and Extrapancreatic Inflammation in HLA-DQ8 Mice: An Animal Model of Type 1 Autoimmune Pancreatitis. ( 27114422 )
2016
49
Role of contrast harmonic endoscopic ultrasonography in other pancreatic solid lesions: Neuroendocrine tumors, autoimmune pancreatitis and metastases. ( 28000628 )
2016
50
Early pancreatic volume reduction on CT predicts relapse in patients with type 1 autoimmune pancreatitis treated with steroids. ( 27465035 )
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 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.45 CTLA4 HLA-DQB1 HLA-DRB1 TNF
2
Show member pathways
12.31 HLA-DQB1 HLA-DRB1 PRSS1 TNF
3
Show member pathways
12.28 HLA-DQB1 HLA-DRB1 TNF
4 12.12 HLA-DQB1 HLA-DRB1 TNF
5
Show member pathways
12.07 CTLA4 HLA-DQB1 HLA-DRB1
6
Show member pathways
11.93 HLA-DQB1 HLA-DRB1 TNF
7 11.89 HLA-DQB1 HLA-DRB1 TNF
8
Show member pathways
11.85 CTLA4 HLA-DQB1 HLA-DRB1
9 11.71 CTLA4 HLA-DQB1 HLA-DRB1
10
Show member pathways
11.48 HLA-DQB1 HLA-DRB1
11 11.42 HLA-DQB1 HLA-DRB1 TNF
12 11.31 HLA-DQB1 HLA-DRB1
13 11.31 CA2 CFTR PRSS1
14 11.2 CA2 TNF
15 10.98 CTLA4 HLA-DQB1 HLA-DRB1
16 10.97 HLA-DRB1 TNF
17 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:

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.95 CA2 CFTR CTLA4 FCRL3 HLA-DQB1 HLA-DRB1
2 lysosomal membrane GO:0005765 9.61 CFTR HLA-DQB1 HLA-DRB1
3 external side of plasma membrane GO:0009897 9.5 CTLA4 HLA-DRB1 TNF
4 ER to Golgi transport vesicle membrane GO:0012507 9.48 HLA-DQB1 HLA-DRB1
5 transport vesicle membrane GO:0030658 9.46 HLA-DQB1 HLA-DRB1
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 cell surface GO:0009986 9.26 CFTR FCRL3 HLA-DRB1 TNF
9 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.16 HLA-DQB1 HLA-DRB1
10 MHC class II protein complex GO:0042613 8.62 HLA-DQB1 HLA-DRB1

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.62 CTLA4 HLA-DQB1 HLA-DRB1 TNF
2 bicarbonate transport GO:0015701 9.48 CA2 CFTR
3 antigen processing and presentation GO:0019882 9.46 HLA-DQB1 HLA-DRB1
4 negative regulation of T cell proliferation GO:0042130 9.43 CTLA4 HLA-DRB1
5 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.4 CFTR HLA-DRB1
6 positive regulation of osteoclast differentiation GO:0045672 9.37 CA2 TNF
7 T cell costimulation GO:0031295 9.33 CTLA4 HLA-DQB1 HLA-DRB1
8 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.26 HLA-DQB1 HLA-DRB1
9 humoral immune response mediated by circulating immunoglobulin GO:0002455 8.96 HLA-DQB1 HLA-DRB1
10 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.62 HLA-DQB1 HLA-DRB1

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

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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