MCID: ATM024
MIFTS: 50

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 53 59 73
Lymphoplasmocytic Sclerosing Pancreatitis 53
Aip 59

Characteristics:

Orphanet epidemiological data:

59
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA103919
UMLS via Orphanet 74 C2609129
ICD10 via Orphanet 34 K86.1
UMLS 73 C2609129

Summaries for Autoimmune Pancreatitis

NIH Rare Diseases : 53 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.

MalaCards based summary : Autoimmune Pancreatitis, also known as lymphoplasmocytic sclerosing pancreatitis, is related to sclerosing cholangitis and sialadenitis. An important gene associated with Autoimmune Pancreatitis is CA2 (Carbonic Anhydrase 2), and among its related pathways/superpathways are TCR Signaling (Qiagen) and Allograft rejection. The drugs rituximab and Immunoglobulin G 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 : 76 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
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 30.5 CFTR HLA-DRB1 TNF
2 sialadenitis 30.1 HLA-DQB1 TNF
3 cholangitis 30.0 CA2 CFTR HLA-DRB1 TNF
4 autoimmune hepatitis 29.1 CTLA4 HLA-DQB1 HLA-DRB1
5 arthritis 29.0 HLA-DQB1 HLA-DRB1 TNF
6 celiac disease 1 29.0 CTLA4 HLA-DQB1 TNF
7 inflammatory bowel disease 28.7 CTLA4 HLA-DQB1 HLA-DRB1 TNF
8 autoimmune disease 28.4 CTLA4 HLA-DQB1 HLA-DRB1 TNF
9 diabetes mellitus 28.4 CTLA4 HLA-DQB1 HLA-DRB1 TNF
10 asthma 28.3 CFTR HLA-DQB1 HLA-DRB1 TNF
11 rheumatoid arthritis 28.0 CTLA4 HLA-DQB1 HLA-DRB1 TNF
12 aip-related familial isolated pituitary adenomas 12.1
13 autoimmune pancreatitis type 1 12.1
14 autoimmune pancreatitis type 2 12.1
15 porphyria, acute intermittent 12.1
16 pituitary adenoma 1, multiple types 11.9
17 acromegaly 11.8
18 acute interstitial pneumonia 11.8
19 pituitary adenoma, prolactin-secreting 11.6
20 igg4-related disease 11.2
21 igg4-related dacryoadenitis and sialadenitis 11.2
22 pancreatitis 11.0
23 microscopic polyangiitis 10.5 HLA-DRB1 TNF
24 actinic prurigo 10.5 HLA-DRB1 TNF
25 microscopic colitis 10.5 HLA-DRB1 TNF
26 limbic encephalitis with lgi1 antibodies 10.5 HLA-DQB1 HLA-DRB1
27 central nervous system vasculitis 10.5 HLA-DRB1 TNF
28 rheumatoid vasculitis 10.5 HLA-DRB1 TNF
29 metal allergy 10.4 HLA-DQB1 HLA-DRB1
30 focal epithelial hyperplasia 10.4 HLA-DQB1 HLA-DRB1
31 hypersomnia 10.4 HLA-DQB1 HLA-DRB1
32 alveolar echinococcosis 10.4 HLA-DRB1 TNF
33 lichen planopilaris 10.4 HLA-DQB1 HLA-DRB1
34 hemorrhagic fever 10.4 HLA-DRB1 TNF
35 type ii mixed cryoglobulinemia 10.4 HLA-DQB1 HLA-DRB1
36 recurrent respiratory papillomatosis 10.4 HLA-DQB1 HLA-DRB1
37 pityriasis rosea 10.4 HLA-DQB1 HLA-DRB1
38 whipple disease 10.4 HLA-DQB1 HLA-DRB1
39 pancreatic cancer 10.4
40 mycobacterium abscessus 10.4 CFTR TNF
41 acute disseminated encephalomyelitis 10.4 HLA-DQB1 HLA-DRB1
42 pediatric multiple sclerosis 10.4 HLA-DQB1 HLA-DRB1
43 osteonecrosis of the jaw 10.4 HLA-DQB1 HLA-DRB1
44 palindromic rheumatism 10.4 HLA-DRB1 TNF
45 hemorrhagic fever with renal syndrome 10.4 HLA-DRB1 TNF
46 rheumatic fever 10.4 HLA-DRB1 TNF
47 peanut allergy 10.4 HLA-DQB1 HLA-DRB1
48 idiopathic neutropenia 10.4 HLA-DRB1 TNF
49 rheumatic heart disease 10.3 HLA-DRB1 TNF
50 mixed connective tissue disease 10.3 HLA-DRB1 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.73 CA2 CD4 CFTR CTLA4 HLA-DQB1 TNF
2 endocrine/exocrine gland MP:0005379 9.63 CA2 CD4 CFTR CTLA4 HLA-DQB1 TNF
3 immune system MP:0005387 9.43 CA2 CD4 CFTR CTLA4 HLA-DQB1 TNF
4 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 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
2 Immunoglobulin G Phase 1, Phase 2,Not Applicable
3 Antibodies Phase 1, Phase 2,Not Applicable
4 Immunoglobulins Phase 1, Phase 2,Not Applicable
5 Antirheumatic Agents Phase 1, Phase 2
6
Lenalidomide Approved Phase 1 191732-72-6 216326
7 Angiogenesis Inhibitors Phase 1
8 Angiogenesis Modulating Agents Phase 1
9 Anti-Bacterial Agents Phase 1
10 Immunosuppressive Agents Phase 1
11 Anti-Infective Agents Phase 1
12
Pancrelipase Approved, Investigational 53608-75-6
13
Secretin Approved, Investigational 108153-74-8
14
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
15 Liver Extracts
16 pancreatin
17 Gastrointestinal Agents
18 Hormone Antagonists
19 Hormones
20 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 Not Applicable
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 Not Applicable
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 Not Applicable
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 Not Applicable 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:

41
Pancreas, Salivary Gland, Kidney, Lymph Node, T Cells, Liver, Bone

Publications for Autoimmune Pancreatitis

Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 713)
# Title Authors Year
1
Clinical course of type 1 autoimmune pancreatitis patients without steroid treatment: a Japanese multicenter study of 97 patients. ( 29430861 )
2018
2
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
3
Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis. ( 29761833 )
2018
4
Prevalence of inflammatory bowel disease in alcoholic, non-alcoholic and autoimmune pancreatitis. ( 29734447 )
2018
5
Diagnosis and treatment of autoimmune pancreatitis. ( 29901516 )
2018
6
Comparison of clinical characteristics of radiological forms of autoimmune pancreatitis. ( 29843984 )
2018
7
Focal Autoimmune Pancreatitis Mimicking Pancreatic Cancer on FDG PET/CT Imaging. ( 29189377 )
2018
8
Comparison of diagnostic performance between CT and MRI in differentiating non-diffuse-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma. ( 29948075 )
2018
9
Monitoring and predicting disease activity in autoimmune pancreatitis with the M-ANNHEIM-AiP-Activity-Score. ( 29158146 )
2018
10
Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus from INSPPIRE. ( 29746340 )
2018
11
Autoimmune pancreatitis with concomitant autoimmune haemolytic anaemia. ( 29574435 )
2018
12
A case report of autoimmune pancreatitis associated with a pancreatic pseudocyst. ( 29742687 )
2018
13
Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review. ( 29332510 )
2018
14
Contrast-Enhanced Endoscopic Ultrasound for Differentially Diagnosing Autoimmune Pancreatitis and Pancreatic Cancer. ( 29699060 )
2018
15
Updates in the field of autoimmune pancreatitis: a clinical guide. ( 29923430 )
2018
16
Predominant Improvement of Alpha Cell Function after Steroid Therapy in a Patient with Autoimmune Pancreatitis: Case Report. ( 29725970 )
2018
17
Autoimmune Pancreatitis. ( 29910463 )
2018
18
Metastatic rhabdomyosarcoma mimicking autoimmune pancreatitis diagnosed by EUS-guided fine-needle biopsy. ( 29627494 )
2018
19
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
20
A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma. ( 29681791 )
2018
21
Endoscopic ultrasound guided 22 gauge core needle biopsy for the diagnosis of Autoimmune pancreatitis. ( 29338920 )
2018
22
The Role of Endoscopic Ultrasound-guided Drainage for Autoimmune Pancreatitis-associated Pancreatic Cysts: A Report of Five Cases and a Literature Review. ( 29434161 )
2018
23
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
24
Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent. ( 29610171 )
2018
25
Serum Immunoglobulin G4 in Discriminating Autoimmune Pancreatitis From Pancreatic Cancer: A Diagnostic Meta-analysis. ( 29351121 )
2018
26
Qualitative and quantitative evaluation for morphological changes of the splenic artery in autoimmune pancreatitis: novel imaging findings for differentiation from pancreatic adenocarcinoma. ( 29948059 )
2018
27
The clinical utility of immunoglobulin G4 in the evaluation of autoimmune pancreatitis and pancreatic adenocarcinoma. ( 29033025 )
2018
28
Differences in Gut Microbiota Profiles between Autoimmune Pancreatitis and Chronic Pancreatitis. ( 29434076 )
2018
29
Serum apolipoprotein A2 isoforms in autoimmune pancreatitis. ( 29481802 )
2018
30
Hypothyroidism in patients with autoimmune pancreatitis. ( 29736303 )
2018
31
A Case of Autoimmune Pancreatitis Presenting as a Deterioration in Glycaemic Control in a Patient with Pre-Existing Type 2 Diabetes. ( 28737310 )
2017
32
Development of autoimmune pancreatitis is independent of CDKN1A/p21-mediated pancreatic inflammation. ( 28774888 )
2017
33
Multifocal Mass Lesions in Autoimmune Pancreatitis. ( 29282391 )
2017
34
Fifteen-Year-Old Male with Type 2 Autoimmune Pancreatitis: An Argument for Endoscopy. ( 28626379 )
2017
35
Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease. ( 28634132 )
2017
36
Autoimmune pancreatitis: Imaging features. ( 28836516 )
2017
37
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
38
Pancreatic Duct in Autoimmune Pancreatitis: Intraindividual Comparison of Magnetic Resonance Pancreatography at 1.5 T and 3.0 T. ( 28697133 )
2017
39
IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. ( 29196804 )
2017
40
Autoimmune Pancreatitis Type II: First Report from India. ( 29207780 )
2017
41
Immunoglobulin G4-related Coronary Periarteritis and Luminal Stenosis in a Patient with a History of Autoimmune Pancreatitis. ( 28824056 )
2017
42
Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. ( 28921377 )
2017
43
Type 2 Autoimmune Pancreatitis: A Challenge in the Differential Diagnosis of a Pancreatic Mass. ( 29255773 )
2017
44
Chronic Fibro-Inflammatory Responses in Autoimmune Pancreatitis Depend on IFN-I+ and IL-33 Produced by Plasmacytoid Dendritic Cells. ( 28373582 )
2017
45
A case of pancreatic intraepithelial neoplasia-3 with autoimmune pancreatitis. ( 28579586 )
2017
46
Autoimmune Pancreatitis Type 2: Case Report. ( 29051892 )
2017
47
<sup>18</sup> F- FDG PET/CT helps differentiate autoimmune pancreatitis from pancreatic cancer. ( 29061130 )
2017
48
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
49
Diagnosis and Management of Autoimmune Pancreatitis. ( 28842855 )
2017
50
Endoscopic Ultrasonography-Guided Fine Needle Aspiration Can Be Used to Rule Out Malignancy in Autoimmune Pancreatitis Patients. ( 28670760 )
2017

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)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.65 CD4 CTLA4 HLA-DQB1 HLA-DRB1
2
Show member pathways
12.54 CD4 CTLA4 HLA-DQB1 HLA-DRB1 TNF
3
Show member pathways
12.51 HLA-DQB1 HLA-DRB1 PRSS1 TNF
4
Show member pathways
12.18 CD4 CTLA4 TNF
5
Show member pathways
12.18 CD4 HLA-DQB1 HLA-DRB1 TNF
6 12.17 HLA-DQB1 HLA-DRB1 TNF
7
Show member pathways
12.11 CD4 HLA-DQB1 HLA-DRB1
8
Show member pathways
12.11 CD4 CTLA4 HLA-DQB1 HLA-DRB1
9
Show member pathways
12.01 HLA-DQB1 HLA-DRB1 TNF
10 11.98 HLA-DQB1 HLA-DRB1 TNF
11
Show member pathways
11.92 CD4 HLA-DQB1 HLA-DRB1
12
Show member pathways
11.91 CD4 CTLA4 HLA-DQB1 HLA-DRB1
13 11.72 CD4 CTLA4 HLA-DQB1 HLA-DRB1
14
Show member pathways
11.71 CD4 HLA-DQB1 HLA-DRB1
15 11.69 CD4 HLA-DQB1 HLA-DRB1
16 11.64 CA2 CFTR PRSS1
17 11.44 CD4 HLA-DQB1 HLA-DRB1 TNF
18 11.36 HLA-DQB1 HLA-DRB1
19 11.26 CA2 TNF
20 11.21 CTLA4 HLA-DQB1 HLA-DRB1 TNF
21 11.02 CD4 HLA-DRB1
22 10.97 CD4 HLA-DRB1 TNF
23 10.78 CD4 CTLA4 HLA-DQB1 HLA-DRB1

GO Terms for Autoimmune Pancreatitis

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.95 CA2 CD4 CFTR CTLA4 HLA-DQB1 HLA-DRB1
2 lysosomal membrane GO:0005765 9.61 CFTR HLA-DQB1 HLA-DRB1
3 endosome membrane GO:0010008 9.5 CFTR HLA-DQB1 HLA-DRB1
4 ER to Golgi transport vesicle membrane GO:0012507 9.49 HLA-DQB1 HLA-DRB1
5 clathrin-coated vesicle membrane GO:0030665 9.48 CD4 CFTR
6 transport vesicle membrane GO:0030658 9.46 HLA-DQB1 HLA-DRB1
7 cell surface GO:0009986 9.46 CD4 CFTR HLA-DRB1 TNF
8 clathrin-coated endocytic vesicle membrane GO:0030669 9.37 HLA-DQB1 HLA-DRB1
9 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.26 HLA-DQB1 HLA-DRB1
10 MHC class II protein complex GO:0042613 8.96 HLA-DQB1 HLA-DRB1
11 external side of plasma membrane GO:0009897 8.92 CD4 CTLA4 HLA-DRB1 TNF

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 T cell receptor signaling pathway GO:0050852 9.54 CD4 HLA-DQB1 HLA-DRB1
2 positive regulation of protein kinase activity GO:0045860 9.51 CD4 TNF
3 bicarbonate transport GO:0015701 9.49 CA2 CFTR
4 antigen processing and presentation GO:0019882 9.48 HLA-DQB1 HLA-DRB1
5 negative regulation of T cell proliferation GO:0042130 9.46 CTLA4 HLA-DRB1
6 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.43 CFTR HLA-DRB1
7 positive regulation of osteoclast differentiation GO:0045672 9.4 CA2 TNF
8 immune response GO:0006955 9.35 CD4 CTLA4 HLA-DQB1 HLA-DRB1 TNF
9 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.32 HLA-DQB1 HLA-DRB1
10 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.26 HLA-DQB1 HLA-DRB1
11 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 9.16 HLA-DQB1 HLA-DRB1
12 T cell costimulation GO:0031295 8.92 CD4 CTLA4 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
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
Content
Loading form....