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AIP
MCID: ATM024
MIFTS: 48

Autoimmune Pancreatitis (AIP)

Categories: Bone diseases, Endocrine diseases, Gastrointestinal diseases, Genetic diseases, Immune diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Autoimmune Pancreatitis

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MalaCards integrated aliases for Autoimmune Pancreatitis:

Name: Autoimmune Pancreatitis 12 53 59 15 73
Lymphoplasmocytic Sclerosing Pancreatitis 53
Aip 59

Characteristics:

Orphanet epidemiological data:

59
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases
Anatomical: Gastrointestinal diseases Bone diseases Immune diseases Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare gastroenterological diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:0040091
Orphanet 59 ORPHA103919
UMLS via Orphanet 74 C2609129
ICD10 via Orphanet 34 K86.1
UMLS 73 C2609129
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Summaries for Autoimmune Pancreatitis

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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 cholangitis and polyradiculoneuropathy. An important gene associated with Autoimmune Pancreatitis is CA2 (Carbonic Anhydrase 2), and among its related pathways/superpathways are NF-kappaB Signaling and Allograft rejection. The drugs rituximab and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include pancreas, salivary gland and kidney.

Disease Ontology : 12 An autoimmune disease of endocrine system that is located in the pancreas.

Wikipedia : 76 Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be... more...

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Related Diseases for Autoimmune Pancreatitis

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Diseases in the Pancreatitis family:

Pancreatitis, Hereditary Autoimmune Pancreatitis
Acute Pancreatitis Prss1-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 207)
# Related Disease Score Top Affiliating Genes
1 cholangitis 31.0 CA2 CFTR HLA-DRB1
2 polyradiculoneuropathy 29.7 HLA-DRB1 IL10
3 colitis 29.6 CTLA4 FOXP3 IL10
4 inflammatory bowel disease 29.6 CTLA4 FOXP3 HLA-DRB1 IL10
5 celiac disease 1 29.5 CTLA4 FOXP3 IL10
6 malaria 29.3 CTLA4 FOXP3 HLA-DRB1 IL10
7 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 29.0 CTLA4 FOXP3 IL10 TNFSF13B
8 autoimmune disease 28.9 CTLA4 ENO1 FCRL3 FOXP3 HLA-DRB1 IL10
9 rheumatoid arthritis 28.6 CTLA4 FCRL3 FOXP3 HLA-DRB1 IL10 TNFSF13B
10 systemic lupus erythematosus 28.6 CTLA4 FCRL3 FOXP3 HLA-DRB1 IL10 TNFSF13B
11 aip-related familial isolated pituitary adenomas 12.3
12 autoimmune pancreatitis type 1 12.2
13 autoimmune pancreatitis type 2 12.2
14 porphyria, acute intermittent 12.2
15 acromegaly 12.0
16 pituitary adenoma 1, multiple types 12.0
17 acute interstitial pneumonia 12.0
18 pituitary adenoma, prolactin-secreting 11.8
19 pituitary adenoma 11.7
20 familial isolated pituitary adenoma 11.6
21 acroleukopathy, symmetric 11.5
22 gigantism 11.5
23 pituitary apoplexy 11.5
24 pituitary tumors 11.5
25 growth hormone secreting pituitary adenoma 11.4
26 igg4-related disease 11.4
27 mikulicz disease 11.4
28 hepatitis b 11.3
29 chester porphyria 11.2
30 pancreatitis 11.2
31 carney complex variant 11.1
32 multiple endocrine neoplasia, type iv 11.1
33 cell type benign neoplasm 11.1
34 hyperpituitarism 11.1
35 pituitary gland disease 11.1
36 hormone producing pituitary cancer 11.1
37 cancer-associated retinopathy 11.1
38 acth-secreting pituitary adenoma 11.0
39 endocrine organ benign neoplasm 10.9
40 silent pituitary adenoma 10.9
41 null pituitary adenoma 10.9
42 pancreatic cancer 10.6
43 sclerosing cholangitis 10.5
44 porphyria 10.4
45 adenocarcinoma 10.3
46 recurrent acute pancreatitis 10.2 CFTR PRSS1
47 type 1 diabetes mellitus 12 10.2 CTLA4 HLA-DRB1
48 autoimmune addison disease 10.2 CTLA4 HLA-DRB1
49 retroperitoneal fibrosis 10.2
50 anca-associated vasculitis 10.2 CTLA4 HLA-DRB1

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



Diseases related to Autoimmune Pancreatitis
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Symptoms & Phenotypes for Autoimmune Pancreatitis

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Drugs & Therapeutics for Autoimmune Pancreatitis

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

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
2 Antibodies Phase 1, Phase 2,Not Applicable
3 Immunoglobulin G Phase 1, Phase 2,Not Applicable
4 Immunoglobulins Phase 1, Phase 2,Not Applicable
5 Antirheumatic Agents Phase 1, Phase 2
6 Immunologic Factors Phase 1, Phase 2
7
Lenalidomide Approved Phase 1 191732-72-6 216326
8 Immunosuppressive Agents Phase 1
9 Anti-Infective Agents Phase 1
10 Angiogenesis Modulating Agents Phase 1
11 Angiogenesis Inhibitors Phase 1
12 Anti-Bacterial Agents Phase 1
13
Pancrelipase Approved, Investigational 53608-75-6
14
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
15 Liver Extracts
16 pancreatin

Interventional clinical trials:

(show all 13)
# 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 Diagnostic Yield of FNA Needle and FNB Needle for Autoimmune Pancreatitis Recruiting NCT03753815 Not Applicable
12 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Active, not recruiting NCT02797665 Not Applicable corticosteroids
13 Efficacy of Secretin MRCP in the Diagnosis and Follow up of Auto Immune Pancreatitis Withdrawn NCT01845467

Search NIH Clinical Center for Autoimmune Pancreatitis

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Genetic Tests for Autoimmune Pancreatitis

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Anatomical Context for Autoimmune Pancreatitis

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MalaCards organs/tissues related to Autoimmune Pancreatitis:

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

The Foundational Model of Anatomy Ontology organs/tissues related to Autoimmune Pancreatitis:

19
The Pancreas
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Publications for Autoimmune Pancreatitis

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Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 1025)
# Title Authors Year
1
Risk Factors for Pancreatic Stone Formation in Type 1 Autoimmune Pancreatitis: A Long-term Japanese Multicenter Analysis of 624 Patients. ( 30540679 )
2019
2
Clinical course of type 1 autoimmune pancreatitis patients without steroid treatment: a Japanese multicenter study of 97 patients. ( 29430861 )
2018
3
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
4
Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis. ( 29761833 )
2018
5
Prevalence of inflammatory bowel disease in alcoholic, non-alcoholic and autoimmune pancreatitis. ( 29734447 )
2018
6
Diagnosis and treatment of autoimmune pancreatitis. ( 29901516 )
2018
7
Comparison of clinical characteristics of radiological forms of autoimmune pancreatitis. ( 29843984 )
2018
8
Focal Autoimmune Pancreatitis Mimicking Pancreatic Cancer on FDG PET/CT Imaging. ( 29189377 )
2018
9
Response to the Letter by Poddighe et al. regarding our manuscript &amp;quot;Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis&amp;quot;. ( 29693194 )
2018
10
Comparison of diagnostic performance between CT and MRI in differentiating non-diffuse-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma. ( 29948075 )
2018
11
Monitoring and predicting disease activity in autoimmune pancreatitis with the M-ANNHEIM-AiP-Activity-Score. ( 29158146 )
2018
12
Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus from INSPPIRE. ( 29746340 )
2018
13
Basophils activated via TLR signaling may contribute to pathophysiology of type I autoimmune pancreatitis&amp;quot;. ( 29663078 )
2018
14
Autoimmune pancreatitis with concomitant autoimmune haemolytic anaemia. ( 29574435 )
2018
15
A case report of autoimmune pancreatitis associated with a pancreatic pseudocyst. ( 29742687 )
2018
16
Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review. ( 29332510 )
2018
17
Contrast-Enhanced Endoscopic Ultrasound for Differentially Diagnosing Autoimmune Pancreatitis and Pancreatic Cancer. ( 29699060 )
2018
18
Updates in the field of autoimmune pancreatitis: a clinical guide. ( 29923430 )
2018
19
Predominant Improvement of Alpha Cell Function after Steroid Therapy in a Patient with Autoimmune Pancreatitis: Case Report. ( 29725970 )
2018
20
Autoimmune Pancreatitis. ( 29910463 )
2018
21
Metastatic rhabdomyosarcoma mimicking autoimmune pancreatitis diagnosed by EUS-guided fine-needle biopsy. ( 29627494 )
2018
22
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
23
A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma. ( 29681791 )
2018
24
Endoscopic ultrasound guided 22 gauge core needle biopsy for the diagnosis of Autoimmune pancreatitis. ( 29338920 )
2018
25
The Role of Endoscopic Ultrasound-guided Drainage for Autoimmune Pancreatitis-associated Pancreatic Cysts: A Report of Five Cases and a Literature Review. ( 29434161 )
2018
26
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
27
Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent. ( 29610171 )
2018
28
Serum Immunoglobulin G4 in Discriminating Autoimmune Pancreatitis From Pancreatic Cancer: A Diagnostic Meta-analysis. ( 29351121 )
2018
29
Qualitative and quantitative evaluation for morphological changes of the splenic artery in autoimmune pancreatitis: novel imaging findings for differentiation from pancreatic adenocarcinoma. ( 29948059 )
2018
30
The clinical utility of immunoglobulin G4 in the evaluation of autoimmune pancreatitis and pancreatic adenocarcinoma. ( 29033025 )
2018
31
Differences in Gut Microbiota Profiles between Autoimmune Pancreatitis and Chronic Pancreatitis. ( 29434076 )
2018
32
Serum apolipoprotein A2 isoforms in autoimmune pancreatitis. ( 29481802 )
2018
33
The use of magnetic resonance elastography in differentiating autoimmune pancreatitis from pancreatic ductal adenocarcinoma: A preliminary study. ( 30396645 )
2018
34
Clinical and pathophysiological aspects of type 1 autoimmune pancreatitis. ( 29460239 )
2018
35
Postoperative hemorrhage caused by portal hypertension associated with autoimmune pancreatitis: A case report. ( 29465601 )
2018
36
Hepatobiliary and Pancreatic: A rare form of autoimmune pancreatitis mimicking mixed-type intraductal papillary mucinous neoplasm. ( 29469233 )
2018
37
Clinical Utility of FDG PET/CT in Patients with Autoimmune Pancreatitis: a Case-Control Study. ( 29483544 )
2018
38
Correction to: Basophils activated via TLR signaling may contribute to pathophysiology of type 1 autoimmune pancreatitis. ( 29484507 )
2018
39
IgG4-related autoimmune pancreatitis complicated by splenic artery pseudoaneurysm. ( 29507039 )
2018
40
Autoimmune Pancreatitis Mouse Model. ( 29512140 )
2018
41
Relative Rise of Serum IgG4 Levels After Steroid Therapy for Autoimmune Pancreatitis Predicts the Likelihood of Relapse. ( 29517627 )
2018
42
Type 2 Autoimmune Pancreatitis with Crohn's Disease. ( 29526939 )
2018
43
Focal immune-related pancreatitis occurring after treatment with programmed cell death 1 inhibitors: a distinct form of autoimmune pancreatitis? ( 29530539 )
2018
44
Promoter hypomethylation of SKI in autoimmune pancreatitis. ( 29534839 )
2018
45
Pancreatic head mass and jaundice as revealing signs of autoimmune pancreatitis type 2 in a 15-year-old girl. ( 29550256 )
2018
46
Intraductal Tubulopapillary Epithelial Proliferation Associated with Type 1 Autoimmune Pancreatitis. ( 29557419 )
2018
47
Hypothyroidism in patients with autoimmune pancreatitis. ( 29736303 )
2018
48
Clinical profile and treatment outcomes in autoimmune pancreatitis: a report from North India. ( 29991897 )
2018
49
Current Concept of Autoimmune Pancreatitis and IgG4-related Disease. ( 30002467 )
2018
50
Type-1 Seronegative Autoimmune Pancreatitis: A Rare Case of Autoimmune Pancreatitis with Sclerosing Cholangitis and Hashimoto's Disease. ( 30027016 )
2018
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Variations for Autoimmune Pancreatitis

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Expression for Autoimmune Pancreatitis

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Search GEO for disease gene expression data for Autoimmune Pancreatitis.
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Pathways for Autoimmune Pancreatitis

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

# Super pathways Score Top Affiliating Genes
1
NF-kappaB Signaling 4
12.07 CTLA4 FOXP3 IL10 TNFSF13B
2
Allograft rejection 1 37
Show member pathways
 11.76 CTLA4 FOXP3 HLA-DRB1 IL10 TNFSF13B
3
Pancreatic secretion 37
11.31 CA2 CFTR PRSS1
4
Rheumatoid arthritis 37
11.09 CTLA4 HLA-DRB1 TNFSF13B
5
Cytokines and Inflammatory Response 1
10.78 HLA-DRB1 IL10
6
G-protein signaling_RhoB regulation pathway 22
10.77 IL10 TNFSF13B UBR2
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GO Terms for Autoimmune Pancreatitis

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.71 CTLA4 HLA-DRB1 IL10 TNFSF13B
2 positive regulation of B cell proliferation GO:0030890 9.48 FCRL3 TNFSF13B
3 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.46 CFTR HLA-DRB1
4 negative regulation of interleukin-6 production GO:0032715 9.43 FOXP3 IL10
5 B cell homeostasis GO:0001782 9.37 FOXP3 TNFSF13B
6 negative regulation of B cell proliferation GO:0030889 9.32 CTLA4 IL10
7 regulation of regulatory T cell differentiation GO:0045589 9.26 CTLA4 FOXP3
8 negative regulation of immune response GO:0050777 9.16 CTLA4 FOXP3
9 negative regulation of interferon-gamma production GO:0032689 9.13 FOXP3 HLA-DRB1 IL10
10 negative regulation of T cell proliferation GO:0042130 8.92 CTLA4 FOXP3 HLA-DRB1 IL10
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Sources for Autoimmune Pancreatitis

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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