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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 54 60 15 74
Lymphoplasmocytic Sclerosing Pancreatitis 54
Aip 60

Characteristics:

Orphanet epidemiological data:

60
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: 35
Orphanet: 60  
Rare gastroenterological diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:0040091
ICD10 via Orphanet 35 K86.1
UMLS via Orphanet 75 C2609129
Orphanet 60 ORPHA103919
UMLS 74 C2609129
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Summaries for Autoimmune Pancreatitis

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NIH Rare Diseases : 54 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 colitis. 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 Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include the pancreas, pancreas and salivary gland.

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

Wikipedia : 77 Autoimmune pancreatitis 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 216)
# Related Disease Score Top Affiliating Genes
1 cholangitis 31.2 CA2 CFTR HLA-DRB1
2 colitis 29.7 CTLA4 FOXP3 IL10
3 polyradiculoneuropathy 29.7 HLA-DRB1 IL10
4 malaria 29.0 CTLA4 FOXP3 HLA-DRB1 IL10
5 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 28.8 CTLA4 FOXP3 IL10 TNFSF13B
6 systemic lupus erythematosus 28.4 CTLA4 FCRL3 FOXP3 HLA-DRB1 IL10 TNFSF13B
7 autoimmune disease 28.3 CTLA4 ENO1 FCRL3 FOXP3 HLA-DRB1 IL10
8 rheumatoid arthritis 28.2 CTLA4 FCRL3 FOXP3 HLA-DRB1 IL10 TNFSF13B
9 autoimmune pancreatitis type 1 12.3
10 aip-related familial isolated pituitary adenomas 12.3
11 autoimmune pancreatitis type 2 12.3
12 porphyria, acute intermittent 12.2
13 pituitary adenoma 1, multiple types 12.1
14 acute interstitial pneumonia 12.0
15 pituitary adenoma, prolactin-secreting 11.8
16 acromegaly 11.7
17 pituitary adenoma 11.7
18 familial isolated pituitary adenoma 11.7
19 igg4-related disease 11.6
20 gigantism 11.5
21 pituitary tumors 11.5
22 pituitary apoplexy 11.5
23 acroleukopathy, symmetric 11.5
24 mikulicz disease 11.4
25 hepatitis b 11.3
26 chester porphyria 11.2
27 pancreatitis 11.2
28 carney complex variant 11.1
29 multiple endocrine neoplasia, type iv 11.1
30 cell type benign neoplasm 11.1
31 endocrine organ benign neoplasm 11.1
32 hyperpituitarism 11.1
33 pituitary gland disease 11.1
34 functioning pituitary adenoma 11.1
35 hormone producing pituitary cancer 11.1
36 cancer-associated retinopathy 11.1
37 acth-secreting pituitary adenoma 11.1
38 pituitary adenoma 4, acth-secreting 11.0
39 silent pituitary adenoma 11.0
40 null pituitary adenoma 11.0
41 pancreatic cancer 10.6
42 sclerosing cholangitis 10.5
43 porphyria 10.4
44 recurrent acute pancreatitis 10.4 CFTR PRSS1
45 adenocarcinoma 10.3
46 type 1 diabetes mellitus 12 10.3 CTLA4 HLA-DRB1
47 autoimmune addison disease 10.3 CTLA4 HLA-DRB1
48 anca-associated vasculitis 10.3 CTLA4 HLA-DRB1
49 neuroretinitis 10.3 CA2 CTLA4
50 autoimmune retinopathy 10.3 CA2 ENO1

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 27)
# 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,Not Applicable
3 Antineoplastic Agents, Immunological Phase 1, Phase 2
4 Immunoglobulin G Phase 1, Phase 2,Not Applicable
5 Antibodies Phase 1, Phase 2,Not Applicable
6 Immunologic Factors Phase 1, Phase 2
7 Antirheumatic Agents Phase 1, Phase 2
8
Lenalidomide Approved Phase 1 191732-72-6 216326
9 Angiogenesis Inhibitors Phase 1
10 Angiogenesis Modulating Agents Phase 1
11
Pancrelipase Approved, Investigational 53608-75-6
12
Prednisone Approved, Vet_approved Not Applicable 53-03-2 5865
13
Ergocalciferol Approved, Nutraceutical 50-14-6 5280793
14
Vitamin D Approved, Nutraceutical, Vet_approved 1406-16-2
15
Calcium Approved, Nutraceutical 7440-70-2 271
16 Liver Extracts
17 pancreatin
18 Vitamins
19 Trace Elements
20 Hormones
21 Nutrients
22 Bone Density Conservation Agents
23 Calciferol
24 Vitamin D2
25 Ergocalciferols
26 Micronutrients
27 Calcium, Dietary

Interventional clinical trials:

(show all 14)
# 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 Active, not 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 Assessment of the Different Etiological and Susceptibility Markers in Patients With Pancreatitis: Investigating IG4, Cytomegalovirus, Coxsackie- Virus, Genetic Polymorphism of Vitamin D Receptor Gene Not yet recruiting NCT03830073
14 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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The Foundational Model of Anatomy Ontology organs/tissues related to Autoimmune Pancreatitis:

20
The Pancreas

MalaCards organs/tissues related to Autoimmune Pancreatitis:

42
Pancreas, Salivary Gland, Kidney, T Cells, Bone, Liver, Colon
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Publications for Autoimmune Pancreatitis

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Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 1171)
# Title Authors Year
1
High accuracy differentiating autoimmune pancreatitis from pancreatic ductal adenocarcinoma by immunoglobulin G glycosylation. ( 30622446 )
2019
2
Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up. ( 30455055 )
2019
3
Risk Factors for Pancreatic Stone Formation in Type 1 Autoimmune Pancreatitis: A Long-term Japanese Multicenter Analysis of 624 Patients. ( 30540679 )
2019
4
Autoimmune pancreatitis in children: The impact of immune system in a challenging disease. ( 30572132 )
2019
5
IgG4-related hypophysitis in patients with autoimmune pancreatitis. ( 30607745 )
2019
6
A Diagnostically Challenging Case of Autoimmune Pancreatitis Due to Contamination of the Pathological Specimen with Early Gastric Cancer. ( 30626846 )
2019
7
Pancreatic Cystic Lesions in Cases of Autoimmune Pancreatitis. ( 30640232 )
2019
8
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. ( 30701685 )
2019
9
Native T1 mapping of autoimmune pancreatitis as a quantitative outcome surrogate. ( 30707275 )
2019
10
Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis. ( 30713326 )
2019
11
Autoimmune Pancreatitis Exhibiting Pancreatic Duct Wall Thickening. ( 30713329 )
2019
12
A case of high-grade pancreatic intraepithelial neoplasia concomitant with type 1 autoimmune pancreatitis: the process underlying both conditions. ( 30719801 )
2019
13
Delayed pancreatic ductal leakage after EUS-FNA for autoimmune pancreatitis. ( 30719995 )
2019
14
Unclassified autoimmune pancreatitis mimicking pancreatic cancer. ( 30740201 )
2019
15
A Nondilated Main Pancreatic Duct Predicts Type 2 Autoimmune Pancreatitis: Comparative Study of Resected Pancreatic Head Masses. ( 30759428 )
2019
16
Case of IgG4-associated sclerosing cholangitis with normal serum IgG4 concentration, diagnosed by anti-laminin 511-E8 antibody: a novel autoantibody in patients with autoimmune pancreatitis. ( 30760486 )
2019
17
Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis. ( 30781677 )
2019
18
Differentiation of Autoimmune Pancreatitis from Pancreatic Cancer Remains Challenging. ( 30815742 )
2019
19
Clinical characteristics of autoimmune pancreatitis with IgG4 related kidney disease. ( 30826634 )
2019
20
Nivolumab-related pancreatitis with autoimmune pancreatitis-like imaging features. ( 30828866 )
2019
21
Combination Therapy with Empagliflozin and Insulin Results in Successful Glycemic Control: A Case Report of Uncontrolled Diabetes Caused by Autoimmune Pancreatitis and Subsequent Steroid Treatment. ( 30895163 )
2019
22
Impact of sclerosing dacryoadenitis/sialadenitis on relapse during steroid therapy in patients with type 1 autoimmune pancreatitis. ( 30915865 )
2019
23
Clinical course of type 1 autoimmune pancreatitis patients without steroid treatment: a Japanese multicenter study of 97 patients. ( 29430861 )
2018
24
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
25
Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis. ( 29761833 )
2018
26
Prevalence of inflammatory bowel disease in alcoholic, non-alcoholic and autoimmune pancreatitis. ( 29734447 )
2018
27
Diagnosis and treatment of autoimmune pancreatitis. ( 29901516 )
2018
28
Comparison of clinical characteristics of radiological forms of autoimmune pancreatitis. ( 29843984 )
2018
29
Focal Autoimmune Pancreatitis Mimicking Pancreatic Cancer on FDG PET/CT Imaging. ( 29189377 )
2018
30
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
31
Comparison of diagnostic performance between CT and MRI in differentiating non-diffuse-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma. ( 29948075 )
2018
32
Monitoring and predicting disease activity in autoimmune pancreatitis with the M-ANNHEIM-AiP-Activity-Score. ( 29158146 )
2018
33
Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus from INSPPIRE. ( 29746340 )
2018
34
Basophils activated via TLR signaling may contribute to pathophysiology of type I autoimmune pancreatitis&amp;quot;. ( 29663078 )
2018
35
Autoimmune pancreatitis with concomitant autoimmune haemolytic anaemia. ( 29574435 )
2018
36
A case report of autoimmune pancreatitis associated with a pancreatic pseudocyst. ( 29742687 )
2018
37
Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review. ( 29332510 )
2018
38
Contrast-Enhanced Endoscopic Ultrasound for Differentially Diagnosing Autoimmune Pancreatitis and Pancreatic Cancer. ( 29699060 )
2018
39
Updates in the field of autoimmune pancreatitis: a clinical guide. ( 29923430 )
2018
40
Predominant Improvement of Alpha Cell Function after Steroid Therapy in a Patient with Autoimmune Pancreatitis: Case Report. ( 29725970 )
2018
41
Autoimmune Pancreatitis. ( 29910463 )
2018
42
Metastatic rhabdomyosarcoma mimicking autoimmune pancreatitis diagnosed by EUS-guided fine-needle biopsy. ( 29627494 )
2018
43
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
44
A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma. ( 29681791 )
2018
45
Endoscopic ultrasound guided 22 gauge core needle biopsy for the diagnosis of Autoimmune pancreatitis. ( 29338920 )
2018
46
The Role of Endoscopic Ultrasound-guided Drainage for Autoimmune Pancreatitis-associated Pancreatic Cysts: A Report of Five Cases and a Literature Review. ( 29434161 )
2018
47
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
48
Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent. ( 29610171 )
2018
49
Serum Immunoglobulin G4 in Discriminating Autoimmune Pancreatitis From Pancreatic Cancer: A Diagnostic Meta-analysis. ( 29351121 )
2018
50
Qualitative and quantitative evaluation for morphological changes of the splenic artery in autoimmune pancreatitis: novel imaging findings for differentiation from pancreatic adenocarcinoma. ( 29948059 )
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 38
Show member pathways
 11.76 CTLA4 FOXP3 HLA-DRB1 IL10 TNFSF13B
3
Pancreatic secretion 38
11.32 CA2 CFTR PRSS1
4
Rheumatoid arthritis 38
11.09 CTLA4 HLA-DRB1 TNFSF13B
5
Cytokines and Inflammatory Response 1
10.78 HLA-DRB1 IL10
6
G-protein signaling_RhoB regulation pathway 23
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 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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