MCID: ATM024
MIFTS: 50

Autoimmune Pancreatitis

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

Aliases & Classifications for Autoimmune Pancreatitis

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:



External Ids:

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

Summaries for Autoimmune Pancreatitis

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 pancreas adenocarcinoma 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 Antineoplastic Agents, Immunological have been mentioned in the context of this disorder. Affiliated tissues include the pancreas, pancreas and pituitary, and related phenotypes are digestive/alimentary and craniofacial

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...

Related Diseases for Autoimmune Pancreatitis

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 219)
# Related Disease Score Top Affiliating Genes
1 pancreas adenocarcinoma 30.1 FOXP3 HRAS PRSS1
2 colitis 29.9 CTLA4 FOXP3 IL10
3 autoimmune lymphoproliferative syndrome 29.3 CTLA4 HRAS IL10
4 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 29.0 CTLA4 FOXP3 IL10 TNFSF13B
5 autoimmune disease 28.9 CTLA4 ENO1 FCRL3 FOXP3 IL10 TNFSF13B
6 systemic lupus erythematosus 28.9 CTLA4 FCRL3 FOXP3 IL10 TNFSF13B
7 rheumatoid arthritis 28.7 CTLA4 FCRL3 FOXP3 IL10 TNFSF13B
8 autoimmune pancreatitis type 1 12.3
9 porphyria, acute intermittent 12.3
10 autoimmune pancreatitis type 2 12.3
11 aip-related familial isolated pituitary adenomas 12.2
12 acromegaly 12.1
13 pituitary adenoma 1, multiple types 12.1
14 acute interstitial pneumonia 12.0
15 pituitary adenoma, prolactin-secreting 12.0
16 pituitary adenoma 11.7
17 familial isolated pituitary adenoma 11.7
18 igg4-related disease 11.6
19 gigantism 11.6
20 pituitary tumors 11.5
21 pituitary apoplexy 11.5
22 acroleukopathy, symmetric 11.5
23 mikulicz disease 11.4
24 hepatitis b 11.3
25 chester porphyria 11.2
26 pancreatitis 11.2
27 carney complex variant 11.1
28 multiple endocrine neoplasia, type iv 11.1
29 cell type benign neoplasm 11.1
30 endocrine organ benign neoplasm 11.1
31 pituitary infarct 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 growth hormone secreting pituitary adenoma 11.1
37 cancer-associated retinopathy 11.1
38 acth-secreting pituitary adenoma 11.1
39 pituitary adenoma 4, acth-secreting 11.0
40 silent pituitary adenoma 11.0
41 null pituitary adenoma 11.0
42 pancreatic cancer 10.6
43 cholangitis 10.6
44 sclerosing cholangitis 10.5
45 porphyria 10.4
46 adenocarcinoma 10.4
47 recurrent acute pancreatitis 10.3 CFTR PRSS1
48 autoimmune retinopathy 10.3 CA2 ENO1
49 neuroretinitis 10.3 CA2 CTLA4
50 johanson-blizzard syndrome 10.3 CFTR UBR2

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.73 CA2 CFTR CTLA4 FOXP3 HRAS IL10
2 craniofacial MP:0005382 9.72 CFTR FOXP3 HRAS IL10 SPTAN1
3 endocrine/exocrine gland MP:0005379 9.7 CA2 CFTR CTLA4 FOXP3 HRAS IL10
4 normal MP:0002873 9.5 CFTR CTLA4 FOXP3 HRAS IL10 KCNA3
5 respiratory system MP:0005388 9.1 CA2 CFTR CTLA4 FOXP3 HRAS IL10

Drugs & Therapeutics for Autoimmune Pancreatitis

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 Antineoplastic Agents, Immunological Phase 1, Phase 2
3 Antirheumatic Agents Phase 1, Phase 2
4 Immunologic Factors Phase 1, Phase 2
5 Immunoglobulin G Phase 1, Phase 2,Not Applicable
6 Immunoglobulins Phase 1, Phase 2,Not Applicable
7 Antibodies Phase 1, Phase 2,Not Applicable
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
Vitamin D Approved, Nutraceutical, Vet_approved 1406-16-2
14
Calcium Approved, Nutraceutical 7440-70-2 271
15
Ergocalciferol Approved, Nutraceutical 50-14-6 5280793
16 Liver Extracts
17 pancreatin
18 Calciferol
19 Vitamins
20 Vitamin D2
21 Bone Density Conservation Agents
22 Hormones
23 Calcium, Dietary
24 Micronutrients
25 Trace Elements
26 Nutrients
27 Ergocalciferols

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

Genetic Tests for Autoimmune Pancreatitis

Anatomical Context for Autoimmune Pancreatitis

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

20
The Pancreas

MalaCards organs/tissues related to Autoimmune Pancreatitis:

42
Pancreas, Pituitary, Kidney, Salivary Gland, Lymph Node, T Cells, Bone

Publications for Autoimmune Pancreatitis

Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 1182)
# Title Authors Year
1
Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience. ( 30970429 )
2019
2
Differentiating focal autoimmune pancreatitis and pancreatic ductal adenocarcinoma: contrast-enhanced MRI with special emphasis on the arterial phase. ( 31028441 )
2019
3
High accuracy differentiating autoimmune pancreatitis from pancreatic ductal adenocarcinoma by immunoglobulin G glycosylation. ( 30622446 )
2019
4
Risk Factors for Rate of Relapse and Effects of Steroid Maintenance Therapy in Patients With Autoimmune Pancreatitis: Systematic Review and Meta-analysis. ( 30312787 )
2019
5
Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis. ( 30424589 )
2019
6
Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up. ( 30455055 )
2019
7
IgG4 related autoimmune pancreatitis and sclerosing cholangitis. ( 30457561 )
2019
8
Risk Factors for Pancreatic Stone Formation in Type 1 Autoimmune Pancreatitis: A Long-term Japanese Multicenter Analysis of 624 Patients. ( 30540679 )
2019
9
IgG4-related hypophysitis in patients with autoimmune pancreatitis. ( 30607745 )
2019
10
A Diagnostically Challenging Case of Autoimmune Pancreatitis Due to Contamination of the Pathological Specimen with Early Gastric Cancer. ( 30626846 )
2019
11
Pancreatic Cystic Lesions in Cases of Autoimmune Pancreatitis. ( 30640232 )
2019
12
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. ( 30701685 )
2019
13
Native T1 mapping of autoimmune pancreatitis as a quantitative outcome surrogate. ( 30707275 )
2019
14
Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis. ( 30713326 )
2019
15
Autoimmune Pancreatitis Exhibiting Pancreatic Duct Wall Thickening. ( 30713329 )
2019
16
A case of high-grade pancreatic intraepithelial neoplasia concomitant with type 1 autoimmune pancreatitis: the process underlying both conditions. ( 30719801 )
2019
17
Delayed pancreatic ductal leakage after EUS-FNA for autoimmune pancreatitis. ( 30719995 )
2019
18
Unclassified autoimmune pancreatitis mimicking pancreatic cancer. ( 30740201 )
2019
19
A Nondilated Main Pancreatic Duct Predicts Type 2 Autoimmune Pancreatitis: Comparative Study of Resected Pancreatic Head Masses. ( 30759428 )
2019
20
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
21
Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis. ( 30781677 )
2019
22
Differentiation of Autoimmune Pancreatitis from Pancreatic Cancer Remains Challenging. ( 30815742 )
2019
23
Clinical characteristics of autoimmune pancreatitis with IgG4 related kidney disease. ( 30826634 )
2019
24
Nivolumab-related pancreatitis with autoimmune pancreatitis-like imaging features. ( 30828866 )
2019
25
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
26
Impact of sclerosing dacryoadenitis/sialadenitis on relapse during steroid therapy in patients with type 1 autoimmune pancreatitis. ( 30915865 )
2019
27
Feasibility and usefulness of endoscopic ultrasonography-guided shear-wave measurement for assessment of autoimmune pancreatitis activity: a prospective exploratory study. ( 30993580 )
2019
28
Methotrexate as Induction of Remission Therapy for Type 1 Autoimmune Pancreatitis. ( 30998520 )
2019
29
Management of Autoimmune Pancreatitis. ( 31042584 )
2019
30
A Curious Case of Autoimmune Pancreatitis with IgG4-related Sclerosing Cholangitis. ( 31058036 )
2019
31
Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer. ( 31079114 )
2019
32
The Challenge of Autoimmune Pancreatitis: A Portrayal From the Pediatric Perspective. ( 31090661 )
2019
33
Type 1 autoimmune pancreatitis: a capsule-like rim surrounding the enlarged pancreas. ( 30107608 )
2019
34
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
35
The use of magnetic resonance elastography in differentiating autoimmune pancreatitis from pancreatic ductal adenocarcinoma: A preliminary study. ( 30396645 )
2018
36
Qualitative and quantitative evaluation for morphological changes of the splenic artery in autoimmune pancreatitis: novel imaging findings for differentiation from pancreatic adenocarcinoma. ( 29948059 )
2018
37
Comparison of diagnostic performance between CT and MRI in differentiating non-diffuse-type autoimmune pancreatitis from pancreatic ductal adenocarcinoma. ( 29948075 )
2018
38
Gastric varices and splenic vein obstruction during steroid treatment for autoimmune pancreatitis: A case report and literature review. ( 30142813 )
2018
39
There Is No Association between Coeliac Disease and Autoimmune Pancreatitis. ( 30149525 )
2018
40
Autoimmune pancreatitis associated with retroperitoneal fibrosis mimicking cholangiocarcinoma. ( 30159151 )
2018
41
Unique properties of IgG4 antibody and its clinical application in autoimmune pancreatitis. ( 30175675 )
2018
42
Disappearance of multiple pancreatic cysts after prednisolone treatment in a patient with autoimmune pancreatitis. ( 30214790 )
2018
43
Diagnosis, treatment and long-term outcome of autoimmune pancreatitis in Sweden. ( 30236651 )
2018
44
Management of Autoimmune Pancreatitis. ( 30241640 )
2018
45
Predictive Factors for Autoimmune Pancreatitis Relapse After 3 Years of Maintenance Therapy. ( 30286015 )
2018
46
MR Imaging of Autoimmune Pancreatitis. ( 30376982 )
2018
47
Mechanistic Insights into Autoimmune Pancreatitis and IgG4-Related Disease. ( 30401468 )
2018
48
Serum and histological IgG4-negative type 1 autoimmune pancreatitis. ( 30414073 )
2018
49
The role of EUS-guided fine needle aspiration in autoimmune pancreatitis: a single center prospective study. ( 30422724 )
2018
50
Autoimmune pancreatitis after nivolumab anti-programmed death receptor-1 treatment. ( 30454710 )
2018

Variations for Autoimmune Pancreatitis

Expression for Autoimmune Pancreatitis

Search GEO for disease gene expression data for Autoimmune Pancreatitis.

Pathways for Autoimmune Pancreatitis

GO Terms for Autoimmune Pancreatitis

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of B cell proliferation GO:0030890 9.46 FCRL3 TNFSF13B
2 negative regulation of interferon-gamma production GO:0032689 9.43 FOXP3 IL10
3 negative regulation of interleukin-6 production GO:0032715 9.4 FOXP3 IL10
4 B cell homeostasis GO:0001782 9.37 FOXP3 TNFSF13B
5 defense response to protozoan GO:0042832 9.32 HRAS IL10
6 negative regulation of B cell proliferation GO:0030889 9.26 CTLA4 IL10
7 regulation of regulatory T cell differentiation GO:0045589 9.16 CTLA4 FOXP3
8 negative regulation of immune response GO:0050777 8.96 CTLA4 FOXP3
9 negative regulation of T cell proliferation GO:0042130 8.8 CTLA4 FOXP3 IL10

Sources for Autoimmune Pancreatitis

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
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