AIP
MCID: ATM024
MIFTS: 47

Autoimmune Pancreatitis (AIP) malady

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

Aliases & Classifications for Autoimmune Pancreatitis

Aliases & Descriptions for Autoimmune Pancreatitis:

Name: Autoimmune Pancreatitis 50 56 69
Lymphoplasmocytic Sclerosing Pancreatitis 50
Aip 56

Characteristics:

Orphanet epidemiological data:

56
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 56 ORPHA103919
UMLS via Orphanet 70 C2609129
ICD10 via Orphanet 34 K86.1

Summaries for Autoimmune Pancreatitis

NIH Rare Diseases : 50 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 b-cell lymphomas and hepatitis. 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 Secretin and Gastrointestinal Agents 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 : 71 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
Cftr-Related Hereditary Pancreatitis Ctrc-Related Hereditary Pancreatitis
Spink1-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 171)
id Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 29.5 CTLA4 HLA-DQB1 HLA-DRB1
2 hepatitis 29.4 CTLA4 HLA-DRB1 TNF
3 rheumatoid arthritis 28.9 CTLA4 HLA-DQB1 HLA-DRB1 TNF
4 aip-related familial isolated pituitary adenomas 12.3
5 autoimmune pancreatitis type 1 12.0
6 autoimmune pancreatitis type 2 11.8
7 acromegaly 11.7
8 porphyria, acute intermittent 11.7
9 pituitary adenoma, prolactin-secreting 11.6
10 pituitary adenoma, acth-secreting 11.4
11 pituitary adenoma 11.4
12 igg4-related disease 11.1
13 pancreatitis 10.9
14 pancreatic cancer 10.3
15 cholangitis 10.3
16 sclerosing cholangitis 10.2
17 apple allergy 10.2 HLA-DQB1 HLA-DRB1
18 hemoglobin d disease 10.2 HLA-DQB1 HLA-DRB1
19 immune deficiency, familial variable 10.2 HLA-DQB1 HLA-DRB1
20 lin-gettig syndrome 10.2 HLA-DQB1 HLA-DRB1
21 midline interhemispheric variant of holoprosencephaly 10.2 HLA-DQB1 HLA-DRB1
22 renal agenesis meningomyelocele mullerian defect 10.2 HLA-DQB1 HLA-DRB1
23 lyme disease - neurological complications 10.2 HLA-DQB1 HLA-DRB1
24 learning disability 10.2 HLA-DQB1 HLA-DRB1
25 linear lichen planus 10.2 HLA-DQB1 HLA-DRB1
26 night blindness 10.2 HLA-DQB1 HLA-DRB1
27 cervical adenoid basal carcinoma 10.2 HLA-DQB1 HLA-DRB1
28 neonatal antiphospholipid syndrome 10.2 HLA-DQB1 HLA-DRB1
29 myofibroma 10.2 HLA-DQB1 HLA-DRB1
30 oromandibular dystonia 10.2 HLA-DQB1 HLA-DRB1
31 t-cell prolymphocytic leukemia 10.2 HLA-DQB1 TNF
32 celiac disease 3 10.2 CTLA4 HLA-DRB1
33 anaerobic meningitis 10.2 HLA-DQB1 HLA-DRB1
34 mirizzi syndrome 10.2 HLA-DRB1 TNF
35 complement component 8 deficiency 10.2 CTLA4 HLA-DRB1
36 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 10.2 HLA-DQB1 TNF
37 swine influenza 10.2 HLA-DRB1 TNF
38 pneumoconiosis 10.1 HLA-DQB1 TNF
39 ragweed sensitivity 10.1 HLA-DQB1 HLA-DRB1
40 human immunodeficiency virus infectious disease 10.1 HLA-DRB1 TNF
41 migraine with aura 10.1 HLA-DQB1 TNF
42 right ventricle hypoplasia 10.1 HLA-DRB1 TNF
43 aseptic meningitis 10.1 HLA-DRB1 TNF
44 pancreatic signet ring cell adenocarcinoma 10.1 HLA-DRB1 TNF
45 hereditary alpha tryptasemia syndrome 10.1 HLA-DRB1 TNF
46 benign deep fibrous histiocytoma 10.1 HLA-DRB1 TNF
47 motility-related diarrhea 10.1 HLA-DRB1 TNF
48 folinic acid-responsive seizures 10.1 HLA-DQB1 HLA-DRB1
49 endogenous depression 10.1 HLA-DRB1 TNF
50 infantile hypotonia 10.1 HLA-DQB1 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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.35 CA2 CFTR CTLA4 HLA-DQB1 TNF
2 endocrine/exocrine gland MP:0005379 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 43)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Secretin Approved, Investigational Phase 4 108153-74-8
2 Gastrointestinal Agents Phase 4,Phase 3,Phase 2
3 Hormone Antagonists Phase 4,Phase 3,Phase 2
4 Hormones Phase 4,Phase 3,Phase 2
5 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2
6
Prednisolone Approved, Vet_approved Phase 3,Phase 2 50-24-8 5755
7
Methylprednisolone Approved, Vet_approved Phase 3,Phase 2 83-43-2 6741
8 Prednisolone acetate Phase 3,Phase 2
9 glucocorticoids Phase 3,Phase 2
10 Neuroprotective Agents Phase 3,Phase 2
11 Peripheral Nervous System Agents Phase 3,Phase 2
12 Prednisolone hemisuccinate Phase 3,Phase 2
13 Prednisolone phosphate Phase 3,Phase 2
14 Protective Agents Phase 3,Phase 2
15 Antiemetics Phase 3,Phase 2
16 Anti-Inflammatory Agents Phase 3,Phase 2
17 Methylprednisolone acetate Phase 3,Phase 2
18 Methylprednisolone Hemisuccinate Phase 3,Phase 2
19 Autonomic Agents Phase 3,Phase 2
20 Antineoplastic Agents, Hormonal Phase 3,Phase 2
21 Trace Elements Phase 3
22 Vitamins Phase 3
23 Micronutrients Phase 3
24
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
25 insulin Phase 2
26 pancreatic polypeptide Phase 2
27 Hypoglycemic Agents Phase 2
28 Insulin, Globin Zinc Phase 2
29 Liver Extracts Phase 2
30 Immunoglobulin G Phase 1, Phase 2
31 Immunoglobulins Phase 1, Phase 2
32 Antibodies Phase 1, Phase 2
33 Antirheumatic Agents Phase 1, Phase 2
34
Lenalidomide Approved Phase 1 191732-72-6 216326
35 Angiogenesis Inhibitors Phase 1
36 Angiogenesis Modulating Agents Phase 1
37 Anti-Bacterial Agents Phase 1
38 Anti-Infective Agents Phase 1
39 Immunosuppressive Agents Phase 1
40
Pancrelipase Approved 53608-75-6
41
Ethanol Approved 64-17-5 702
42
Prednisone Approved, Vet_approved 53-03-2 5865
43 pancreatin

Interventional clinical trials:

(show all 15)
id Name Status NCT ID Phase
1 Efficacy of Secretin MRCP in the Diagnosis and Follow up of Auto Immune Pancreatitis Unknown status NCT01845467 Phase 4
2 Comparison of Bovine Colostrum Versus Placebo in Treatment of Severe Alcoholic Hepatitis: A Randomized Double Blind Controlled Trial Not yet recruiting NCT02473341 Phase 3
3 The Effect of Pancreatic Polypeptide on Insulin Requirements for Type 1 & Post-pancreatectomy Diabetic Patients Unknown status NCT00791076 Phase 2
4 Rituximab in IgG4-related Disease: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2
5 Efficacy of Combination Therapy of Glucocorticoids and Bovine Colostrum in Treatment of Severe Alcoholic Hepatitis. Completed NCT02265328 Phase 2
6 Evaluation of a New EUS Guided Biopsy Needle (SharkCore) Comparing to Standard EUS Needle (ProCore) Withdrawn NCT02766842 Phase 2
7 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1
8 EUS-guided Procore-biopsy in Diagnosing Autoimmune Pancreatitis Unknown status NCT01774513
9 Follow-up Study of Complications of Acute Pancreatitis Unknown status NCT02305914
10 Pancreatic Duct Evaluation in Autoimmune Pancreatitis: MR Pancreatography Completed NCT01773031
11 Genetic Analysis for Predicting of Relapse During Steroid Treatment for Autoimmune Pancreatitis (AIP) Completed NCT00444444
12 CT Pancreas Perfusion Completed NCT00588367
13 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Recruiting NCT02797665
14 A Randomized Prospective Comparison of the New EUS Guided Acquire TM Needle Biopsy vs EUS Guided Fine Needle Aspiration for Suspected Solid Gastrointestinal Lesions Recruiting NCT03109639
15 Utility of EUS-elastography to Predict Portal Hypertension Recruiting NCT03155282

Search NIH Clinical Center for Autoimmune Pancreatitis

Genetic Tests for Autoimmune Pancreatitis

Anatomical Context for Autoimmune Pancreatitis

MalaCards organs/tissues related to Autoimmune Pancreatitis:

39
Pancreas, Salivary Gland, Kidney, Lymph Node, T Cells, Bone, Colon

Publications for Autoimmune Pancreatitis

Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 648)
id Title Authors Year
1
Autoimmune Pancreatitis Complicated With Pancreatic Ascites, Pancreatic Ductal Leakage, and Multiple Pseudocyst. ( 27977636 )
2017
2
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
3
Preventing disease relapses in autoimmune pancreatitis with maintenance steroids: are we there yet? ( 27707776 )
2016
4
Association of newly diagnosed type 1 diabetes and autoimmune pancreatitis. ( 27855231 )
2016
5
Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis. ( 27543430 )
2016
6
A Case Report of Childhood Recurrent Autoimmune Pancreatitis: A Rare Emerging Entity. ( 27504369 )
2016
7
Autoimmune Pancreatitis: A Case of Atypical Radiographic Findings. ( 27920645 )
2016
8
An Unusual Presentation of Pediatric Autoimmune Pancreatitis. ( 26752256 )
2016
9
Type 1 autoimmune pancreatitis: case scenario and review of the disease. ( 27716763 )
2016
10
Autoimmune Pancreatitis:Typing,Diagnosis,and Treatment. ( 28065243 )
2016
11
Autoimmune Pancreatitis Can Transform Into Chronic Features Similar to Advanced Chronic Pancreatitis With Functional Insufficiency Following Severe Calcification. ( 27400257 )
2016
12
Assessing the usefulness of salivary gland biopsy for diagnosis of type-1 autoimmune pancreatitis. ( 27426059 )
2016
13
Dilemmas in autoimmune pancreatitis. Surgical resection or not? ( 27546699 )
2016
14
Identification of Novel Serum Autoantibodies for Differential Diagnosis of Autoimmune Pancreatitis and Pancreatic Ductal Adenocarcinoma. ( 27623556 )
2016
15
A Pitfall to Avoid: Hyperlipasemia, Azathioprine, and Autoimmune Pancreatitis. ( 27385401 )
2016
16
Mass-forming type 1 autoimmune pancreatitis mimicking pancreatic cancer. ( 27121246 )
2016
17
Prospective multicenter study on the usefulness of EUS-guided FNAA biopsy for the diagnosis of autoimmune pancreatitis. ( 26777565 )
2016
18
Diagnosis, treatment and long-term outcomes of autoimmune pancreatitis in Spain based on the International Consensus DiagnosticA Criteria: A multi-centre study. ( 26944001 )
2016
19
Is autoimmune pancreatitis a subclass of chronic pancreatitis? ( 28034552 )
2016
20
A Rare Case of Diffuse Large B-cell Lymphoma in a Patient with IgG4-Related Autoimmune Pancreatitis. ( 27549673 )
2016
21
Sequential Evaluation of Pancreato-Biliary Findings in a Case with IgG4-Associated Cholangiopathy and Autoimmune Pancreatitis during Corticosteroid Treatment. ( 27606144 )
2016
22
Autoimmune Pancreatitis and Inflammatory Bowel Disease: An Updated Review. ( 27779612 )
2016
23
Long-term outcomes of autoimmune pancreatitis. ( 27678359 )
2016
24
Nonoverlapping roles of PD-1 and FoxP3 in maintaining immune tolerance in a novel autoimmune pancreatitis mouse model. ( 27410049 )
2016
25
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
26
Alteration of serum N-glycan profile in patients with autoimmune pancreatitis. ( 26723536 )
2016
27
Eosinophilic annular erythema in a patient with autoimmune pancreatitis: Nicotinamide therapy may be beneficial for achieving remission. ( 27129768 )
2016
28
Comment re: "Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting". ( 27329667 )
2016
29
Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting. ( 27107633 )
2016
30
Why is one arm stronger than two arms? IgG4 antibodies in IgG4-related autoimmune pancreatitis. ( 27196569 )
2016
31
Clinical and pathophysiological issues associated with type 1 autoimmune pancreatitis. ( 26864047 )
2016
32
Early pancreatic volume reduction on CT predicts relapse in patients with type 1 autoimmune pancreatitis treated with steroids. ( 27465035 )
2016
33
Relapsing sepsis episodes of Escherichia coli with CTX-M ESBL or derepressed ampC genes in a patient with chronic autoimmune pancreatitis complicated by IgG4 hypergammaglobulinaemia. ( 26862435 )
2016
34
Epidemiology, clinical presentation, diagnosis and treatment of autoimmune pancreatitis: A retrospective analysis of 53 patients. ( 26694138 )
2016
35
Diagnosis of autoimmune pancreatitis by endoscopic ultrasonography -guided fine-needle aspiration using a 22-gauge needle: a prospective multicenter study. ( 27068878 )
2016
36
Specific MAPK-Associated MicroRNAs in Serum Differentiate Pancreatic Cancer from Autoimmune Pancreatitis. ( 27380024 )
2016
37
Localized (18)F-fluorodeoxyglucose uptake at the pancreatic head during remission phase of autoimmune pancreatitis: A case report. ( 27602112 )
2016
38
Fine-needle aspiration for autoimmune pancreatitis-not ready forA prime time. ( 27425798 )
2016
39
Autoimmune pancreatitis or pancreatic cancer? ( 27048909 )
2016
40
Differentiating Mass-Forming Autoimmune Pancreatitis From Pancreatic Ductal Adenocarcinoma on the Basis of Contrast-Enhanced MRI and DWI Findings. ( 26797355 )
2016
41
EUS-guided FNA for diagnosing autoimmune pancreatitis: DoesA it enhance existing consensus criteria? ( 27742043 )
2016
42
A case of autoimmune pancreatitis effectively treated with an immunosuppressant (azathioprine). ( 27450404 )
2016
43
Role of contrast harmonic endoscopic ultrasonography in other pancreatic solid lesions: Neuroendocrine tumors, autoimmune pancreatitis and metastases. ( 28000628 )
2016
44
In vitro studies implicate an imbalanced activation of dendritic cells in the pathogenesis of murine autoimmune pancreatitis. ( 27356751 )
2016
45
Very high serum levels of CA 19-9 in autoimmune pancreatitis: report of 4 cases and brief review of literature. ( 27579898 )
2016
46
Autoimmune Pancreatitis and IgG4 Related Disease in Three Children. ( 27622194 )
2016
47
Transpapillary biliary stenting is a risk factor for pancreatic stones in patients with autoimmune pancreatitis. ( 27540582 )
2016
48
Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations. ( 27141136 )
2016
49
International consensus for the treatment of autoimmune pancreatitis. ( 28027896 )
2016
50
Autoimmune Pancreatitis: An Update on Diagnosis and Management. ( 26895679 )
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 19)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.41 CTLA4 HLA-DQB1 HLA-DRB1 TNF
2
Show member pathways
12.31 HLA-DQB1 HLA-DRB1 PRSS1 TNF
3
Show member pathways
12.3 HLA-DQB1 HLA-DRB1 TNF
4
Show member pathways
12.26 HLA-DQB1 HLA-DRB1 TNF
5 12.11 HLA-DQB1 HLA-DRB1 TNF
6
Show member pathways
12.07 CTLA4 HLA-DQB1 HLA-DRB1
7
Show member pathways
11.93 HLA-DQB1 HLA-DRB1 TNF
8 11.89 HLA-DQB1 HLA-DRB1 TNF
9
Show member pathways
11.85 CTLA4 HLA-DQB1 HLA-DRB1
10 11.74 CTLA4 HLA-DQB1 HLA-DRB1
11 11.44 CA2 CFTR
12 11.42 CTLA4 TNF
13 11.42 HLA-DQB1 HLA-DRB1 TNF
14 11.31 HLA-DQB1 HLA-DRB1
15 11.31 CA2 CFTR PRSS1
16 11.19 CA2 TNF
17 10.98 CTLA4 HLA-DQB1 HLA-DRB1
18 10.97 HLA-DRB1 TNF
19 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:

id Name GO ID Score Top Affiliating Genes
1 lysosomal membrane GO:0005765 9.5 CFTR HLA-DQB1 HLA-DRB1
2 endocytic vesicle membrane GO:0030666 9.46 HLA-DQB1 HLA-DRB1
3 ER to Golgi transport vesicle membrane GO:0012507 9.43 HLA-DQB1 HLA-DRB1
4 external side of plasma membrane GO:0009897 9.43 CTLA4 HLA-DRB1 TNF
5 transport vesicle membrane GO:0030658 9.4 HLA-DQB1 HLA-DRB1
6 clathrin-coated endocytic vesicle membrane GO:0030669 9.37 HLA-DQB1 HLA-DRB1
7 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.16 HLA-DQB1 HLA-DRB1
8 endosome membrane GO:0010008 9.13 CFTR HLA-DQB1 HLA-DRB1
9 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:

(show all 11)
id Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.56 CTLA4 HLA-DQB1 HLA-DRB1 TNF
2 interferon-gamma-mediated signaling pathway GO:0060333 9.49 HLA-DQB1 HLA-DRB1
3 antigen processing and presentation GO:0019882 9.48 HLA-DQB1 HLA-DRB1
4 bicarbonate transport GO:0015701 9.46 CA2 CFTR
5 negative regulation of T cell proliferation GO:0042130 9.43 CTLA4 HLA-DRB1
6 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.4 CFTR HLA-DRB1
7 positive regulation of osteoclast differentiation GO:0045672 9.37 CA2 TNF
8 T cell costimulation GO:0031295 9.33 CTLA4 HLA-DQB1 HLA-DRB1
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 8.96 HLA-DQB1 HLA-DRB1
11 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:

id 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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