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AIP
MCID: ATM024
MIFTS: 50

Autoimmune Pancreatitis (AIP)

Categories: Bone diseases, Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Genetic diseases, Immune diseases, Rare diseases
Genes Variations 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 72
Lymphoplasmocytic Sclerosing Pancreatitis 53
Aip 59

Characteristics:

Orphanet epidemiological data:

59
autoimmune pancreatitis
Inheritance: Not applicable;

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases Cancer 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
ICD10 via Orphanet 34 K86.1
UMLS via Orphanet 73 C2609129
Orphanet 59 ORPHA103919
UMLS 72 C2609129
Sources

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 recurrent acute pancreatitis and alcoholic pancreatitis. An important gene associated with Autoimmune Pancreatitis is CA2 (Carbonic Anhydrase 2), and among its related pathways/superpathways are Allograft rejection and NF-kappaB Signaling. The drugs rituximab and Immunoglobulin G have been mentioned in the context of this disorder. Affiliated tissues include pancreas, kidney and salivary gland, and related phenotypes are digestive/alimentary and endocrine/exocrine gland

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

Wikipedia : 75 Autoimmune pancreatitis is an increasingly recognized type of chronic pancreatitis that can be difficult... 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 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 370)
# Related Disease Score Top Affiliating Genes
1 recurrent acute pancreatitis 30.9 PRSS1 CFTR
2 alcoholic pancreatitis 30.8 PRSS1 CFTR
3 colitis 29.6 IL10 FOXP3 CTLA4
4 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 29.3 TNFSF13B IL10 FOXP3 CTLA4
5 chronic graft versus host disease 29.1 TNFSF13B IL10 CD4
6 myasthenia gravis 28.8 TNFSF13B IL10 CTLA4
7 autoimmune disease 28.6 TNFSF13B IL10 FOXP3 FCRL3 ENO1 CTLA4
8 rheumatoid arthritis 28.0 TNFSF13B IL10 FOXP3 FCRL3 CTLA4
9 systemic lupus erythematosus 27.9 TNFSF13B IL10 FOXP3 FCRL3 CTLA4
10 aip-related familial isolated pituitary adenomas 12.6
11 autoimmune pancreatitis type 1 12.5
12 autoimmune pancreatitis type 2 12.4
13 porphyria, acute intermittent 12.4
14 acromegaly 12.3
15 pituitary adenoma 1, multiple types 12.2
16 acute interstitial pneumonia 12.1
17 pituitary adenoma, prolactin-secreting 12.1
18 pituitary adenoma 11.9
19 familial isolated pituitary adenoma 11.7
20 igg4-related disease 11.7
21 gigantism 11.7
22 acroleukopathy, symmetric 11.7
23 pituitary tumors 11.7
24 pituitary apoplexy 11.7
25 idiopathic interstitial pneumonia 11.7
26 hormone producing pituitary cancer 11.6
27 carney complex variant 11.6
28 multiple endocrine neoplasia, type iv 11.6
29 functioning pituitary adenoma 11.6
30 porphyria 11.6
31 acth-secreting pituitary adenoma 11.5
32 mikulicz disease 11.5
33 hepatitis b 11.5
34 chester porphyria 11.3
35 endocrine organ benign neoplasm 11.3
36 pituitary infarct 11.3
37 hyperpituitarism 11.3
38 cancer-associated retinopathy 11.3
39 pancreatitis 11.2
40 silent pituitary adenoma 11.1
41 null pituitary adenoma 11.1
42 pancreatic cancer 10.9
43 cholangitis 10.8
44 obstructive jaundice 10.8
45 sclerosing cholangitis 10.8
46 retroperitoneal fibrosis 10.6
47 acute pancreatitis 10.6
48 cholangitis, primary sclerosing 10.6
49 multiple endocrine neoplasia 10.5
50 sialadenitis 10.5

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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MGI Mouse Phenotypes related to Autoimmune Pancreatitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.63 CA2 CD4 CFTR CTLA4 FOXP3 IL10
2 endocrine/exocrine gland MP:0005379 9.5 CA2 CD4 CFTR CTLA4 FOXP3 IL10
3 normal MP:0002873 9.17 CD4 CFTR CTLA4 FOXP3 IL10 KCNA3
Sources

Drugs & Therapeutics for Autoimmune Pancreatitis

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

(show all 39)
# 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
3 Antineoplastic Agents, Immunological Phase 1, Phase 2
4 Antirheumatic Agents Phase 1, Phase 2
5
Lenalidomide Approved Phase 1 191732-72-6 216326
6 Angiogenesis Inhibitors Phase 1
7 Angiogenesis Modulating Agents Phase 1
8
Gemcitabine Approved 95058-81-4 60750
9
Pancrelipase Approved, Investigational 53608-75-6
10
Prednisone Approved, Vet_approved 53-03-2 5865
11
Hydrocortisone Approved, Vet_approved 50-23-7 5754
12
Hydrocortisone acetate Approved, Vet_approved 50-03-3
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 Anti-Infective Agents
18 Immunosuppressive Agents
19 Antimetabolites
20 Antimetabolites, Antineoplastic
21 Antiviral Agents
22 pancreatin
23 Antibodies
24 Immunologic Factors
25 Immunoglobulins
26 Micronutrients
27 Vitamins
28 Ergocalciferols
29 Nutrients
30 Vitamin D2
31 Trace Elements
32 Calciferol
33 Calcium, Dietary
34 Bone Density Conservation Agents
35 Hydrocortisone-17-butyrate
36 Anti-Inflammatory Agents
37 Hydrocortisone 17-butyrate 21-propionate
38 Antibodies, Antinuclear
39 Hydrocortisone hemisuccinate

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 Rituximab (RTX) for IgG4-related Disease (IgG4-RD): a Prospective,Open-label Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
2 Treatment of IgG4-Related Disease With Revlimid and Rituximab: The TIGR2 Trial Completed NCT02705638 Phase 1 Rituximab;Lenalidomide
3 EUS-guided Procore-biopsy in Diagnosing Autoimmune Pancreatitis Unknown status NCT01774513
4 A Large Sample Follow-up Study of Long-term Complications of Acute Pancreatitis Unknown status NCT02305914
5 Dynamic Changes of the Serum Level of Immunoglobulin G4(IgG4) and Interleukin-10(IL-10) in the Patients of Pancreatic Cancer After Gemcitabine-based Chemotherapy Unknown status NCT02654288
6 Pancreatic Duct Evaluation in Autoimmune Pancreatitis: Intraindividual Comparison of MR Pancreatography at 3.0 T and 1.5 T Completed NCT01773031
7 Clinical Analysis for Predicting of Relapse During Steroid Treatment for Autoimmune Pancreatitis (AIP) in Korean Population Based on the HLA Analysis by Using a High Resolution (Sequence Based) Techniques Completed NCT00444444
8 CT Perfusion of Pancreatic Cancer and Chronic Pancreatitis: Feasibility Study Completed NCT00588367
9 Evaluation of EUS-guided FNA for Diagnosing Autoimmune Pancreatitis: a Prospective, Single-center Study Completed NCT03241797
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
11 Endoscopic Ultrasound Guided Fine Needle Biopsy With a New Core Histology Needle Versus Conventional Fine Needle Aspiration. Completed NCT01774162
12 A Randomized Trial Comparing a Winged Perimeter Stent and Traditional Polyethylene Plastic Stent in the Management of Malignant Distal Biliary Strictures Completed NCT01514214
13 A Survey of Patients With Elevated Serum IGG4 With Unknown IGG4-related Disease to Whom Biopsy and Performance of Subsequent Specific Staining for Clinical, Laboratory and Pathologic Correlation. Completed NCT02229448
14 A Randomized Trial Comparing a 19-gauge EUS Fine-needle Aspiration Device With a 20-gauge Fine-needle Biopsy Device for the Diagnosis of Autoimmune Pancreatitis Recruiting NCT03753815
15 Evaluation of the FOCUS Diffusion's Added Clinical Value Compared to Conventional MRI With Wide Field View Diffusion in Focal Pancreatic Lesions Characterization Recruiting NCT03961646
16 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Active, not recruiting NCT02797665 corticosteroids
17 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
18 Clinical Usefulness of Cortisol, Antinuclear Antibodies and High-sensitivity C-reactive Protein in Diagnostic and Prognostic Evaluation of Acute Pancreatitis Not yet recruiting NCT03830060
19 Efficacy of Secretin Assisted Dynamic MRCP in the Diagnosis and Follow up of Auto Immune Pancreatitis(AIP) 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, Kidney, Salivary Gland, Liver, Lymph Node, T Cells, Bone

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

19
Pancreas
Sources

Publications for Autoimmune Pancreatitis

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Articles related to Autoimmune Pancreatitis:

(show top 50) (show all 1988)
# Title Authors PMID Year
1
Autoimmune Pancreatitis and IgG4-Related Disease: The Storiform Discovery to Treatment. 38
31363956 2019
2
IgG4-related Disease Involving the Cardiovascular System: An Intracardiac Mass and a Mass Lesion Surrounding a Coronary Artery. 38
31118383 2019
3
The Propagation Display Method Improves the Reproducibility of Pancreatic Shear Wave Elastography. 38
31103347 2019
4
The multifaceted nature of diabetes mellitus induced by checkpoint inhibitors. 38
31423559 2019
5
Native T1 mapping of autoimmune pancreatitis as a quantitative outcome surrogate. 38
30707275 2019
6
Variant and Specific Forms of Autoimmune Cholestatic Liver Diseases. 38
31165900 2019
7
Pancreatic tumor in type 1 autoimmune pancreatitis: a diagnostic challenge. 38
31419961 2019
8
Clinical features of isolated proximal-type immunoglobulin G4-related sclerosing cholangitis. 38
30570170 2019
9
Multimodality imaging studies of intraductal tubulopapillary neoplasms of the pancreas. 38
31147310 2019
10
Quantitative pancreatic MRI: a pathology-based review. 38
30982337 2019
11
Intestinal dysbiosis mediates experimental autoimmune pancreatitis via activation of plasmacytoid dendritic cells. 38
31287532 2019
12
Differentiation of Type 2 Autoimmune Pancreatitis Presenting as Clinical Acute Pancreatitis. 38
31284361 2019
13
Radiomics analysis for the differentiation of autoimmune pancreatitis and pancreatic ductal adenocarcinoma in 18 F-FDG PET/CT. 38
31348535 2019
14
Dramatic Improvement of Glycemic Control by Promptly Starting Steroid Therapy at an Early Stage of Autoimmune Pancreatitis in a Subject with Type 2 Diabetes. 38
31366799 2019
15
Immunoglobulin G4-related Liver Disease Overlapping with Non-alcoholic Steatohepatitis that was Diagnosed Simultaneously with Autoimmune Pancreatitis: A Case Report and Review of the Literature. 38
31366800 2019
16
Duct-obstructive pancreatitis with granulocytic epithelial lesion in a patient with ulcerative colitis: An atypical manifestation of type 2 autoimmune pancreatitis? 38
31219205 2019
17
Immunoglobulin G4-related sclerosing cholangitis. 38
31112324 2019
18
Delayed pancreatic ductal leakage after EUS-FNA for autoimmune pancreatitis. 38
30719995 2019
19
Autoimmune Pancreatitis Type 2: Diagnostic Utility of PD-L1 Immunohistochemistry. 38
31135488 2019
20
Diagnosis and management of IgG4-related disease. 38
31288262 2019
21
Autoimmune Pancreatitis Masquerading as Celiac Disease. 38
31206470 2019
22
Effectiveness of steroid therapy for pancreatic cysts complicating autoimmune pancreatitis and management strategy for cyst-related complications. 38
31164010 2019
23
Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type. 38
31248720 2019
24
Genetic analysis of IgG4-related disease. 38
31104539 2019
25
Differentiation of Autoimmune Pancreatitis from Pancreatic Cancer Remains Challenging. 38
30815742 2019
26
A Case of Diffuse Pancreatic Cancer Mimicking Autoimmune Pancreatitis. 38
31178502 2019
27
The Etiology of Pancreatic Manifestations in Patients with Inflammatory Bowel Disease. 38
31247968 2019
28
Serum and histological IgG4-negative type 1 autoimmune pancreatitis. 38
30414073 2019
29
Recent Updates on the Relationship between Cancer and Autoimmune Pancreatitis. 38
30713326 2019
30
Autoimmune Pancreatitis Exhibiting Pancreatic Duct Wall Thickening. 38
30713329 2019
31
A case of alopecia as IgG4-related skin disease. 38
27846747 2019
32
Female urinary retention from a huge periurethral mass caused by immunoglobulin G4-related disease (IgG4-RD). 38
31211055 2019
33
Type 1 Autoimmune Pancreatitis with Imaging Appearance Similar to That of Malignant Cystic Tumor. 38
31275090 2019
34
The Challenge of Autoimmune Pancreatitis: A Portrayal From the Pediatric Perspective. 38
31090661 2019
35
Autoimmune pancreatitis with spontaneous remission on 18F-fluorodeoxyglucose positron emission tomography/computed tomography. 38
31191774 2019
36
Methotrexate as Induction of Remission Therapy for Type 1 Autoimmune Pancreatitis. 38
30998520 2019
37
Risk Factors for Rate of Relapse and Effects of Steroid Maintenance Therapy in Patients With Autoimmune Pancreatitis: Systematic Review and Meta-analysis. 38
30312787 2019
38
PLA2R-Negative Membranous Nephropathy Presenting as a Prodrome of IgG4-Related Autoimmune Pancreatitis. 38
31367609 2019
39
A Diagnostically Challenging Case of Autoimmune Pancreatitis Due to Contamination of the Pathological Specimen with Early Gastric Cancer. 38
30626846 2019
40
Differentiating focal autoimmune pancreatitis and pancreatic ductal adenocarcinoma: contrast-enhanced MRI with special emphasis on the arterial phase. 38
31028441 2019
41
Feasibility and usefulness of endoscopic ultrasonography-guided shear-wave measurement for assessment of autoimmune pancreatitis activity: a prospective exploratory study. 38
30993580 2019
42
Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience. 38
30970429 2019
43
Clinicopathological and immunological features of follicular pancreatitis - a distinct disease entity characterised by Th17 activation. 38
30515871 2019
44
Method development of immunoglobulin G purification from micro-volumes of human serum for untargeted and targeted proteomics-based antibody repertoire studies. 38
30987718 2019
45
Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis. 38
31045595 2019
46
Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis. 38
31033594 2019
47
Management of Autoimmune Pancreatitis. 38
31042584 2019
48
Multifocal gastrointestinal varices: a rare manifestation of immunoglobulin G4-related disease. 38
30632442 2019
49
Etiology and Risk Factors of Acute and Chronic Pancreatitis. 38
31192240 2019
50
IgG4-related disease: what a hematologist needs to know. 38
30705099 2019
Sources

Variations for Autoimmune Pancreatitis

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Cosmic variations for Autoimmune Pancreatitis:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM132853 NFE2L2 kidney,NS,carcinoma,Type 2 c.245A>G p.E82G 2:177234072-177234072 3
2 COSM691 MET kidney,NS,carcinoma,Type 1 c.3803T>C p.M1268T 7:116783420-116783420 3
3 COSM3724572 MET kidney,NS,carcinoma,Type 1 c.3328G>A p.V1110I 7:116777403-116777403 3
4 COSM690 MET kidney,NS,carcinoma,Type 1 c.3742T>C p.Y1248H 7:116783359-116783359 3
5 COSM3724573 MET kidney,NS,carcinoma,Type 1 c.3476C>G p.S1159W 7:116778857-116778857 3
6 COSM3724578 MET kidney,NS,carcinoma,Type 1 c.3637C>T p.L1213F 7:116782048-116782048 3
7 COSM3724575 MET kidney,NS,carcinoma,Type 1 c.3538C>T p.H1180Y 7:116778919-116778919 3
8 COSM3724577 MET kidney,NS,carcinoma,Type 1 c.3637C>A p.L1213I 7:116782048-116782048 3
9 COSM704 MET kidney,NS,carcinoma,Type 1 c.3446T>C p.M1149T 7:116778827-116778827 3
10 COSM3724576 MET kidney,NS,carcinoma,Type 1 c.3616G>T p.V1206L 7:116782027-116782027 3
Sources

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
Allograft rejection 37 1
Show member pathways
 12.29 TNFSF13B IL10 FOXP3 CTLA4 CD4
2
NF-kappaB Signaling 4
12.14 TNFSF13B IL10 FOXP3 CTLA4
3
Pancreatic secretion 37
11.44 PRSS1 CFTR CA2
4
B Cell Development Pathways 65
10.86 IL10 FCRL3 CD4
5
Cytokines and Inflammatory Response 1
10.83 IL10 CD4
6
G-protein signaling_RhoB regulation pathway 22
10.81 UBR2 TNFSF13B IL10
7
Th2 Differentiation Pathway 65
10.46 IL10 FOXP3 CD4
Sources

GO Terms for Autoimmune Pancreatitis

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

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.92 FCRL3 ENO1 CFTR CD4

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.67 TNFSF13B IL10 CTLA4 CD4
2 positive regulation of B cell proliferation GO:0030890 9.48 TNFSF13B FCRL3
3 negative regulation of interleukin-6 production GO:0032715 9.46 IL10 FOXP3
4 negative regulation of interferon-gamma production GO:0032689 9.43 IL10 FOXP3
5 cytokine production GO:0001816 9.4 FOXP3 CD4
6 B cell homeostasis GO:0001782 9.32 TNFSF13B FOXP3
7 regulation of regulatory T cell differentiation GO:0045589 9.26 FOXP3 CTLA4
8 negative regulation of B cell proliferation GO:0030889 9.16 IL10 CTLA4
9 negative regulation of immune response GO:0050777 8.96 FOXP3 CTLA4
10 negative regulation of T cell proliferation GO:0042130 8.8 IL10 FOXP3 CTLA4

Molecular functions related to Autoimmune Pancreatitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein tyrosine kinase binding GO:1990782 8.62 FCRL3 CD4
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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
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 TGDB
71 Tocris
72 UMLS
73 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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