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MCID: MKL001
MIFTS: 39

Mikulicz Disease

Categories: Bone diseases, Endocrine diseases, Eye diseases, Immune diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Mikulicz Disease

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MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 12 54 60 15 74
Igg4-Related Dacryoadenitis and Sialadenitis 12 54 60
Chronic Dacryoadenitis and Sialadenitis 54 60
Mikulicz's Disease 12 54
Mikulicz Syndrome 77 54
Mikulicz' Disease 45

Characteristics:

Orphanet epidemiological data:

60
igg4-related dacryoadenitis and sialadenitis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Eye diseases Bone diseases Neuronal diseases Immune diseases Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 34 35
Orphanet: 60  
Rare eye diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:12900
MeSH 45 D008882
NCIt 51 C34819
SNOMED-CT 69 7826003
ICD10 34 K11.8
MESH via Orphanet 46 D008882
ICD10 via Orphanet 35 K11.8
UMLS via Orphanet 75 C0026103
Orphanet 60 ORPHA79078
UMLS 74 C0026103
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Summaries for Mikulicz Disease

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NIH Rare Diseases : 54 IgG4-related dacryoadenitis and sialoadenitis (formerly called Mikulicz disease) is an IgG4-related disease characterized by inflammation of the lacrimal glandsĀ (which produce tears), parotid glands, and submandibular glands (two of the major salivary glands). In some cases, it also affects other glands or organs. The condition is usually painless, mainly causing mouth and eye dryness, and swelling over the affected glands. When other organs are affected, it can be accompanied by complications such as autoimmune pancreatitis, retroperitoneal fibrosis, and tubulointerstitial nephritis. The underlying cause of IgG4-related disease is still not known. Treatment involves corticosteroids, which are usually effective. Medicines that suppress the immune system (immunosuppressants) may also be used in cases that do not respond to corticosteroids. IgG4-related dacryoadenitis and sialoadenitis was previously considered a subtype of Sjogren syndrome, but it is now known to be a distinct condition.

MalaCards based summary : Mikulicz Disease, also known as igg4-related dacryoadenitis and sialadenitis, is related to dacryoadenitis and sialadenitis, and has symptoms including lumps of the face An important gene associated with Mikulicz Disease is IL21 (Interleukin 21), and among its related pathways/superpathways are Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers and Innate Lymphoid Cell Differentiation Pathways. The drugs Leflunomide and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include eye, salivary gland and liver, and related phenotype is homeostasis/metabolism.

Disease Ontology : 12 An autoimmune hypersensitivity disease that is characterized by chronic enlargement of the glands of the head and neck, has symptom lumps of the face and neck, dry eyes, dry mouth, and intermittent fever, and is often associated with an underlying autoimmune disorder, hematologic malignancy, tuberculosis infection, or syphilis infection.

Wikipedia : 77 Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands. ... more...

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Related Diseases for Mikulicz Disease

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Diseases related to Mikulicz Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 50)
# Related Disease Score Top Affiliating Genes
1 dacryoadenitis 31.0 AQP5 IL21
2 sialadenitis 10.7
3 igg4-related disease 10.2
4 sjogren syndrome 10.1
5 interstitial nephritis 10.0
6 thyroiditis 10.0
7 lung cancer 10.0
8 autoimmune pancreatitis 10.0
9 lymphoma 10.0
10 pancreatitis 10.0
11 connective tissue disease 10.0
12 lymphosarcoma 10.0
13 lupus erythematosus 10.0
14 gastric lymphoma 9.9
15 mastitis 9.9
16 autoimmune disease 9.9
17 radin blood group antigen 9.9
18 lymphoma, mucosa-associated lymphoid type 9.9
19 myeloma, multiple 9.9
20 autoimmune disease 1 9.9
21 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
22 epidermolysis bullosa dystrophica 9.9
23 mantle cell lymphoma 9.9
24 thrombocytopenia 9.9
25 parotitis 9.9
26 hypothyroidism 9.9
27 lymphadenitis 9.9
28 mononeuritis multiplex 9.9
29 cystadenoma 9.9
30 epidermolysis bullosa 9.9
31 churg-strauss syndrome 9.9
32 hyperglobulinemic purpura 9.9
33 purpura 9.9
34 miliary tuberculosis 9.9
35 allergic angiitis 9.9
36 lymphocytic hypophysitis 9.9
37 retroperitoneal fibrosis 9.9
38 igg4-related submandibular gland disease 9.9
39 distal trisomy 3p 9.9
40 multicentric castleman disease 9.9 IL21 IL5
41 nut allergy 9.8 IL5 PTGDR2
42 folliculitis 9.8 IL5 PTGDR2
43 allergic conjunctivitis 9.8 IL5 PTGDR2
44 nasal cavity disease 9.8 IL5 PTGDR2
45 nose disease 9.8 IL5 PTGDR2
46 leukocyte disease 9.7 IL5 SMUG1
47 upper respiratory tract disease 9.7 IL5 PTGDR2
48 respiratory system disease 9.7 IL5 SMUG1
49 dermatitis, atopic 9.6 IL5 PTGDR2
50 common variable immunodeficiency 9.6 IL21 IL5

Graphical network of the top 20 diseases related to Mikulicz Disease:



Diseases related to Mikulicz Disease
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Symptoms & Phenotypes for Mikulicz Disease

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Symptoms:

12
  • • lumps of the face

MGI Mouse Phenotypes related to Mikulicz Disease:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.1 AQP5 IL21 IL5 PHB PTGDR2 SMUG1
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Drugs & Therapeutics for Mikulicz Disease

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Drugs for Mikulicz Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
2
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
3
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
4
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
5
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
6
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Immunoglobulins Phase 4
9 Prednisolone acetate Phase 4
10 Anti-Inflammatory Agents Phase 4
11 Hormones Phase 4
12 Immunoglobulin G Phase 4
13 Immunosuppressive Agents Phase 4
14 Antibodies Phase 4
15 Antineoplastic Agents, Hormonal Phase 4
16 Hormone Antagonists Phase 4
17 Immunologic Factors Phase 4
18 Methylprednisolone Acetate Phase 4
19 Liver Extracts Phase 4
20 glucocorticoids Phase 4
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
2 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695

Search NIH Clinical Center for Mikulicz Disease

Cochrane evidence based reviews: mikulicz' disease

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Genetic Tests for Mikulicz Disease

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Anatomical Context for Mikulicz Disease

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MalaCards organs/tissues related to Mikulicz Disease:

42
Eye, Salivary Gland, Liver, Bone, Lung
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Publications for Mikulicz Disease

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Articles related to Mikulicz Disease:

(show all 24)
# Title Authors Year
1
The diagnostic utility of submandibular gland sonography and labial salivary gland biopsy in IgG4-related dacryoadenitis and sialadenitis: its potential application to the diagnostic criteria. ( 30696319 )
2019
2
Mikulicz Disease Mimicking Intraorbital Tumors. ( 28987847 )
2018
3
Early Therapeutic Intervention for IgG4-related Dacryoadenitis and Sialadenitis: The Balance Between Risk of Observation Only and Therapeutic Adverse Effects. ( 30173184 )
2018
4
Interleukin 5-producing ST2+ memory Th2 cells in IgG4-related dacryoadenitis and sialadenitis. ( 30354922 )
2018
5
A rare case of Mikulicz disease. ( 27728670 )
2016
6
IgG4-related Mikulicz Disease in a 76-year-old Chinese Male: A Case Report. ( 24432837 )
2014
7
"Quasi-symmetric" FDG avidity of bilateral salivary glands in Mikulicz disease yielding false-positive staging in lung cancer. ( 22996254 )
2012
8
Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. ( 21898360 )
2012
9
Connective tissue diseases: Immune responses in Mikulicz disease differ from those in SS. ( 21971284 )
2011
10
Highly active state of autoimmune pancreatitis with mikulicz disease. ( 19904224 )
2010
11
Clinicopathologic similarities between Mikulicz disease and KA1ttner tumor. ( 20015799 )
2010
12
Sonographic diagnosis for Mikulicz disease. ( 19451003 )
2009
13
F-18 FDG PET-CT findings in Mikulicz disease and systemic involvement of IgG4-related lesions. ( 19352281 )
2009
14
Diffuse periairway thickening in patients with Mikulicz disease. ( 18728553 )
2008
15
Mikulicz disease of a minor salivary gland. ( 280669 )
1978
16
Mikulicz disease and subsequent lupus erythematosus development. ( 576964 )
1977
17
Malignant lymphoma of parotid associated with Mikulicz disease (benign lymphoepithelial lesion). ( 4943296 )
1971
18
The parotid gland in Mikulicz disease and Sjogren's syndrome. ( 13713214 )
1960
19
The parotid gland in Mikulicz disease and Sjogren's syndrome. ( 13713215 )
1960
20
Mikulicz' disease: report of a case of one week's duration. ( 14908801 )
1952
21
Benign lymphoepithelial lesion of the parotid gland adenolymphoma, chronic inflammation, lymphoepithelioma, lymphocytic tumor, Mikulicz disease. ( 12998015 )
1952
22
Mikulicz' disease and Mikulicz' syndrome. ( 18119668 )
1949
23
A CASE OF MIKULICZ' DISEASE. ( 18168915 )
1930
24
LYMPHOID HYPERPLASIA OF LACRYMAL AND SALIVARY GLANDS: MIKULICZ' DISEASE. ( 17865927 )
1928
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Variations for Mikulicz Disease

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Expression for Mikulicz Disease

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Search GEO for disease gene expression data for Mikulicz Disease.
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Pathways for Mikulicz Disease

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Pathways related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 66
11.02 IL5 PTGDR2
2
Innate Lymphoid Cell Differentiation Pathways 66
10.53 IL5 PTGDR2
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GO Terms for Mikulicz Disease

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Biological processes related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.33 IL21 IL5 PTGDR2
2 positive regulation of B cell proliferation GO:0030890 8.96 IL21 IL5
3 positive regulation of G protein-coupled receptor signaling pathway GO:0045745 8.62 PHB PTGDR2
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Sources for Mikulicz Disease

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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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