MCID: MKL001
MIFTS: 37

Mikulicz Disease

Categories: Bone diseases, Endocrine diseases, Eye diseases, Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Mikulicz Disease

MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 12 53 59 15 73
Igg4-Related Dacryoadenitis and Sialadenitis 12 53 59
Chronic Dacryoadenitis and Sialadenitis 53 59
Mikulicz's Disease 12 53
Mikulicz Syndrome 76 53
Mikulicz' Disease 44

Characteristics:

Orphanet epidemiological data:

59
igg4-related dacryoadenitis and sialadenitis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:



External Ids:

Disease Ontology 12 DOID:12900
ICD10 33 K11.8
MeSH 44 D008882
NCIt 50 C34819
SNOMED-CT 68 7826003
Orphanet 59 ORPHA79078
UMLS via Orphanet 74 C0026103
ICD10 via Orphanet 34 K11.8
MESH via Orphanet 45 D008882
UMLS 73 C0026103

Summaries for Mikulicz Disease

NIH Rare Diseases : 53 IgG4-related dacryoadenitis and sialoadenitis (formerly called Mikulicz disease) is an IgG4-related disease characterized by inflammation of the lacrimal glands (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. 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 Prednisolone and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include salivary gland, eye and liver, and related phenotype is homeostasis/metabolism.

Wikipedia : 76 Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands. ... more...

Related Diseases for Mikulicz Disease

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

(show all 23)
# Related Disease Score Top Affiliating Genes
1 dacryoadenitis 30.9 AQP5 IL21
2 sialadenitis 10.6
3 sjogren syndrome 10.0
4 multicentric castleman disease 9.9 IL21 IL5
5 cardiac sarcoidosis 9.9 IL5 SMUG1
6 nut allergy 9.9 IL5 PTGDR2
7 folliculitis 9.9 IL5 PTGDR2
8 allergic conjunctivitis 9.9 IL5 PTGDR2
9 lung cancer 9.9
10 autoimmune pancreatitis 9.9
11 lymphoma 9.9
12 pancreatitis 9.9
13 connective tissue disease 9.9
14 lymphosarcoma 9.9
15 lupus erythematosus 9.9
16 gastric lymphoma 9.8
17 mastitis 9.8
18 nasal cavity disease 9.8 IL5 PTGDR2
19 nose disease 9.8 IL5 PTGDR2
20 leukocyte disease 9.8 IL5 SMUG1
21 upper respiratory tract disease 9.8 IL5 PTGDR2
22 respiratory system disease 9.8 IL5 SMUG1
23 common variable immunodeficiency 9.7 IL21 IL5

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



Diseases related to Mikulicz Disease

Symptoms & Phenotypes for Mikulicz Disease

MGI Mouse Phenotypes related to Mikulicz Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.1 AQP5 IL21 IL5 PHB PTGDR2 SMUG1

Drugs & Therapeutics for Mikulicz Disease

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

(show all 22)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
2
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Methylprednisolone acetate Phase 4
9 Immunoglobulins Phase 4
10 Immunosuppressive Agents Phase 4
11 Immunologic Factors Phase 4
12 Antineoplastic Agents, Hormonal Phase 4
13 Hormone Antagonists Phase 4
14 Prednisolone acetate Phase 4
15 Immunoglobulin G Phase 4
16 Antibodies Phase 4
17 Antirheumatic Agents Phase 4
18 Hormones Phase 4
19 Anti-Inflammatory Agents Phase 4
20 glucocorticoids Phase 4
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
22 Liver Extracts Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting 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

Genetic Tests for Mikulicz Disease

Anatomical Context for Mikulicz Disease

MalaCards organs/tissues related to Mikulicz Disease:

41
Salivary Gland, Eye, Liver, Bone, Lung

Publications for Mikulicz Disease

Articles related to Mikulicz Disease:

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

Variations for Mikulicz Disease

Expression for Mikulicz Disease

Search GEO for disease gene expression data for Mikulicz Disease.

Pathways for Mikulicz Disease

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

# Super pathways Score Top Affiliating Genes
1 11.02 IL5 PTGDR2
2 10.53 IL5 PTGDR2

GO Terms for Mikulicz Disease

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

Sources for Mikulicz Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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