MCID: MKL001
MIFTS: 27

Mikulicz Disease

Categories: Rare diseases, Eye diseases, Immune diseases

Aliases & Classifications for Mikulicz Disease

MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 12 50 14 69
Mikulicz's Disease 12 50
Mikulicz' Disease 42

Classifications:



External Ids:

Disease Ontology 12 DOID:12900
ICD10 33 K11.8
MeSH 42 D008882
NCIt 47 C34819
SNOMED-CT 64 196478007 7826003
UMLS 69 C0026103

Summaries for Mikulicz Disease

NIH Rare Diseases : 50 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. last updated: 9/20/2017

MalaCards based summary : Mikulicz Disease, also known as mikulicz's disease, is related to igg4-related dacryoadenitis and sialadenitis and sjogren's syndrome. An important gene associated with Mikulicz Disease is IL21 (Interleukin 21), and among its related pathways/superpathways is MAPK Signaling: Mitogen Stimulation Pathway. The drugs Prednisolone and Leflunomide have been mentioned in the context of this disorder. Affiliated tissues include salivary gland, eye and liver, and related phenotype is homeostasis/metabolism.

Related Diseases for Mikulicz Disease

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

id Related Disease Score Top Affiliating Genes
1 igg4-related dacryoadenitis and sialadenitis 10.9
2 sjogren's syndrome 9.8
3 connective tissue disease 9.6
4 lupus erythematosus 9.6
5 autoimmune pancreatitis 9.6
6 lung cancer 9.6
7 lymphoma 9.6
8 pancreatitis 9.6
9 loeffler syndrome 9.6 AQP5 IL21
10 sialolithiasis 7.6 AQP5 IL21 PHB PTGDR2 SMUG1

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.02 AQP5 IL21 PHB PTGDR2 SMUG1

Drugs & Therapeutics for Mikulicz Disease

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

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

Interventional clinical trials:


id 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:

39
Salivary Gland, Eye, Liver, Lung

Publications for Mikulicz Disease

Articles related to Mikulicz Disease:

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

id Super pathways Score Top Affiliating Genes
1
Show member pathways
10.84 IL21 PHB

GO Terms for Mikulicz Disease

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

id Name GO ID Score Top Affiliating Genes
1 positive regulation of G-protein coupled receptor protein signaling pathway GO:0045745 8.62 PHB PTGDR2

Sources for Mikulicz Disease

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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