MCID: MKL001
MIFTS: 45

Mikulicz Disease

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

Aliases & Classifications for Mikulicz Disease

MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 12 52 58 15 71
Igg4-Related Dacryoadenitis and Sialadenitis 12 52 58
Chronic Dacryoadenitis and Sialadenitis 52 58
Mikulicz's Disease 12 52
Mikulicz Syndrome 74 52
Mikulicz' Disease 43

Characteristics:

Orphanet epidemiological data:

58
igg4-related dacryoadenitis and sialadenitis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare eye diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:12900
MeSH 43 D008882
NCIt 49 C34819
SNOMED-CT 67 7826003
ICD10 32 K11.8
MESH via Orphanet 44 D008882
ICD10 via Orphanet 33 K11.8
UMLS via Orphanet 72 C0026103
Orphanet 58 ORPHA79078
UMLS 71 C0026103

Summaries for Mikulicz Disease

NIH Rare Diseases : 52 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 cytokine deficiency 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 Innate Immune System and PEDF Induced Signaling. The drugs Methylprednisolone hemisuccinate and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include eye, salivary gland and b cells, and related phenotypes are hematopoietic system and digestive/alimentary

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 : 74 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 top 50) (show all 450)
# Related Disease Score Top Affiliating Genes
1 cytokine deficiency 30.7 IL5 IL13
2 sialadenitis 30.4 ICOSLG CD4 CCR6
3 xerophthalmia 30.3 CRP CD4
4 salivary gland disease 30.1 ICOSLG CD4 CCR6
5 mononeuritis multiplex 30.1 CRP CD4
6 polyclonal hypergammaglobulinemia 30.0 CRP CD4
7 miliary tuberculosis 29.9 CRP CD4
8 keratoconjunctivitis sicca 29.9 IL4 CD4 CCR6
9 purpura 29.9 IL4 IL10 CRP
10 lymphoma, hodgkin, classic 29.8 IL13 ICOSLG CCR6
11 thrombocytopenia due to platelet alloimmunization 29.8 IL10 ICOSLG CD4 CCR6
12 dacryoadenitis 29.7 KNTC1 IL21 IL13 IL10 ICOSLG CD4
13 keratoconjunctivitis 29.6 IL5 IL4 IL13
14 parotitis 29.5 SPINK1 CRP CD4
15 acquired immunodeficiency syndrome 29.5 IL10 CRP CD4 CCR6
16 sarcoidosis 1 29.4 IL13 ICOSLG CRP CD4 CCR6
17 castleman disease 29.4 IL5 IL4 IL10 CRP
18 exanthem 29.2 IL10 ICOSLG CRP CD4 CCR6
19 hypereosinophilic syndrome 29.2 IL5 IL4 IL13 IL10
20 lymphadenitis 29.2 IL10 ICOSLG CRP CD4 CCR6
21 allergic rhinitis 29.2 IL5 IL4 IL13 IL10
22 multicentric castleman disease 29.1 IL5 IL21 IL10 CRP CD4
23 lymphoma, non-hodgkin, familial 29.1 IL4 IL10 ICOSLG CD4 CCR6
24 rhinitis 28.9 IL5 IL4 IL13 IL10 ICOSLG
25 bronchiolitis 28.6 IL5 IL4 IL13 IL10 CRP
26 crohn's disease 28.6 IL4 IL10 ICOSLG CRP CD4 CCR6
27 connective tissue disease 28.6 IL4 IL10 ICOSLG CRP CD4 CCR6
28 mycobacterium tuberculosis 1 28.6 IL4 IL10 ICOSLG CRP CD4 CCR6
29 igg4-related disease 28.2 SLAMF7 PLA2R1 KNTC1 IL4 ICOSLG CRP
30 myeloma, multiple 28.2 SLAMF7 IL4 IL10 ICOSLG CRP CD4
31 rheumatoid arthritis 28.1 IL4 IL13 IL10 CRP CD4 CCR6
32 autoimmune disease 27.7 IL5 IL4 IL21 IL13 IL10 ICOSLG
33 diffuse cutaneous systemic sclerosis 10.4 ICOSLG CCR6
34 pustulosis palmaris et plantaris 10.3 IL5 CCR6
35 selective immunoglobulin deficiency disease 10.3 SLAMF7 ICOSLG
36 suppurative lymphadenitis 10.3 CD4 CCR6
37 spongiotic dermatitis 10.3 CD4 CCR6
38 leukemia 10.3
39 thyroiditis 10.3
40 retroperitoneal fibrosis 10.3
41 anti-basement membrane glomerulonephritis 10.3 CD4 CCR6
42 inflammatory bowel disease 5 10.3 ICOSLG CCR6
43 autoimmune peripheral neuropathy 10.3 ICOSLG CD4
44 chancroid 10.3 CD4 CCR6
45 esophageal candidiasis 10.3 CD4 CCR6
46 pneumonic tularemia 10.3 CD4 CCR6
47 orbital plasma cell granuloma 10.3 KNTC1 CRP
48 lymphocytic hypophysitis 10.3
49 igg4-related thyroid disease 10.3
50 primary hypophysitis 10.3

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



Diseases related to Mikulicz Disease

Symptoms & Phenotypes for Mikulicz Disease

Symptoms:

12
  • lumps of the face

MGI Mouse Phenotypes related to Mikulicz Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 CCR6 CD4 IL10 IL13 IL21 IL4
2 digestive/alimentary MP:0005381 9.73 CD4 IL10 IL13 IL4 IL5 SPINK1
3 homeostasis/metabolism MP:0005376 9.65 CCR6 CD4 CRP IL10 IL13 IL21
4 immune system MP:0005387 9.36 CCR6 CD4 CRP IL10 IL13 IL21

Drugs & Therapeutics for Mikulicz Disease

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

(show all 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
2
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
3
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
4
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
8
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
9 Immunologic Factors Phase 4
10 Methylprednisolone Acetate Phase 4
11 Liver Extracts Phase 4
12 Anti-Inflammatory Agents Phase 4
13 Immunoglobulin G Phase 4
14 Hormone Antagonists Phase 4
15 Immunoglobulins Phase 4
16 Antineoplastic Agents, Hormonal Phase 4
17 glucocorticoids Phase 4
18 Hormones Phase 4
19 Immunosuppressive Agents Phase 4
20 Antibodies Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Study of Safety and Efficacy of Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
2 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

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:

40
Eye, Salivary Gland, B Cells, Bone, Lung, Skin, T Cells

Publications for Mikulicz Disease

Articles related to Mikulicz Disease:

(show top 50) (show all 89)
# Title Authors PMID Year
1
Cutting Edge: A Critical Role of Lesional T Follicular Helper Cells in the Pathogenesis of IgG4-Related Disease. 61 52
28916523 2017
2
Mikulicz Disease. 61
31913425 2020
3
Interleukin 5-producing ST2+ memory Th2 cells in IgG4-related dacryoadenitis and sialadenitis. 61
30354922 2019
4
Potential utility of core needle biopsy in the diagnosis of IgG4-related dacryoadenitis and sialadenitis. 61
29656682 2019
5
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. 61
30696319 2019
6
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. 61
30701685 2019
7
IgG4-related respiratory disease. 61
30474465 2019
8
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment. 61
29385874 2018
9
Early Therapeutic Intervention for IgG4-related Dacryoadenitis and Sialadenitis: The Balance Between Risk of Observation Only and Therapeutic Adverse Effects. 61
30173184 2018
10
Estimation of proton density fat fraction of the salivary gland. 61
29376736 2018
11
Genes and pathways associated with the occurrence of malignancy in benign lymphoepithelial lesions. 61
29207199 2018
12
Mikulicz Disease Mimicking Intraorbital Tumors. 61
28987847 2018
13
[Non-Hodgkin's lymphoma mimicking Mikulicz disease: a case report]. 61
27987516 2016
14
Gallium SPECT/CT in evaluation of IgG4-related disease: A case report and literature review. 61
27631251 2016
15
IgG4-related disease. 61
26259069 2016
16
IgG4-related hypophysitis presenting as diabetes insipidus with tubulo-interstital nephritis and mediastinal lymphadenopathy. 61
27398219 2016
17
[Review of ear and nose and throat involvement in IgG4-RD]. 61
26911076 2015
18
[112th Scientific Meeting of the Japanese Society of Internal Medicine: Symposium: Recent Progress in IgG4-related Disease: IgG4-related Dacryoadenitis and Sialadenitis]. 61
30160920 2015
19
[IgG4-related Dacryoadenitis and Sialadenitis]. 61
30160881 2015
20
Effectiveness of imaging modalities for screening IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) and for differentiating it from Sjögren's syndrome (SS), with an emphasis on sonography. 61
26298875 2015
21
IgG4-related Mikulicz Disease in a 76-year-old Chinese Male: A Case Report. 61
24432837 2015
22
IgG4 related disease of the head and neck. 61
25804380 2015
23
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment. 61
24907151 2015
24
Diagnostic criteria for IgG4-related ophthalmic disease. 61
25392273 2015
25
IgG4-related skin disease. 61
25065694 2014
26
The role of cytotoxic T cells in IgG4-related dacryoadenitis and sialadenitis, the so-called Mikulicz's disease. 61
24593171 2014
27
¹⁸F-FDG PET-CT usefulness in extra-pancreatic involvement in IgG4 related diseases. 61
24630373 2014
28
Mechanisms and assessment of IgG4-related disease: lessons for the rheumatologist. 61
24296677 2014
29
IgG4-related multiorgan disease: report of the first autopsy case. 61
23645656 2013
30
Rare diagnosis of IgG4-related systemic disease by lip biopsy in an international Sjögren syndrome registry. 61
23146570 2013
31
"Quasi-symmetric" FDG avidity of bilateral salivary glands in Mikulicz disease yielding false-positive staging in lung cancer. 61
22996254 2012
32
IgG4-related disease of the head and neck: CT and MR imaging manifestations. 61
23150850 2012
33
IgG4-related disease in the head and neck. 61
23068303 2012
34
Autoimmune pancreatitis that developed over a 3-month observation period for IgG4-related dacryoadenitis and sialadenitis. 61
26182321 2012
35
Utility of FDG PET/CT in IgG4-related systemic disease. 61
22119099 2012
36
Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. 61
21898360 2012
37
[Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease]. 61
22374440 2012
38
Jan Mikulicz-Radecki (1850-1905): his impact on modern medicine. 61
25872240 2012
39
[A case of IgG4-related disease with marked thickening of the bronchial wall]. 61
22352056 2011
40
Cytohistological features of rhinoscleroma. 61
22234117 2011
41
Connective tissue diseases: Immune responses in Mikulicz disease differ from those in SS. 61
21971284 2011
42
Membranous nephropathy associated with IgG4-related disease. 61
21658826 2011
43
IgG4-associated sialadenitis. 61
21124091 2011
44
Clinicopathologic similarities between Mikulicz disease and Küttner tumor. 61
20015799 2010
45
[Clinical analysis of 11 cases of Mikulicz disease in the salivary glands]. 61
21176583 2010
46
[The first experience of using rituximab in Mikulicz disease]. 61
20731115 2010
47
Highly active state of autoimmune pancreatitis with mikulicz disease. 61
19904224 2010
48
[Autoimmune pancreatitis]. 61
19860211 2009
49
Sonographic diagnosis for Mikulicz disease. 61
19451003 2009
50
F-18 FDG PET-CT findings in Mikulicz disease and systemic involvement of IgG4-related lesions. 61
19352281 2009

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:

(show all 26)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.84 SLAMF7 IL5 IL4 IL13 IL10 ICOSLG
2
Show member pathways
13.51 IL5 IL4 IL21 IL13 IL10 CD4
3
Show member pathways
13.19 IL5 IL4 IL21 IL13 IL10 CD4
4
Show member pathways
13.07 IL5 IL4 IL21 IL13 IL10 CD4
5
Show member pathways
12.61 IL5 IL4 IL21 IL13 IL10 ICOSLG
6
Show member pathways
12.49 IL4 IL10 ICOSLG CD4
7
Show member pathways
12.45 IL5 IL4 IL10 CD4
8
Show member pathways
12.38 IL5 IL4 IL21 IL13 IL10 CD4
9
Show member pathways
12.17 IL5 IL4 IL21 IL13 IL10
10
Show member pathways
12.11 IL5 IL4 IL13 IL10
11
Show member pathways
11.91 IL21 IL10 CD4
12 11.84 IL4 IL13 IL10
13 11.8 IL5 IL4 IL10 CD4
14 11.76 IL5 IL4 CD4
15 11.67 IL5 IL4 IL13
16 11.59 IL4 IL13 IL10
17 11.52 IL5 IL4 IL13
18 11.51 IL5 IL4 IL10
19 11.42 IL21 IL10 CD4
20 11.38 IL4 IL13 IL10
21 11.22 IL5 IL4 IL13 IL10 CD4
22 11.16 IL5 IL4 IL13
23 11 IL5 IL4 IL13 IL10 CD4 CCR6
24 10.97 IL5 IL4 IL13 IL10
25 10.86 IL5 IL4 IL21 IL13 IL10
26 10.82 IL5 IL4 IL13 IL10 CD4

GO Terms for Mikulicz Disease

Cellular components related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.5 SPINK1 IL5 IL4 IL21 IL13 IL10
2 external side of plasma membrane GO:0009897 9.46 IL13 ICOSLG CD4 CCR6
3 extracellular region GO:0005576 9.23 SPINK1 PLA2R1 IL5 IL4 IL21 IL13

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.07 IL5 IL4 IL21 IL10 ICOSLG CD4
2 inflammatory response GO:0006954 9.85 IL5 IL13 IL10 CRP
3 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.65 IL4 IL21 IL13
4 positive regulation of T cell proliferation GO:0042102 9.63 IL4 IL21 CD4
5 T cell activation GO:0042110 9.58 IL4 ICOSLG CD4
6 cytokine-mediated signaling pathway GO:0019221 9.55 IL5 IL4 IL13 IL10 CD4
7 positive regulation of macrophage activation GO:0043032 9.54 IL13 IL10
8 positive regulation of MHC class II biosynthetic process GO:0045348 9.51 IL4 IL10
9 negative regulation of tumor necrosis factor biosynthetic process GO:0042536 9.49 IL4 IL10
10 type 2 immune response GO:0042092 9.43 IL4 IL10
11 negative regulation of endothelial cell apoptotic process GO:2000352 9.43 IL4 IL13 IL10
12 regulation of isotype switching GO:0045191 9.37 IL4 IL10
13 negative regulation of complement-dependent cytotoxicity GO:1903660 9.32 IL4 IL13
14 positive regulation of B cell proliferation GO:0030890 9.26 IL5 IL4 IL21 IL13
15 immune response GO:0006955 9.17 IL5 IL4 IL21 IL13 IL10 CD4

Molecular functions related to Mikulicz Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine receptor binding GO:0005126 9.13 IL4 IL21 IL13
2 cytokine activity GO:0005125 9.02 IL5 IL4 IL21 IL13 IL10

Sources for Mikulicz Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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