MCID: MKL001
MIFTS: 48

Mikulicz Disease

Categories: Bone diseases, Endocrine diseases, Eye diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Mikulicz Disease

MalaCards integrated aliases for Mikulicz Disease:

Name: Mikulicz Disease 11 19 58 14 71 33
Igg4-Related Dacryoadenitis and Sialadenitis 11 19 58
Chronic Dacryoadenitis and Sialadenitis 19 58
Mikulicz's Disease 11 19
Mikulicz Syndrome 19 75
Mikulicz' Disease 43 75

Characteristics:


Age Of Onset:

Igg4-Related Dacryoadenitis and Sialadenitis: All ages 58

Classifications:

Orphanet: 58  
Rare eye diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 11 DOID:12900
MeSH 43 D008882
NCIt 49 C34819
SNOMED-CT 68 7826003
ICD10 31 K11.8
MESH via Orphanet 44 D008882
ICD10 via Orphanet 32 K11.8
UMLS via Orphanet 72 C0026103
Orphanet 58 ORPHA79078
ICD11 33 2012865457
UMLS 71 C0026103

Summaries for Mikulicz Disease

GARD: 19 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.

MalaCards based summary: Mikulicz Disease, also known as igg4-related dacryoadenitis and sialadenitis, is related to thyroiditis and chronic rhinitis, and has symptoms including lumps of the face An important gene associated with Mikulicz Disease is CCR6 (C-C Motif Chemokine Receptor 6), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. Affiliated tissues include eye, liver and bone, and related phenotypes are enlarged lacrimal glands and abnormality of the submandibular glands

Orphanet: 58 IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease (see this term) characterized by persistent, usually painless, bilateral enlargement of the lacrimal, parotid, and submandibular glands associated with elevated levels of serum immunoglobulin (Ig) G4 and with lymphocyte and IgG4-positive plasmacyte infiltration. It predominantly causes mouth and eye dryness but can also affect other organs such as the lungs, liver, and kidneys, and be accompanied by complications such as autoimmune pancreatitis (AIP), retroperitoneal fibrosis, and tubulointerstitial nephritis (see these terms).

Disease Ontology: 11 An autoimmune 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: 75 Benign lymphoepithelial lesion or Mikulicz' disease is a type of benign enlargement of the parotid... 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 657)
# Related Disease Score Top Affiliating Genes
1 thyroiditis 30.4 IL4 IL10 CRP
2 chronic rhinitis 30.1 IL5 IL4 IL13
3 bell's palsy 30.0 CRP CD4
4 interstitial nephritis 29.9 ICOSLG CRP CD4 CCR6
5 cholangitis 29.9 IL10 CRP CA2
6 erythema nodosum 29.8 IL13 IL10 CRP
7 keratoconjunctivitis 29.8 TRIM21 IL5 IL4 IL13
8 hypereosinophilic syndrome 29.8 IL5 IL4 IL13 IL10
9 allergic rhinitis 29.8 IL5 IL4 IL13 IL10
10 aplastic anemia 29.7 IL4 IL10 CD4 CCR6
11 juvenile rheumatoid arthritis 29.7 ICOSLG CRP CD4 CCR6
12 sarcoidosis 1 29.7 IL13 IL10 ICOSLG CRP CD4 CCR6
13 lymphadenitis 29.7 IL10 ICOSLG CRP CCR6
14 acquired immunodeficiency syndrome 29.7 IL10 CRP CD4
15 multicentric castleman disease 29.6 IL10 CRP CD4 CCR6
16 castleman disease 29.6 IL10 CRP CD4 CCR6
17 miliary tuberculosis 29.6 IL4 IL10 CRP CD4
18 rhinitis 29.6 IL5 IL4 IL13 IL10 ICOSLG
19 lymphoma, hodgkin, classic 29.5 TNFSF13B IL13 ICOSLG CCR6
20 vasculitis 29.5 TNFSF13B IL10 CRP
21 purpura 29.5 TNFSF13B IL4 IL10 CRP
22 mantle cell lymphoma 29.5 TNFSF13B ICOSLG CD4 CCR6
23 lymphoma, mucosa-associated lymphoid type 29.5 TNFSF13B ICOSLG CD4 CCR6
24 pancreatitis 29.4 SPINK1 IL10 CRP CA2
25 sclerosing cholangitis 29.4 IL10 ICOSLG CRP CD4 CCR6
26 bronchiolitis 29.4 IL5 IL4 IL13 IL10 CRP
27 mononeuropathy 29.3 TRIM21 SSB CRP CD4
28 dacryoadenitis 29.2 TRIM21 TNFSF13B SSB IL4 IL13 IL10
29 cholangitis, primary sclerosing 29.2 IL4 IL10 ICOSLG CRP CD4 CCR6
30 thrombocytopenia due to platelet alloimmunization 29.2 TNFSF13B IL10 ICOSLG CD4 CCR6
31 leukemia, chronic lymphocytic 29.2 TNFSF13B IL4 IL10 ICOSLG CD4 CCR6
32 parotitis 29.1 TRIM21 SSB SPINK1 CRP CD4
33 sjogren syndrome 29.1 TRIM21 TNFSF13B SSB IL10 ICOSLG CCR6
34 mononeuritis multiplex 29.1 TRIM21 SSB IL5 CRP CD4
35 thrombocytopenia 28.9 TRIM21 TNFSF13B IL4 IL10 CRP CD4
36 exanthem 28.9 TRIM21 IL4 IL10 ICOSLG CRP CD4
37 autoimmune pancreatitis 28.8 SPINK1 IL10 ICOSLG CRP CD4 CCR6
38 polyclonal hypergammaglobulinemia 28.8 TRIM21 TNFSF13B ICOSLG CRP CD4 CCR6
39 common cold 28.7 IL5 IL4 IL13 IL10 ICOSLG CRP
40 sialadenitis 28.7 TRIM21 TNFSF13B SSB IL13 ICOSLG CD4
41 xerophthalmia 28.7 TRIM21 TNFSF13B SSB ICOSLG CRP CD4
42 keratoconjunctivitis sicca 28.5 TRIM21 TNFSF13B SSB IL4 ICOSLG CD4
43 autoimmune disease 28.4 TRIM21 TNFSF13B IL5 IL4 IL13 IL10
44 immune deficiency disease 28.3 TNFSF13B IL4 IL13 IL10 ICOSLG CRP
45 igg4-related disease 28.3 TNFSF13B IL5 IL4 IL13 IL10 ICOSLG
46 asthma 28.1 TNFSF13B IL5 IL4 IL13 IL10 ICOSLG
47 salivary gland disease 27.8 TRIM21 TNFSF13B SSB IL4 IL10 ICOSLG
48 connective tissue disease 27.8 TRIM21 TNFSF13B IL5 IL4 IL13 IL10
49 enterobiasis 10.3 IL5 IL4
50 aztreonam allergy 10.3 IL5 IL13

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



Diseases related to Mikulicz Disease

Symptoms & Phenotypes for Mikulicz Disease

Human phenotypes related to Mikulicz Disease:

58 30 (show all 33)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 enlarged lacrimal glands 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0007734
2 abnormality of the submandibular glands 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010287
3 increased circulating igg4 level 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0032300
4 enlargement of parotid gland 58 30 Frequent (33%) Frequent (79-30%)
HP:0011801
5 fatigue 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012378
6 fever 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001945
7 palpebral edema 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100540
8 proptosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000520
9 weight loss 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001824
10 keratoconjunctivitis sicca 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001097
11 generalized muscle weakness 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003324
12 myositis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100614
13 xerostomia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000217
14 increased circulating iga level 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003261
15 increased circulating igm level 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003496
16 periorbital fullness 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000629
17 antinuclear antibody positivity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003493
18 abnormality of the extraocular muscles 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0008049
19 cytoplasmic antineutrophil antibody positivity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0032230
20 blindness 58 30 Very rare (1%) Very rare (<4-1%)
HP:0000618
21 thyroiditis 58 30 Very rare (1%) Very rare (<4-1%)
HP:0100646
22 thrombocytopenia 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001873
23 abnormal lacrimal duct morphology 58 30 Very rare (1%) Very rare (<4-1%)
HP:0011481
24 lymphadenopathy 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002716
25 tubulointerstitial nephritis 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001970
26 optic nerve compression 58 30 Very rare (1%) Very rare (<4-1%)
HP:0007807
27 retroperitoneal fibrosis 58 30 Very rare (1%) Very rare (<4-1%)
HP:0005200
28 nodular goiter 58 30 Very rare (1%) Very rare (<4-1%)
HP:0005994
29 autoimmunity 58 Occasional (29-5%)
30 abnormal salivary gland morphology 58 Very frequent (99-80%)
31 abnormality of the optic nerve 58 Occasional (29-5%)
32 facial edema 58 Occasional (29-5%)
33 abnormality of the orbital region 58 Occasional (29-5%)

Symptoms:

11
  • lumps of the face

MGI Mouse Phenotypes related to Mikulicz Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10 CA2 CCR6 CD4 CRP ICOSLG IL10
2 immune system MP:0005387 9.77 CA2 CCR6 CD4 CRP ICOSLG IL10
3 digestive/alimentary MP:0005381 9.7 CA2 CD4 IL10 IL13 IL4 IL5
4 hematopoietic system MP:0005397 9.36 CCR6 CD4 ICOSLG IL10 IL13 IL4

Drugs & Therapeutics for Mikulicz Disease

Search Clinical Trials, NIH Clinical Center for Mikulicz Disease

Cochrane evidence based reviews: mikulicz' disease

Genetic Tests for Mikulicz Disease

Anatomical Context for Mikulicz Disease

Organs/tissues related to Mikulicz Disease:

MalaCards : Eye, Liver, Bone, Salivary Gland, T Cells, Bone Marrow, Lung

Publications for Mikulicz Disease

Articles related to Mikulicz Disease:

(show top 50) (show all 347)
# Title Authors PMID Year
1
Thoracic manifestations of IgG4-related disease. 62
36437514 2022
2
Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study. 62
36288914 2022
3
Ultrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness. 62
34918161 2022
4
Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients. 62
35927746 2022
5
CD163+ M2 Macrophages Promote Fibrosis in IgG4-Related Disease Via Toll-like Receptor 7/Interleukin-1 Receptor-Associated Kinase 4/NF-κB Signaling. 62
34907668 2022
6
[A case of Mikulicz disease of submandibular gland]. 62
35527446 2022
7
Cases with IgG4-related ophthalmic disease with mass lesions surrounding the optic nerve. 62
35146198 2022
8
Three-dimensional ultrasonographic imaging of Mikulicz's disease. 62
34559333 2022
9
IgG4-Related Dacryoadenitis in Egyptian Patients: A Retrospective Study. 62
36039106 2022
10
T Cell Roles and Activity in Chronic Sclerosing Sialadenitis as IgG4-Related Disease: Current Concepts in Immunopathogenesis. 62
35769404 2022
11
Routine IgG4 staining in minor salivary gland biopsy in a cohort of Italian Caucasian patients suffering from xerostomia. 62
35645421 2022
12
Significance of high serum IgG4 in complete or non-full-fledged IgG4-related disease-a retrospective investigation of 845 patients and its clinical relevance. 62
34455508 2022
13
[A Case of IgG4-Related Disease Arising in Female Periurethra]. 62
34856791 2021
14
Marginal Zone Lymphoma and Lung Adenocarcinoma with an EGFR Exon 19 E746-S752del Mutation in a Patient with IgG4-related Disease. 62
33775999 2021
15
Differences in clinical characteristics of IgG4-related disease across age groups: a prospective study of 737 patients. 62
33211878 2021
16
The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. 62
33274670 2021
17
IgG4-related Dacryoadenitis and Sialadenitis with Palatal Gland Swelling. 62
33583876 2021
18
Increased Expression of Activation-Induced Cytidine Deaminase in Sinus Mucosa from IgG4-Related Disease Patients with Comorbid Chronic Rhinosinusitis. 62
34077941 2021
19
Phenotyping of IgG4-related diseases based on affected organ pattern: A multicenter cohort study using cluster analysis. 62
31902317 2021
20
Immunoglobulin G4 (IgG4)-Related Sialadenitis and Dacryoadenitis With Chronic Rhinosinusitis. 62
32821631 2020
21
Clinical features of immunoglobulin G4-related disease with central nervous system involvement: an analysis of 15 cases. 62
32083541 2020
22
Pediatric Parotid Chronic Sclerosing Sialadenitis in an African-American Female: A Rare Case and Review of the Literature. 62
32754389 2020
23
IgG4-related disease with pseudotumor formation in the larynx. 62
31230814 2020
24
Serum immunoglobulin G4 in Sjögren's syndrome: a pilot study. 62
32062697 2020
25
Mikulicz Disease. 62
31913425 2020
26
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. 62
30696319 2020
27
Genetic analysis of IgG4-related disease. 62
31104539 2020
28
A case report of Mikulicz's disease. 62
33598067 2020
29
Mikulicz's disease and Küttner's tumor as manifestations of IgG4-related diseases: a review of the literature. 62
32921832 2020
30
IgG4-related small-sized occlusive vasculitis in Mikulicz's disease. 62
31334401 2019
31
Interleukin 5-producing ST2+ memory Th2 cells in IgG4-related dacryoadenitis and sialadenitis. 62
30354922 2019
32
Mikulicz's Disease With Immune Thrombocytopenia: A New Immunoglobulin G4-Mediated Disorder and Literature Review. 62
31598602 2019
33
Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. 62
30561747 2019
34
A case of immunoglobulin G4-related inflammatory pseudotumor mimicking renal cell carcinoma. 62
30607433 2019
35
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. 62
30701685 2019
36
Immunoglobulin G4-related Disease: An Overview. 62
33681509 2019
37
Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. 62
30612117 2019
38
IgG4-related respiratory disease. 62
30474465 2019
39
Potential utility of core needle biopsy in the diagnosis of IgG4-related dacryoadenitis and sialadenitis. 62
29656682 2019
40
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment. 62
29385874 2018
41
Early Therapeutic Intervention for IgG4-related Dacryoadenitis and Sialadenitis: The Balance Between Risk of Observation Only and Therapeutic Adverse Effects. 62
30173184 2018
42
Estimation of proton density fat fraction of the salivary gland. 62
29376736 2018
43
Genes and pathways associated with the occurrence of malignancy in benign lymphoepithelial lesions. 62
29207199 2018
44
A case of recurrent Mikulicz's disease with mononeuritis multiplex. 62
30799998 2018
45
Mikulicz Disease Mimicking Intraorbital Tumors. 62
28987847 2018
46
Immunoglobulin G4-related ophthalmic disease. 62
29675343 2018
47
IgG4-Related Disease, the Malignancy Mimicker: Case Series from Bahrain. 62
30510832 2018
48
[IgG4-related disease: a case report]. 62
29190875 2017
49
Cutting Edge: A Critical Role of Lesional T Follicular Helper Cells in the Pathogenesis of IgG4-Related Disease. 62
28916523 2017
50
A Case Report of Mikulicz Syndrome. 62
29299096 2017

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 27)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.73 TRIM21 TNFSF13B IL5 IL4 IL13 IL10
2
Show member pathways
13.64 TNFSF13B IL5 IL4 IL13 IL10 CD4
3
Show member pathways
13.46 TNFSF13B IL5 IL4 IL13 IL10 CD4
4
Show member pathways
13.25 TNFSF13B IL5 IL4 IL13 IL10 CD4
5
Show member pathways
13.15 CD4 IL10 IL13 IL4 IL5 TNFSF13B
6
Show member pathways
13.12 TNFSF13B IL5 IL4 IL13 IL10 CD4
7
Show member pathways
12.03 IL5 IL4 IL10 CD4
8 11.96 IL4 IL13 IL10
9 11.86 IL5 IL4 IL10 CD4
10 11.82 IL4 IL13 IL10
11 11.73 IL5 IL4 IL13 IL10
12 11.66 IL5 IL4 IL13
13 11.61 IL10 IL4 IL5
14 11.59 CRP ICOSLG IL10 IL4
15 11.55 IL5 IL4 IL13
16 11.52 IL5 IL4 IL10
17
Show member pathways
11.44 IL5 IL4 IL13 IL10
18 11.4 IL4 IL10 CD4
19 11.22 IL5 IL4 IL13
20 11.22 IL5 IL4 IL13 IL10 CD4
21 11.05 IL5 IL4 IL13 IL10
22 11 IL5 IL4 IL13 IL10 CD4 CCR6
23 10.97 IL5 IL4 IL13 IL10
24 10.88 TNFSF13B IL5 IL4 IL13 IL10
25 10.82 IL5 IL4 IL13 IL10 CD4
26 10.79 IL4 CD4
27 10.66 IL5 IL4 IL13

GO Terms for Mikulicz Disease

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.32 TNFSF13B IL5 IL4 IL13 IL10 ICOSLG
2 T cell activation GO:0042110 9.91 IL4 ICOSLG CD4
3 positive regulation of mast cell degranulation GO:0043306 9.8 IL4 IL13
4 negative regulation of endothelial cell apoptotic process GO:2000352 9.8 IL10 IL13 IL4
5 type 2 immune response GO:0042092 9.76 IL4 IL10
6 regulation of isotype switching GO:0045191 9.71 IL4 IL10
7 B cell costimulation GO:0031296 9.67 TNFSF13B IL4
8 positive regulation of immunoglobulin production GO:0002639 9.63 IL5 IL13 IL10
9 negative regulation of complement-dependent cytotoxicity GO:1903660 9.56 IL4 IL13
10 positive regulation of B cell proliferation GO:0030890 9.56 TNFSF13B IL5 IL4 IL13
11 immune response GO:0006955 9.4 TNFSF13B IL5 IL4 IL13 IL10 CD4

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.32 TNFSF13B IL5 IL4 IL13 IL10

Sources for Mikulicz Disease

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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