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MCID: MKL001
MIFTS: 40

Mikulicz Disease

Categories: Bone diseases, Endocrine diseases, Eye diseases, Immune 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 53 59 15 72
Igg4-Related Dacryoadenitis and Sialadenitis 12 53 59
Chronic Dacryoadenitis and Sialadenitis 53 59
Mikulicz's Disease 12 53
Mikulicz Syndrome 75 53
Mikulicz' Disease 44

Characteristics:

Orphanet epidemiological data:

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

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Eye diseases Bone diseases Immune diseases Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 33 34
Orphanet: 59  
Rare eye diseases
Rare systemic and rhumatological diseases


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Disease Ontology 12 DOID:12900
MeSH 44 D008882
NCIt 50 C34819
SNOMED-CT 68 7826003
ICD10 33 K11.8
MESH via Orphanet 45 D008882
ICD10 via Orphanet 34 K11.8
UMLS via Orphanet 73 C0026103
Orphanet 59 ORPHA79078
UMLS 72 C0026103
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Summaries for Mikulicz Disease

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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 multicentric castleman disease, 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 Allograft rejection and Th17 cell differentiation. The drugs Prednisone and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include eye, salivary gland and bone, 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 : 75 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 top 50) (show all 89)
# Related Disease Score Top Affiliating Genes
1 dacryoadenitis 31.1 IL21 AQP5
2 multicentric castleman disease 30.0 IL5 IL21
3 leukemia 10.3
4 thyroiditis 10.3
5 retroperitoneal fibrosis 10.3
6 sarcoidosis 1 10.3
7 lymphocytic hypophysitis 10.3
8 igg4-related thyroid disease 10.3
9 primary hypophysitis 10.3
10 cytokine deficiency 10.2
11 sjogren syndrome 10.2
12 igg4-related disease 10.2
13 xerophthalmia 10.2
14 sialadenitis 10.1
15 lymphosarcoma 10.1
16 parotitis 10.1
17 interstitial nephritis 10.1
18 purpura 10.1
19 b-cell lymphoma 10.1
20 nut allergy 10.0 PTGDR2 IL5
21 folliculitis 10.0 PTGDR2 IL5
22 autoimmune disease 9.9
23 rheumatoid arthritis 9.9
24 reticulum cell sarcoma 9.9
25 human immunodeficiency virus type 1 9.9
26 autoimmune pancreatitis 9.9
27 lymphoma 9.9
28 nodal marginal zone lymphoma 9.9
29 castleman disease 9.9
30 mumps 9.9
31 pancytopenia 9.9
32 keratoconjunctivitis sicca 9.9
33 polyclonal hypergammaglobulinemia 9.9
34 arthropathy 9.9
35 pancreatitis 9.9
36 acquired immunodeficiency syndrome 9.9
37 connective tissue disease 9.9
38 vasculitis 9.9
39 lupus erythematosus 9.9
40 keratoconjunctivitis 9.9
41 splenomegaly 9.9
42 warthin tumor 9.9
43 exanthem 9.9
44 gastric lymphoma 9.9
45 mastitis 9.9
46 uveitis 9.9
47 crohn's disease 9.9
48 macroglobulinemia 9.9
49 amyloidosis 9.9
50 lymphoma, mucosa-associated lymphoid type 9.9

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.1 AQP5 CD4 IL21 IL5 PHB PTGDR2
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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
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Anti-Inflammatory Agents Phase 4
9 Immunoglobulin G Phase 4
10 glucocorticoids Phase 4
11 Hormones Phase 4
12 Prednisolone acetate Phase 4
13 Methylprednisolone Acetate Phase 4
14 Antibodies Phase 4
15 Antineoplastic Agents, Hormonal Phase 4
16 Immunosuppressive Agents Phase 4
17 Liver Extracts Phase 4
18 Hormone Antagonists Phase 4
19 Immunoglobulins Phase 4
20 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
21 Immunologic Factors 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

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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:

41
Eye, Salivary Gland, Bone, Liver, Lung, Skin, Bone Marrow
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Publications for Mikulicz Disease

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Articles related to Mikulicz Disease:

(show top 50) (show all 88)
# Title Authors PMID Year
1
Cutting Edge: A Critical Role of Lesional T Follicular Helper Cells in the Pathogenesis of IgG4-Related Disease. 38 6
28916523 2017
2
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. 38
30701685 2019
3
IgG4-related respiratory disease. 38
30474465 2019
4
Potential utility of core needle biopsy in the diagnosis of IgG4-related dacryoadenitis and sialadenitis. 38
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. 38
30696319 2019
6
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment. 38
29385874 2018
7
Interleukin 5-producing ST2+ memory Th2 cells in IgG4-related dacryoadenitis and sialadenitis. 38
30354922 2018
8
Early Therapeutic Intervention for IgG4-related Dacryoadenitis and Sialadenitis: The Balance Between Risk of Observation Only and Therapeutic Adverse Effects. 38
30173184 2018
9
Estimation of proton density fat fraction of the salivary gland. 38
29376736 2018
10
Genes and pathways associated with the occurrence of malignancy in benign lymphoepithelial lesions. 38
29207199 2018
11
Mikulicz Disease Mimicking Intraorbital Tumors. 38
28987847 2018
12
[Non-Hodgkin's lymphoma mimicking Mikulicz disease: a case report]. 38
27987516 2016
13
Gallium SPECT/CT in evaluation of IgG4-related disease: A case report and literature review. 38
27631251 2016
14
IgG4-related disease. 38
26259069 2016
15
IgG4-related hypophysitis presenting as diabetes insipidus with tubulo-interstital nephritis and mediastinal lymphadenopathy. 38
27398219 2016
16
[Review of ear and nose and throat involvement in IgG4-RD]. 38
26911076 2015
17
[112th Scientific Meeting of the Japanese Society of Internal Medicine: Symposium: Recent Progress in IgG4-related Disease: IgG4-related Dacryoadenitis and Sialadenitis]. 38
30160920 2015
18
[IgG4-related Dacryoadenitis and Sialadenitis]. 38
30160881 2015
19
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. 38
26298875 2015
20
IgG4-related Mikulicz Disease in a 76-year-old Chinese Male: A Case Report. 38
24432837 2015
21
IgG4 related disease of the head and neck. 38
25804380 2015
22
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment. 38
24907151 2015
23
Diagnostic criteria for IgG4-related ophthalmic disease. 38
25392273 2015
24
IgG4-related skin disease. 38
25065694 2014
25
The role of cytotoxic T cells in IgG4-related dacryoadenitis and sialadenitis, the so-called Mikulicz's disease. 38
24593171 2014
26
¹⁸F-FDG PET-CT usefulness in extra-pancreatic involvement in IgG4 related diseases. 38
24630373 2014
27
Mechanisms and assessment of IgG4-related disease: lessons for the rheumatologist. 38
24296677 2014
28
IgG4-related multiorgan disease: report of the first autopsy case. 38
23645656 2013
29
Rare diagnosis of IgG4-related systemic disease by lip biopsy in an international Sjögren syndrome registry. 38
23146570 2013
30
IgG4-related disease in the head and neck. 38
23068303 2012
31
"Quasi-symmetric" FDG avidity of bilateral salivary glands in Mikulicz disease yielding false-positive staging in lung cancer. 38
22996254 2012
32
IgG4-related disease of the head and neck: CT and MR imaging manifestations. 38
23150850 2012
33
Autoimmune pancreatitis that developed over a 3-month observation period for IgG4-related dacryoadenitis and sialadenitis. 38
26182321 2012
34
Utility of FDG PET/CT in IgG4-related systemic disease. 38
22119099 2012
35
Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. 38
21898360 2012
36
[Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease]. 38
22374440 2012
37
Jan Mikulicz-Radecki (1850-1905): his impact on modern medicine. 38
25872240 2012
38
[A case of IgG4-related disease with marked thickening of the bronchial wall]. 38
22352056 2011
39
Cytohistological features of rhinoscleroma. 38
22234117 2011
40
Connective tissue diseases: Immune responses in Mikulicz disease differ from those in SS. 38
21971284 2011
41
Membranous nephropathy associated with IgG4-related disease. 38
21658826 2011
42
IgG4-associated sialadenitis. 38
21124091 2011
43
Clinicopathologic similarities between Mikulicz disease and Küttner tumor. 38
20015799 2010
44
[Clinical analysis of 11 cases of Mikulicz disease in the salivary glands]. 38
21176583 2010
45
[The first experience of using rituximab in Mikulicz disease]. 38
20731115 2010
46
Highly active state of autoimmune pancreatitis with mikulicz disease. 38
19904224 2010
47
[Autoimmune pancreatitis]. 38
19860211 2009
48
Sonographic diagnosis for Mikulicz disease. 38
19451003 2009
49
F-18 FDG PET-CT findings in Mikulicz disease and systemic involvement of IgG4-related lesions. 38
19352281 2009
50
Diffuse periairway thickening in patients with Mikulicz disease. 38
18728553 2008
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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
Allograft rejection 37 1
Show member pathways
 12.35 IL5 IL21 CD4
2
Th17 cell differentiation 37
Show member pathways
 11.97 IL5 IL21 CD4
3
Th1 Differentiation Pathway 65
Show member pathways
 11.57 IL21 CD4
4
Hematopoietic cell lineage 37
11.39 IL5 CD4
5
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.2 PTGDR2 IL5 CD4
6
B Cell Development Pathways 65
11.09 IL21 CD4
7
Th2 Differentiation Pathway 65
10.98 IL5 CD4
8
Innate Lymphoid Cell Differentiation Pathways 65
10.7 PTGDR2 IL5 CD4
9
Cytokines and Inflammatory Response 1
10.62 IL5 CD4
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GO Terms for Mikulicz Disease

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

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 8.92 PTGDR2 PHB CD4 AQP5

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

# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 9.71 PTGDR2 PHB IL21 CD4
2 positive regulation of transcription, DNA-templated GO:0045893 9.54 PHB IL5 CD4
3 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.32 IL5 CD4
4 positive regulation of T cell proliferation GO:0042102 9.26 IL21 CD4
5 positive regulation of B cell proliferation GO:0030890 9.16 IL5 IL21
6 positive regulation of G protein-coupled receptor signaling pathway GO:0045745 8.96 PTGDR2 PHB
7 immune response GO:0006955 8.92 PTGDR2 IL5 IL21 CD4
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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
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 TGDB
71 Tocris
72 UMLS
73 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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