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IgG4-related dacryoadenitis and sialadenitis

MedGen UID:
6399
•Concept ID:
C0026103
•
Disease or Syndrome
Synonyms: Mikulicz disease; Mikulicz' Disease; Mikulicz's disease; Non-Sjogren syndrome; Salivary gland hyperplasia
SNOMED CT: Mikulicz's disease (7826003); Mikulicz's syndrome (7826003); Mikulicz disease (7826003); Mikulicz syndrome (7826003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019191
Orphanet: ORPHA79078

Definition

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). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgG4-related dacryoadenitis and sialadenitis
Follow this link to review classifications for IgG4-related dacryoadenitis and sialadenitis in Orphanet.

Professional guidelines

PubMed

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.
Sakamoto M, Moriyama M, Shimizu M, Chinju A, Mochizuki K, Munemura R, Ohyama K, Maehara T, Ogata K, Ohta M, Yamauchi M, Ishiguro N, Matsumura M, Ohyama Y, Kiyoshima T, Nakamura S
Mod Rheumatol 2020 Mar;30(2):379-384. Epub 2019 Mar 4 doi: 10.1080/14397595.2019.1576271. PMID: 30696319
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment.
Yamamoto M, Nojima M, Takahashi H, Yokoyama Y, Ishigami K, Yajima H, Shimizu Y, Tabeya T, Matsui M, Suzuki C, Naishiro Y, Takano K, Himi T, Imai K, Shinomura Y
Rheumatology (Oxford) 2015 Jan;54(1):45-9. Epub 2014 Jun 6 doi: 10.1093/rheumatology/keu228. PMID: 24907151

Recent clinical studies

Etiology

Ultrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness.
Komori T, Inoue D, Izumozaki A, Sugiura T, Terada K, Yoneda N, Toshima F, Yoshida K, Kitao A, Kozaka K, Takahira M, Kawano M, Kobayashi S, Gabata T
Mod Rheumatol 2022 Aug 20;32(5):986-993. doi: 10.1093/mr/roab063. PMID: 34918161
CD163+ M2 Macrophages Promote Fibrosis in IgG4-Related Disease Via Toll-like Receptor 7/Interleukin-1 Receptor-Associated Kinase 4/NF-κB Signaling.
Chinju A, Moriyama M, Kakizoe-Ishiguro N, Chen H, Miyahara Y, Haque ASMR, Furusho K, Sakamoto M, Kai K, Kibe K, Hatakeyama-Furukawa S, Ito-Ohta M, Maehara T, Nakamura S
Arthritis Rheumatol 2022 May;74(5):892-901. Epub 2022 Apr 10 doi: 10.1002/art.42043. PMID: 34907668Free PMC Article
Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis.
Takahashi Y, Mizushima I, Konishi M, Kawahara H, Sanada H, Suzuki K, Takeji A, Hara S, Ito K, Fujii H, Kawano M
Mod Rheumatol 2021 Nov;31(6):1164-1170. Epub 2021 Feb 11 doi: 10.1080/14397595.2021.1878623. PMID: 33491522
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment.
Yamamoto M, Takano K, Kamekura R, Suzuki C, Ichimiya S, Himi T, Nakase H, Takahashi H
Mod Rheumatol 2018 Nov;28(6):1004-1008. Epub 2018 Feb 23 doi: 10.1080/14397595.2018.1436029. PMID: 29385874

Diagnosis

Ultrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness.
Komori T, Inoue D, Izumozaki A, Sugiura T, Terada K, Yoneda N, Toshima F, Yoshida K, Kitao A, Kozaka K, Takahira M, Kawano M, Kobayashi S, Gabata T
Mod Rheumatol 2022 Aug 20;32(5):986-993. doi: 10.1093/mr/roab063. PMID: 34918161
IgG4-related Dacryoadenitis and Sialadenitis with Palatal Gland Swelling.
Yoshida M, Baba A, Hashimoto K, Nakamura Y, Itou Y, Yoshida Y, Sato K, Nomura T
Bull Tokyo Dent Coll 2021 Mar 13;62(1):41-47. Epub 2021 Feb 15 doi: 10.2209/tdcpublication.2020-0002. PMID: 33583876
Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis.
Takahashi Y, Mizushima I, Konishi M, Kawahara H, Sanada H, Suzuki K, Takeji A, Hara S, Ito K, Fujii H, Kawano M
Mod Rheumatol 2021 Nov;31(6):1164-1170. Epub 2021 Feb 11 doi: 10.1080/14397595.2021.1878623. PMID: 33491522
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment.
Yamamoto M, Takano K, Kamekura R, Suzuki C, Ichimiya S, Himi T, Nakase H, Takahashi H
Mod Rheumatol 2018 Nov;28(6):1004-1008. Epub 2018 Feb 23 doi: 10.1080/14397595.2018.1436029. PMID: 29385874

Therapy

Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
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.
Shimizu M, Okamura K, Kise Y, Takeshita Y, Furuhashi H, Weerawanich W, Moriyama M, Ohyama Y, Furukawa S, Nakamura S, Yoshiura K
Arthritis Res Ther 2015 Aug 23;17(1):223. doi: 10.1186/s13075-015-0751-x. PMID: 26298875Free PMC Article
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment.
Yamamoto M, Nojima M, Takahashi H, Yokoyama Y, Ishigami K, Yajima H, Shimizu Y, Tabeya T, Matsui M, Suzuki C, Naishiro Y, Takano K, Himi T, Imai K, Shinomura Y
Rheumatology (Oxford) 2015 Jan;54(1):45-9. Epub 2014 Jun 6 doi: 10.1093/rheumatology/keu228. PMID: 24907151

Prognosis

Extraction of characteristic serum microRNAs and prediction of target genes in IgG4-related dacryoadenitis and sialadenitis.
Yamamoto M, Tanaka T, Aochi S, Uehara M, Kamekura R, Takano KI
Mod Rheumatol 2024 Mar 28;34(3):632-638. doi: 10.1093/mr/road089. PMID: 37747366
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.
Sakamoto M, Moriyama M, Shimizu M, Chinju A, Mochizuki K, Munemura R, Ohyama K, Maehara T, Ogata K, Ohta M, Yamauchi M, Ishiguro N, Matsumura M, Ohyama Y, Kiyoshima T, Nakamura S
Mod Rheumatol 2020 Mar;30(2):379-384. Epub 2019 Mar 4 doi: 10.1080/14397595.2019.1576271. PMID: 30696319
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
Stage classification of IgG4-related dacryoadenitis and sialadenitis by the serum cytokine environment.
Yamamoto M, Takano K, Kamekura R, Suzuki C, Ichimiya S, Himi T, Nakase H, Takahashi H
Mod Rheumatol 2018 Nov;28(6):1004-1008. Epub 2018 Feb 23 doi: 10.1080/14397595.2018.1436029. PMID: 29385874
Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment.
Yamamoto M, Nojima M, Takahashi H, Yokoyama Y, Ishigami K, Yajima H, Shimizu Y, Tabeya T, Matsui M, Suzuki C, Naishiro Y, Takano K, Himi T, Imai K, Shinomura Y
Rheumatology (Oxford) 2015 Jan;54(1):45-9. Epub 2014 Jun 6 doi: 10.1093/rheumatology/keu228. PMID: 24907151

Clinical prediction guides

Extraction of characteristic serum microRNAs and prediction of target genes in IgG4-related dacryoadenitis and sialadenitis.
Yamamoto M, Tanaka T, Aochi S, Uehara M, Kamekura R, Takano KI
Mod Rheumatol 2024 Mar 28;34(3):632-638. doi: 10.1093/mr/road089. PMID: 37747366
Ultrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness.
Komori T, Inoue D, Izumozaki A, Sugiura T, Terada K, Yoneda N, Toshima F, Yoshida K, Kitao A, Kozaka K, Takahira M, Kawano M, Kobayashi S, Gabata T
Mod Rheumatol 2022 Aug 20;32(5):986-993. doi: 10.1093/mr/roab063. PMID: 34918161
IgG4-related Dacryoadenitis and Sialadenitis with Palatal Gland Swelling.
Yoshida M, Baba A, Hashimoto K, Nakamura Y, Itou Y, Yoshida Y, Sato K, Nomura T
Bull Tokyo Dent Coll 2021 Mar 13;62(1):41-47. Epub 2021 Feb 15 doi: 10.2209/tdcpublication.2020-0002. PMID: 33583876
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.
Sakamoto M, Moriyama M, Shimizu M, Chinju A, Mochizuki K, Munemura R, Ohyama K, Maehara T, Ogata K, Ohta M, Yamauchi M, Ishiguro N, Matsumura M, Ohyama Y, Kiyoshima T, Nakamura S
Mod Rheumatol 2020 Mar;30(2):379-384. Epub 2019 Mar 4 doi: 10.1080/14397595.2019.1576271. PMID: 30696319
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685

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