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REVIEW PAPER
Mikulicz’s disease and Sjögren’s syndrome as the main autoimmune disorders involving salivary glands
 
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Otolaryngology Ward, Maria Sklodowska-Curie District Hospital, Skarżysko-Kamienna, Poland Head of the Ward: Bartłomiej Kamiński MD, PhD
 
 
Submission date: 2020-02-18
 
 
Final revision date: 2020-07-03
 
 
Acceptance date: 2020-08-21
 
 
Publication date: 2020-09-30
 
 
Medical Studies 2020;36(3):211-218
 
KEYWORDS
TOPICS
ABSTRACT
Mikulicz’s disease and Sjögren’s syndrome involve salivary glands. They are characterised by lymphocytic infiltrations in affected exocrine glands leading to their swelling and dysfunction. Although they present similar clinical symptoms, currently they belong to different classifications and require different multidisciplinary treatments. This study presents and compares Sjögren’s syndrome and Mikulicz’s disease and reviews their main clinical, histopathological, and serologic criteria and the methods used in the diagnostic process. The most reliable diagnostic method is histopathologic examination of glandular tissue obtained by either minor or major salivary gland biopsies. Serologic criteria play an additional diagnostic role. The different radiological modalities allow the exclusion of other inflammatory and neoplastic processes that can mimic the initial stages of Mikulicz’s disease and Sjögren’s syndrome.
REFERENCES (39)
1.
Himi T, Takano K, Yamamoto M, Naishiro Y, Takahashi H. A novel concept of Mikulicz’s disease as IgG4-related disease. Auris Nasus Larynx 2012; 39: 9-17.
 
2.
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, Hamano H, Kamisawa T, Shimosegawa T, Shimatsu A, Nakamura S, Ito T, Notohara K, Sumida T, Tanaka Y, Mimori T, Chiba T, Mishima M, Hibi T, Tsubouchi H, Inui K, Ohara H. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol 2012; 22: 21-30.
 
3.
Mulholland GB, Jeffery CC, Satija P, Côté DW. Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg 2015; 44: 24.
 
4.
Della-Torre E, Lanzillotta M, Doglioni C. Immunology of IgG4-related disease. Clin Exp Immunol 2015; 181: 191-206.
 
5.
Gallo A, Martellucci S, Fusconi M, Pagliuca G, Greco A, De Virgilio A, De Vincentiis M. Sialendoscopic management of autoimmune sialadenitis: a review of literature. Acta Otorhinolaryngol Ital 2017; 37: 148-154.
 
6.
Rolla D, Bellino D, Peloso G, Pia Rastaldi M, Simonini P, Louis Ravetti J. The first case of IgG4-related disease in Italy. J Nephropathol 2013; 2: 144-149.
 
7.
Sebastian A, Dionizy P, Wiland P. IgG4-related disease and the spectrum of mimics in rheumatology. Chronic autoimmune epithelitis. Sjögren’s syndrome and other autoimmune diseases of the exocrine glands. In: Chronic Autoimmune Epithelitis. Sjögren’s Syndrome and Other Autoimmune Disease of the Exocrine Glands. Maslinska M (ed.). IntechOpen, London 2019; 73-85.
 
8.
Wu Y, Xu ZR, Zhou WJ, Yang YM. Immunoglobulin G4-related disease with features of Mikulicz’s disease and autoimmune pancreatitis which firstly presented as asymptomatic lymphadenopathy: a case report. Chin Med J 2015; 128: 706-707.
 
9.
Ochoa ML, López BG, Cabello RR, Feregrino RR. IgG4-related multiorgan disease: report of the first autopsy case. BMJ Case Rep 2013; 2013: bcr2013009636.
 
10.
Khosroshani A, Carruthers MN, Stone JH, Shinagare S, Sainani N, Hasserjian RP, Deshpande V. Rethinking Ormond’s disease: “idiopathic” retroperitoneal fibrosis in the era of IgG4-related disease. Medicine 2013; 92: 82-91.
 
11.
Maslińska M. Introductory Chapter: Autoimmune Epithelitis-Discussion about Sjögren’s Syndrome and Primary Biliary Cholangitis. In: Chronic Autoimmune Epithelitis. Sjögren’s Syndrome and Other Autoimmune Disease of the Exocrine Glands. Maslinska M (ed.). IntechOpen, London 2019; 3-12.
 
12.
Błochowiak KJ, Trzybulska D, Olewicz-Gawlik A, Sikora JJ, Nowak-Gabryel M, Kocięcki J, Witmanowski H, Sokalski J.
 
13.
Levels of EGF and VEGF in patients with primary and secondary Sjögren’s syndrome. Adv Clin Exp Med 2018; 27: 455-461.
 
14.
Błochowiak K, Olewicz-Gawlik A, Polańska A, Nowak-Gabrel M, Kocięcki J, Witmanowski H, Sokalski J. Oral mucosal manifestations in primary and secondary Sjögren’s syndrome and dry mouth syndrome. Adv Dermatol Allergol 2016; 33: 23-27.
 
15.
Błochowiak KJ, Olewicz-Gawlik A, Trzybulska D, Nowak-Gabryel M, Kocięcki J, Witmanowski H, Sokalski J. Serum ICAM-1, VCAM-1 and E-selectin levels in patients with primary and secondary Sjögren’s syndrome. Adv Clin Exp Med 2017; 26: 835-842.
 
16.
Olewicz-Gawlik A, Polańska A, Trzybulska D, Nowak-Gabryel M, Błochowiak K, Kocięcki J, Sokalski J, Żaba R, Adamski Z, Dańczak-Pazdrowska A. Acta Dermatovenerol Croat 2018; 26: 153-156.
 
17.
Xu WL, Ling YC, Wang ZK, Deng F. Diagnostic performance of serum IgG4 level for IgG4-related disease: a meta-analysis. Sci Rep 2016; 6: 32035.
 
18.
Yun J, Wienholt L, Adelstein S. Poor positive predictive value of serum immunoglobulin G4 concentrations in the diagnosis of immunoglobulin G4-related sclerosing disease. Asia Pac Allergy 2014; 4: 172-176.
 
19.
Li S, Deng C, Chen J, Li F. Mikulicz’s disease with immune thrombocytopenia: a new immunoglobulin G4-mediated disorder and literature review. Arch Rheumatol 2018; 34: 343-347.
 
20.
Kim J, Sun D, Ozl R, Beck TG, Birnbaum J, Akpek EK, Baer A.A validated method of labial minor salivary gland biopsy for the diagnosis of Sjögren’s syndrome. Laryngoscope 2016; 126: 2041-2046.
 
21.
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X. International Sjögren’s syndrome criteria working. Classification criteria for primary Sjögren’s syndrome. A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 2017; 76: 9-16.
 
22.
Shiboski CH, Shiboski SC, Seror R. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for primary Sjögren’s syndrome. Arthritis Rheumatol 2017; 69: 35-45.
 
23.
Franceschini F, Cavazzana I, Andreoli L, Tincani A. The 2016 classification criteria for primary Sjogren’s syndrome: what’s new? BMC Medicine 2017; 15: 69.
 
24.
Tarpley TM Jr, Anderson LG, White CL. Minor salivary gland involvement in Sjögren’s syndrome. Oral Surg Oral Med Oral Pathol 1974; 37: 64-74.
 
25.
Chisholm DM, Mason DK. Labial salivary gland biopsy in Sjögren’s disease. J Clin Pathol 1968; 21: 656-660.
 
26.
Kroese F, Haacke E, Bombardieri M. The role of salivary gland histopathology in primary Sjögren’s syndrome: promises and pitfalls. Clin Exp Rheumatol 2018; 112: 222-233.
 
27.
Błochowiak K, Sokalski J. Labial salivary gland biopsy in the diagnosis of Sjögren’s syndrome. Eur J Clin Exp Med 2019; 17: 162-168.
 
28.
Shimizu M, Okamura K, Kise Y, Takeshita Y, Furuhashi H, Weerawanich W, Moriyama M, Ohyama Y, Furukawa S, Nakamura S, Yoshiura K. 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. Arthritis Res Ther 2015; 17: 223.
 
29.
Rao D, Natter P, Fernandes R, Wang ZB, Sandhu SJS. A case report of Mikulicz syndrome. J Radiol Case Rep 2017; 11: 1-7.
 
30.
Giovelli RA, Santos MCS, Serrano E, Valim V. Clinical characteristics and biopsy accuracy in suspected cases of Sjögren’s syndrome referred to labial salivary gland biopsy. BMC Musculoskel Disord 2015; 16: 30.
 
31.
Doe K, Nozawa K, Okada T, Tada K, Yamaji K, Tamura N, Takasaki Y. Usefulness of minor salivary gland biopsy in the diagnosis of IgG4-related disease: a case report. Int J Clin Exp Pathol 2014; 7: 2673-2677.
 
32.
Delli K, Vissink A, Spijkervet FKL. Salivary gland biopsy for Sjögren’s syndrome. Oral Maxillofac Surg Clin North Am 2014; 26: 23-33.
 
33.
Sarioğlu S, KüÇük Ŭ, Cetin P, Sari I, Bĭrlĭk AM. Minor salivary gland evaluation: Sjögren’s syndrome. Turk J Med Sci 2016; 46: 63-65.
 
34.
Varela-Centelles P, Sanchez-Sanchez M, Seoane J. Lip biopsy for the diagnosis of Sjögren’s syndrome: beware of the punch. Int J Oral Maxillofac Surg 2014; 43: 127-130.
 
35.
Yokogawa N, Lieberman SM, Alawi F, Bout-Tabaku S, Guttenberg M, Sherry DD, Vivino FB. Comparison of labial minor salivary gland biopsies from childhood Sjögren syndrome and age-matched controls. J Rheumatol 2014; 41: 1178-1182.
 
36.
Pijpe J, Kalk WW, van der Wal JE, Vissink A, Kluin PM, Roodenburg JL, Bootsma H, Kallenberg CG, Spijkervet FK. Parotid gland biopsy compared with labial biopsy in the diagnosis of patients with primary Sjögren’s syndrome. Rheumatology 2007; 46: 335-341.
 
37.
Moriyama M, Furukawa S, Kawano S, Goto Y, Kiyoshima T, Tanaka A, Maehara T, Hayashida JN, Ohta M, Nakamura S.The diagnostic utility of biopsies from the submandibular and labial salivary glands in IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz’s disease. Int J Oral Maxillofac Surg 2014; 43: 1276-1281.
 
38.
Masaki Y, Kurose N, Umehara H. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century. J Clin Exp Hematop 2011; 51: 13-20.
 
39.
Kamiński B, Guzera Z. The unknown face of labial salivary glands. Labial salivary gland biopsy as a diagnostic tool. Medical Studies 2019; 35: 172-179.
 
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