Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-24T03:53:21.112Z Has data issue: false hasContentIssue false

Immunoglobulin G4 related chronic sclerosing sialadenitis

Published online by Cambridge University Press:  23 March 2015

E L Culver
Affiliation:
Translational Gastroenterology Unit, John Radcliffe Hospital and Oxford University, Oxford, UK
A Hunt
Affiliation:
Department of Otorhinolaryngology – Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
E Crewe
Affiliation:
Department of Otorhinolaryngology – Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
K A Shah
Affiliation:
Department of Cellular Pathology, John Radcliffe Hospital, Oxford, UK
P Martinez-Devesa*
Affiliation:
Department of Otorhinolaryngology – Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK
*
Address for correspondence: Mr Pablo Martinez-Devesa, Department of Otorhinolaryngology – Head and Neck Surgery, John Radcliffe Hospital, Headington, Oxford OX3 9DU, UK Fax: 01865 231 091, E-mail: [email protected]

Abstract

Background:

ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis.

Method:

A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms ‘IgG4’, ‘hyperIgG4 syndrome’ and ‘IgG4 related chronic sclerosing sialadenitis’.

Results:

Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management.

Conclusion:

ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.

Type
Review Articles
Copyright
Copyright © JLO (1984) Limited 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Hamano, H, Kawa, S, Horiuchi, A, Unno, H, Furuya, N, Akamatsu, T et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–8CrossRefGoogle ScholarPubMed
2Kamisawa, T, Funata, N, Hayashi, Y, Eishi, Y, Koike, M, Tsuruta, K et al. A new clinicopathological entity of IgG4 related autoimmune disease. J Gastroenterol 2003;38:982–4Google Scholar
3Stone, JH, Zen, Y, Deshpande, V. IgG4-related disease. N Engl J Med 2012;366:539–51Google Scholar
4Geyer, JT, Ferry, JA, Harris, NL, Stone, JH, Zukerberg, LR, Lauwers, GY et al. Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease. Am J Surg Pathol 2010;34:202–10Google Scholar
5Takano, KL, Yamamoto, M, Takahashi, H, Hinomura, Y, Imai, K, Himi, T. Clinicopathologic similarities between Mikulicz disease and Küttner tumor. Am J Otolaryngol 2010;31:429–34Google Scholar
6Geyer, JT, Deshpande, V. IgG4-associated sialadenitis. Curr Opin Rheumatol 2011;23:95101Google Scholar
7Paul, R, Shekkar, K, Singh, M. Kuttner tumour: an unusual cause of enlargement of a minor salivary gland in the lip. Br J Oral Maxillofac Surg 2010;48:152–3CrossRefGoogle ScholarPubMed
8Yamamoto, M, Takahashi, H, Ohara, M, Suzuki, C, Naishiro, Y, Yamamoto, H et al. A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006;16:335–40CrossRefGoogle ScholarPubMed
9Blanco, M, Mesko, T, Cura, M, Cabello-Inchausti, B. Chronic sclerosing sialadenitis (Kuttner's tumor): unusual presentation with bilateral involvement of major and minor salivary glands. Ann Diagn Pathol 2003;7:2530CrossRefGoogle ScholarPubMed
10Chan, JK. Kuttner tumour (chronic sclerosing sialadenitis) of the submandibular gland: an underrecognized entity. Adv Anat Pathol 1998;5:239–51Google Scholar
11Hamano, H, Arakura, N, Muraki, T, Ozaki, Y, Kiyosawa, K, Kawa, S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol 2006;41:11971205CrossRefGoogle ScholarPubMed
12Ghazale, A, Chari, ST, Smyrk, TC, Levy, MJ, Topazian, MD, Takahashi, N et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007;102:1646–53CrossRefGoogle ScholarPubMed
13De Cocker, JL, D'Arco, F, De Beule, T, Tousseyn, T, Blockmans, D, Hermans, R. IgG4-related systemic disease affecting the parotid and submandibular glands: magnetic resonance imaging features of IgG4-related chronic sclerosing sialadenitis and concomitant lymphadenitis. Clin Imaging 2014;38:195–8Google Scholar
14Smyrk, TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol 2011;23:74–9Google Scholar
15Culver, EL, Bateman, AC. General principles of IgG4-related disease. Diag Histopathol 2013;19:111–18CrossRefGoogle Scholar
16Deshpande, V, Zen, Y, Chan, J, Yi, EE, Sato, Y, Yoshino, T et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181–92Google Scholar
17Strehl, J, Hartmann, A, Agaimy, A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol 2011;64:237–43CrossRefGoogle ScholarPubMed
18Vitali, C, Bombardieri, S, Jonsson, R, Moutsopoulos, HM, Alexander, EL, Carsons, SE et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554–8Google Scholar
19Ambrosetti, A, Zanotti, R, Pattaro, C, Lenzi, L, Chilosi, M, Caramaschi, P et al. Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection. Br J Haematol 2004;126:43–9Google Scholar
20Ghazale, A, Chari, S. Is autoimmune pancreatitis a risk factor for pancreatic cancer? Pancreas 2007;35:376Google Scholar
21Kim, T, Grobmyer, SR, Dixon, LR, Allan, RW, Hochwald, SN. Autoimmune pancreatitis and concurrent small lymphocytic lymphoma: not just a coincidence? J Gastrointest Surg 2008;12:1566–70CrossRefGoogle ScholarPubMed
22Cheuk, W, Yuen, HK, Chan, AC, Shih, LY, Kuo, TT, Ma, MW et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 2008;32:1159–67Google Scholar
23Chari, ST, Smyrk, TC, Levy, MJ, Topazian, MD, Takahashi, N, Zhang, L et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006;4:1010–16CrossRefGoogle ScholarPubMed
24Otsuki, M, Chung, JB, Okazaki, K, Kim, MH, Kamisawa, T, Kawa, S et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. J Gastroenterol 2008;43:403–8Google Scholar
25Umehara, H, Okazaki, K, Masaki, Y, Kawano, M, Yamamoto, M, Saeki, T et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:2130CrossRefGoogle ScholarPubMed
26Kamisawa, T, Shimosegawa, T, Okazaki, K, Nishino, T, Watanabe, H, Kanno, A et al. Standard steroid treatment for autoimmune pancreatitis. Gut 2009;58:1504–7Google Scholar
27Hart, PA, Kamisawa, T, Brugge, WR, Chung, JB, Culver, EL, Czajo, L et al. Treatment and long-term sequelae of autoimmune pancreatitis: a multicenter, international analysis. Gut 2013;62:1771–6Google Scholar
28Khosroshahi, A, Carruthers, MN, Deshpande, V, Unizony, S, Bloch, DB, Stone, JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore) 2012;91:5766Google Scholar