Skip to main content

Recurrent Mastoiditis Mimics IgG4 Related Disease: A Potential Diagnostic Pitfall

Head and Neck Pathology volume 10, pages 314–320 (2016)Cite this article

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized entity that causes progressive fibrosis and formation of mass lesions. IgG4-RD can be diagnosed histologically by its hallmarks of storiform fibrosis, prominent lymphoplasmacytic infiltrate, and obliterative phlebitis, accompanied by the infiltration of excessive numbers of IgG4-positive plasma cells as well as elevations in serum IgG4 concentrations. A recent publication reported a case of IgG4-RD in the mastoid sinus, representing a new anatomic location for this disease. We report two additional cases of IgG4-RD occurring in the mastoid and causing clinical mastoiditis. The presenting symptoms were varied—tinnitus, hearing loss, and cranial nerve palsies. All three cases showed a dense lymphoplasmacytic infiltrate, storiform type fibrosis as well as elevated numbers of IgG4 positive plasma cells. The three patients responded to immunosuppressive therapy that included steroids and Rituximab. We further investigated 162 consecutive mastoiditis cases at our institution in order to determine the frequency of IgG4-RD as a previously unrecognized cause of mastoiditis. Within this latter cohort we identified nine cases of mastoiditis that had two of the histologic features of IgG4-RD, specifically storiform fibrosis and a dense lymphoplasmacytic infiltrate. Two of these cases showed >50 IgG4-positive plasma cells per high-power field with IgG4–IgG ratio of >40 %, thus fulfilling histological criteria for IgG4-RD. However, both were due to severe acute or chronic infection. In conclusion, we reaffirm IgG4 related mastoiditis as a distinct but uncommon cause of recurrent mastoiditis. The diagnosis of IgG4-related mastoiditis should be rendered with caution, and only after the exclusion of potential mimickers, particularly infection.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

Fig. 1
Fig. 2

References

  1. Deshpande V. The pathology of IgG4-related disease: critical issues and challenges. Semin Diagn Pathol. 2012;29:191–6.

    Article  PubMed  Google Scholar 

  2. Mahajan VS, Mattoo H, Deshpande V, et al. IgG4-related disease. Annu Rev Pathol. 2014;9:315–47.

    CAS  Article  PubMed  Google Scholar 

  3. Stone JH. IgG4-related disease: nomenclature, clinical features, and treatment. Semin Diagn Pathol. 2012;29:177–90.

    Article  PubMed  Google Scholar 

  4. Stone JH, Khosroshahi A, Deshpande V, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum. 2012;64:3061–7.

    CAS  Article  PubMed  Google Scholar 

  5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51.

    CAS  Article  PubMed  Google Scholar 

  6. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.

    CAS  Article  PubMed  Google Scholar 

  7. Ebbo M, Grados A, Bernit E, et al. Pathologies associated with serum IgG4 elevation. Int J Rheumatol. 2012;2012:1–6.

    Article  Google Scholar 

  8. Strehl JD, Hartmann A, Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64:237–43.

    Article  PubMed  Google Scholar 

  9. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.

    Article  PubMed  Google Scholar 

  10. Bhatti RM, Stelow EB. IgG4-related disease of the head and neck. Adv Anat Pathol. 2013;20:10–6.

    CAS  Article  PubMed  Google Scholar 

  11. Ferry JA, Deshpande V. IgG4-related disease in the head and neck. Semin Diagn Pathol. 2012;29:235–44.

    Article  PubMed  Google Scholar 

  12. Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34:1812–9.

    Article  PubMed  Google Scholar 

  13. Cheuk W, Yuen HK, Chu SY, et al. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32:671–81.

    Article  PubMed  Google Scholar 

  14. Geyer JT, Ferry JA, Harris NL, et al. Chronic sclerosing sialadenitis (Kuttner tumor) is an IgG4-associated disease. Am J Surg Pathol. 2010;34:202–10.

    Article  PubMed  Google Scholar 

  15. Deshpande V, Khosroshahi A, Nielsen GP, et al. Eosinophilic angiocentric fibrosis is a form of IgG4-related systemic disease. Am J Surg Pathol. 2011;35:701–6.

    Article  PubMed  Google Scholar 

  16. Dahlgren M, Khosroshahi A, Nielsen GP, et al. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res. 2010;62:1312–8.

    Article  Google Scholar 

  17. Schiffenbauer AI, Wahl C, Pittaluga S, et al. IgG4-related disease presenting as recurrent mastoiditis. Laryngoscope. 2012;122:681–4.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Chan SK, Cheuk W, Chan KT, et al. IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009;33:1249–52.

    Article  PubMed  Google Scholar 

  19. Lindstrom KM, Cousar JB, Lopes MBS. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010;120:765–76.

    CAS  Article  PubMed  Google Scholar 

  20. Chang SY, Keogh KA, Lewis JE, et al. IgG4-positive plasma cells in granulomatosis with polyangiitis (Wegener’s): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases. Hum Pathol. 2013;44:2432–7.

    CAS  Article  PubMed  Google Scholar 

  21. Moteki H, Yasuo M, Hamano H, et al. IgG4-related chronic rhinosinusitis: a new clinical entity of nasal disease. Acta Otolaryngol. 2011;131:518–26.

    Article  PubMed  Google Scholar 

  22. Siddiquee Z, Zane NA, Smith RN, et al. Dense IgG4 plasma cell infiltrates associated with chronic infectious aortitis: implications for the diagnosis of IgG4-related disease. Cardiovasc Pathol. 2012;21:470–5.

    CAS  Article  PubMed  Google Scholar 

  23. Deshpande V, Gupta R, Sainani N, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011;35:26–35.

    Article  PubMed  Google Scholar 

  24. Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum. 2010;62:1755–62.

    CAS  Article  PubMed  Google Scholar 

  25. Wallace ZS, Mattoo H, Carruthers M, et al. Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis. 2015;74:190–5.

    CAS  Article  PubMed  Google Scholar 

  26. Ganusov VV. Cellular and population plasticity of helper CD4+ T cell responses. Front Physiol. 2013;16(4):206.

    Google Scholar 

  27. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.

    CAS  Article  PubMed  Google Scholar 

Download references

Author information

Affiliations

  1. Department of Pathology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA, 02114, USA

    Vikram Deshpande, Nicolas A. Zane, Stefan Kraft & William C. Faquin

  2. Department of Medicine, Division of Rheumatology, Massachusetts General Hospital, Boston, MA, USA

    John H. Stone

Authors
  1. Vikram Deshpande
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nicolas A. Zane
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Stefan Kraft
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. John H. Stone
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. William C. Faquin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Vikram Deshpande.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Deshpande, V., Zane, N.A., Kraft, S. et al. Recurrent Mastoiditis Mimics IgG4 Related Disease: A Potential Diagnostic Pitfall. Head and Neck Pathol 10, 314–320 (2016). https://doi.org/10.1007/s12105-016-0710-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-016-0710-0

Keywords

  • IgG4 related disease
  • Mastoiditis
  • IgG4