Skip to main content
SpringerLink
Log in

IgG4-related orbitopathy as an important differential diagnosis of advanced silent sinus syndrome

IgG4-assoziierte Orbitopathie als wichtige Differenzialdiagnose des fortgeschrittenen Silent-Sinus-Syndroms

  • Case reports
  • Published:
HNO Aims and scope Submit manuscript

Abstract

Background

Immunoglobulin (Ig)G4-related disease is classified as an immune-mediated disease. The etiology of this condition has not been explained to date. Manifestations of the disease are diverse, and simultaneous involvement of multiple organs is not unusual.

Case report

We report the case of a patient referred to us after multiple unsuccessful paranasal sinus operations who presented with enophthalmos and a resultant migratory keratitis with a suspected diagnosis of silent sinus syndrome. Preservation of the orbit was no longer feasible. After five years without a definitive diagnosis, we ascertained that this was a case of IgG4-related disease.

Discussion

IgG4-related disease represents an important element in the differential diagnosis of chronic advanced diseases of the orbit and paranasal sinuses. The diagnosis should be considered in the case of unclear disease presentations. Typical histological findings include a storiform pattern of fibrosis, vasculopathy, and tissue infiltration by IgG4 plasma cells.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Stone JH, Khosroshahi A, Deshpande V et al (2012) Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 64:3061–3067. https://doi.org/10.1002/art.34593

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Al-Mujaini A, Al-Khabori M, Shenoy K, Wali U (2018) Immunoglobulin G4-related disease: an update. Oman Med J 33:97–103. https://doi.org/10.5001/omj.2018.20

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Mulholland GB, Jeffery CC, Satija P, Côté DWJ (2015) Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg 44:24. https://doi.org/10.1186/s40463-015-0071-9

    Article  PubMed  PubMed Central  Google Scholar 

  4. Tiegs-Heiden CA, Eckel LJ, Hunt CH et al (2014) Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. Am J Neuroradiol 35:1393–1397. https://doi.org/10.3174/ajnr.A3865

    Article  CAS  PubMed  Google Scholar 

  5. Vandjelovic ND, Humphreys IM (2016) Immunoglobulin G4-related sclerosing disease of the paranasal sinuses: A case report and literature review. Allergy Rhinol 7:85–89. https://doi.org/10.2500/ar.2016.7.0154

    Article  Google Scholar 

  6. Umehara H, Okazaki K, Masaki Y et al (2012) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22:21–30. https://doi.org/10.1007/s10165-011-0571-z

    Article  CAS  PubMed  Google Scholar 

  7. Yamagishi A, Oshitari T, Tawada A et al (2018) The case of IgG4-related ophthalmic disease with perivascular lesions of superior ophthalmic vein associated with optic nerve disturbance. Neuroophthalmology 42:251–255. https://doi.org/10.1080/01658107.2017.1394328

    Article  PubMed  Google Scholar 

  8. Obiorah IE, Henao Velasquez A, Özdemirli M (2018) The clinicopathologic spectrum of IgG4-related disease. Balkan Med J 35:292–300. https://doi.org/10.4274/balkanmedj.2018.0809

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192. https://doi.org/10.1038/modpathol.2012.72

    Article  PubMed  Google Scholar 

  10. Umehara H, Okazaki K, Masaki Y et al (2012) A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 22:1–14. https://doi.org/10.1007/s10165-011-0508-6

    Article  CAS  PubMed  Google Scholar 

  11. Carruthers MN, Khosroshahi A, Augustin T et al (2015) The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 74:14–18. https://doi.org/10.1136/annrheumdis-2013-204907

    Article  CAS  PubMed  Google Scholar 

  12. Kamisawa T, Okazaki K (2016) Diagnosis and treatment of IgG4-related disease. Curr Top Microbiol Immunol. https://doi.org/10.1007/82_2016_36

    Article  Google Scholar 

  13. Khosroshahi A, Wallace ZS, Crowe JL et al (2015) International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol 67:1688–1699. https://doi.org/10.1002/art.39132

    Article  CAS  PubMed  Google Scholar 

  14. Kurien R, Babu TR, Rupa V (2015) Unusual cause of maxillary sinus mass with proptosis. BMJ Case Rep 2015:bcr2015210627. https://doi.org/10.1136/bcr-2015-210627

    Article  PubMed  PubMed Central  Google Scholar 

  15. Gorostis S, Bacha M, Gravier S, Raguin T (2017) Right ethmoid eosinophilic angiocentric fibrosis with orbital extension. Eur Ann Otorhinolaryngol Head Neck Dis 134:351–354. https://doi.org/10.1016/J.ANORL.2017.02.012

    Article  CAS  PubMed  Google Scholar 

  16. Deshpande V, Khosroshahi A, Nielsen GP et al (2011) Eosinophilic angiocentric fibrosis is a form of IgG4-related systemic disease. Am J Surg Pathol 35:701–706. https://doi.org/10.1097/PAS.0b013e318213889e

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Otorhinolaryngology, Charité—Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany

    M. Jurkov,  H. Olze &  P. Arens

  2. Department of Pathology, Charité—Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany

    F. Klauschen

  3. Department of Ophthalmology, Charité—Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany

    E. Bertelmann

  4. Department of Rheumatology, Charité—Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany

    U. Schneider

Authors
  1. M. Jurkov
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. Olze
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. F. Klauschen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. E. Bertelmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. U. Schneider
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. P. Arens
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to M. Jurkov.

Ethics declarations

Conflict of interest

M. Jurkov, H. Olze, F. Klauschen, E. Bertelmann, U. Schneider and P. Arens declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case. Additional written informed consent was obtained from all individual participants or their legal representatives for whom identifying information is included in this article.

The supplement containing this article is not sponsored by industry.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Jurkov, M., Olze, H., Klauschen, F. et al. IgG4-related orbitopathy as an important differential diagnosis of advanced silent sinus syndrome. HNO 68 (Suppl 1), 65–68 (2020). https://doi.org/10.1007/s00106-019-00799-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00106-019-00799-8

Keywords

Search

Navigation