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.
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.
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.
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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
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
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
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
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
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
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
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
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
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
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
Kamisawa T, Okazaki K (2016) Diagnosis and treatment of IgG4-related disease. Curr Top Microbiol Immunol. https://doi.org/10.1007/82_2016_36
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
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
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
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
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.
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Jurkov, M., Olze, H., Klauschen, F. et al. IgG4-related orbitopathy as an important differential diagnosis of advanced silent sinus syndrome. HNO (2020) doi:10.1007/s00106-019-00799-8
- IgG4-related disease
- Immunoglobulin G
- Eosinophilic angiocentric fibrosis
- Chronic sinusitis