Abstract
IgG4 related disease (IgG4-RD) is a systemic fibro-inflammatory disease leading to tumour-like infiltrations potentially affecting all parts of the human body, including the periorbital tissues (IgG4-ROD). IgG4-ROD mimics many infectious, inflammatory and malignant disorders. Therefore, the diagnostic gold standard is histopathology, however, serum IgG4 and the clinical presentation may be supportive in establishing the diagnosis. Glucocorticoids are the preferred first-line systemic treatment of IgG4-ROD. In severe refractory or vision threatening disease, rituximab combined with glucocorticoids is started. When refractory, cyclophosphamide can be initiated. In less severe cases, mycophenolate mofetil or infliximab can be considered as maintenance or steroid-sparing therapy. Radiotherapy or surgery may be indicated in localized refractory disease in patients with compressive optic neuropathy or proptosis.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Brito-Zeron P, Bosch X, Ramos-Casals M, Stone JH. IgG4-related disease: advances in the diagnosis and treatment. Best Pract Res Clin Rheumatol. 2016;30:261–78.
Heeringa JJ, Karim AF, van Laar JAM, Verdijk RM, Paridaens D, van Hagen PM, et al. Expansion of blood IgG4(+) B, TH2, and regulatory T cells in patients with IgG4-related disease. J Allergy Clin Immunol. 2018;141:1831–43.e10.
Andrew N, Kearney D, Selva D. IgG4-related orbital disease: a meta-analysis and review. Acta Ophthalmol. 2013;91:694–700.
Wallace ZS, Deshpande V, Stone JH. Ophthalmic manifestations of IgG4-related disease: single-center experience and literature review. Semin Arthritis Rheum. 2014;43:806–17.
Ebbo M, Patient M, Grados A, Groh M, Desblaches J, Hachulla E, et al. Ophthalmic manifestations in IgG4-related disease: clinical presentation and response to treatment in a French case-series. Medicine (Baltimore). 2017;96:e6205.
Karim AF, Verdijk RM, Guenoun J, van Hagen PM, van Laar JA. An inflammatory condition with different faces: immunoglobulin G4-related disease. Neth J Med. 2016;74:110–5.
Verhoekx JSN, Karim AF, van Laar JAM, Verdijk RM, Paridaens D. The tarsal plate manifestation of IgG4-related disease. Int Ophthalmol. 2019;39:1613–5.
Savino G, Midena G, Tartaglione T, Milonia L, Caputo CG, Grimaldi G. Clinical-radiological patterns and histopathological outcomes in non-thyroid extraocular muscle enlargement: retrospective case series and current concepts. Ophthal Plast Reconstr Surg. 2020;36:284–91.
Karim F, de Hoog J, Paridaens D, Verdijk R, Schreurs M, Rothova A, et al. IgG4-related disease as an emerging cause of scleritis. Acta Ophthalmol. 2017;95:e795–e6.
Detiger SE, Karim F, Monserez D, Verdijk R, van Hagen M, Paridaens D, et al. IgG4-related disease of skull base: case series of 3 patients with headache. World Neurosurg. 2020;134:536–9.
Savino MG, Campana MA, Petrone MG, Grimaldi MG, Murchison AP. In search of a disease. Surv Ophthalmol. 2017;62:716–21.
Karim F, Loeffen J, Bramer W, Westenberg L, Verdijk R, van Hagen M, et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J. 2016;14:18.
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.
Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol. 2015;67:1688–99.
Carruthers MN, Khosroshahi A, Augustin T, Deshpande V, Stone JH. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis. 2015;74:14–8.
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.
Su Y, Sun W, Wang C, Wu X, Miao Y, **ong H, et al. Detection of serum IgG4 levels in patients with IgG4-related disease and other disorders. PLoS One. 2015;10:e0124233.
Tiegs-Heiden CA, Eckel LJ, Hunt CH, Diehn FE, Schwartz KM, Kallmes DF, Salomão DR, Witzig TE, Garrity JA. Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 2014;35:1393–7.
Sato Y, Ohshima K, Ichimura K, Sato M, Yamadori I, Tanaka T, et al. Ocular adnexal IgG4-related disease has uniform clinicopathology. Pathol Int. 2008;58:465–70.
Deschamps R, Deschamps L, Depaz R, Coffin-Pichonnet S, Belange G, Jacomet PV, et al. High prevalence of IgG4-related lymphoplasmacytic infiltrative disorder in 25 patients with orbital inflammation: a retrospective case series. Br J Ophthalmol. 2013;97:999–1004.
Van Tilburg A, Breijer A, Paridaens AD, Vann den Bosch W, Simonsz HJ, Bleyen I, et al. Frequency of IgG4-positive plasma cells in idiopathic orbital inflammation (ESP Abstracts 2014). Virchovs Arch. 2014;465:S34.
Karim AF, Verdijk RM, Nagtegaal AP, Bansie R, Paridaens D, van Hagen PM, et al. To distinguish IgG4-related disease from seronegative granulomatosis with polyangiitis. Rheumatology (Oxford). 2017;56:2245–7.
Deshpande V, Khosroshahi A, Nielsen GP, Hamilos DL, Stone JH. Eosinophilic angiocentric fibrosis is a form of IgG4-related systemic disease. Am J Surg Pathol. 2011;35:701–6.
Karim F, Clahsen-van Groningen M, van Laar JA. AA Amyloidosis and IgG4-related disease. N Engl J Med. 2017;376:599–600.
Heidari P, Verdijk RM, van den Bosch WA, Paridaens D. Biopsy-proven recurrence of unilateral IgG4-related orbital inflammation after 20 years. Orbit. 2014;33:388–91.
Della-Torre E, Stone JH. “How I manage” IgG4-related disease. J Clin Immunol. 2016;36:754–63.
Detiger SE, Karim AF, Verdijk RM, van Hagen PM, van Laar JAM, Paridaens D. The treatment outcomes in IgG4-related orbital disease: a systematic review of the literature. Acta Ophthalmol. 2019;97:451–9.
Karim F, Paridaens D, Westenberg LE, Guenoun J, Verdijk RM, van Hagen PM, et al. Infliximab for IgG4-related orbital disease. Ophthal Plast Reconstr Surg. 2017;33:S162–5.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2024 The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Paridaens, D., Verdijk, R.M., Savino, G. (2024). IgG4-Related Orbital Disease. In: Quaranta Leoni, F.M., Verity, D.H., Paridaens, D. (eds) Oculoplastic, Lacrimal and Orbital Surgery. Springer, Cham. https://doi.org/10.1007/978-3-031-39638-0_18
Download citation
DOI: https://doi.org/10.1007/978-3-031-39638-0_18
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-39637-3
Online ISBN: 978-3-031-39638-0
eBook Packages: MedicineMedicine (R0)