Abstract
Several specific inflammatory diseases may affect the orbit either in isolation or as part of a systemic disease process. Many of these will present with tumefactive lesions that may mimic neoplasia. The classic features of inflammation such as redness, swelling, pain, and loss of function may be absent, which may also lead to the impression of neoplasia. The conditions covered in this chapter include the granulomatous diseases sarcoidosis and the xanthogranulomatous processes, Sjögren’s syndrome, the vasculitic diseases granulomatosis with polyangiitis (GPA or Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and the recently recognized entity of IgG4-related disease. Sjögren’s syndrome and IgG4-related disease also carry an increased risk of the development of lymphoma, as occurs in many other chronic inflammatory diseases. The disease processes in this chapter have specific clinicopathological features, and their systemic manifestations may have significant impacts on the well-being of the patient, and establishing a diagnosis is of importance. A tissue biopsy is nearly always needed to establish a diagnosis, but additional serological tests may also be helpful.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Prabhakaran VC, Saeed P, Esmaeli B, et al. Orbital and adnexal sarcoidosis. Arch Ophthalmol. 2007;125:1657–62.
Mavrikakis I, Rootman J. Diverse clinical presentations of orbital sarcoid. Am J Ophthalmol. 2007;144:760–75.
Demirci H, Christianson MD. Orbital and adnexal involvement in sarcoidosis: analysis of clinical features and systemic disease in 30 cases. Am J Ophthalmol. 2011;151:1074–80.
Sivak-Calcott JA, Rootman J, Rasmussen SL, et al. Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review. Br J Ophthalmol. 2006;90:602–8.
Satchi K, McNab AA, Godfrey T, et al. Adult orbital xanthogranuloma successfully treated with rituximab. Ophthalmology. 2014;121:1664–5.
Sagiv O, Thakar SD, Morell G, et al. Rituximab monotherapy is effective in treating orbital necrobiotic xanthogranuloma. Ophthal Plast Reconstr Surg. 2018;34:e24–7.
McKelvie P, McNab AA, Hardy TG, et al. Comparative study of clinical, pathological, radiological, and genetic features of patients with adult ocular adnexal xanthogranulomatous disease, Erdheim-Chester disease, and IgG4-related disease of the orbit/ocular adnexa. Ophthal Plast Reconstr Surg. 2017;33:112–9.
Rischmueller M, Tieu J, Lester S. Primary Sjögren’s syndrome. Best Pract Res Clin Rheumatol. 2016;30:189–220.
Parkin B, Chew JB, White VA, et al. Lymphocytic infiltration and enlargement of the lacrimal glands. A new subtype of primary Sjögren’s syndrome? Ophthalmology. 2005;112:2040–7.
Liang Y, Yang Z, Qin B, Zhong R. Primary Sjögren’s syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis. 2014;73:1151–6.
Tonami H, Matoba M, Yokata H, et al. Mucosa-associated lymphoid tissue lymphoma in Sjögren’s syndrome. Am J Roentgenol. 2002;179:485–9.
Cassidy DT, McKelvie P, Harris GJ, et al. Lacrimal gland orbital lobe cysts associated with MALT lymphoma and primary Sjögren’s syndrome. Orbit. 2005;24:257–63.
Muller K, Lin JH. Orbital granulomatosis with polyangiitis (Wegener granulomatosis). Arch Pathol Lab Med. 2014;138:1110–4.
Harman LE, Margo CE. Wegener’s granulomatosis. Surv Ophthal. 1998;42:458–80.
Woo TL, Francis IC, Wilcsek GA. Australasian orbital and adnexal Wegener’s granulomatosis. Ophthalmology. 2001;108:1535–43.
Tan LT, Davagnanam I, Isa H, et al. Clinical and imaging features of orbital granulomatosis with polyangiitis and the risk of systemic involvement. Ophthalmology. 2014;121:1304–9.
Tan LT, Davagnanam I, Isa H, et al. Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis. Ophthalmology. 2015;122:2125–9.
Lee BJ, Nelson CC, Lewis CD, et al. External dacryocystorhinostomy in patients with Wegener granulomatosis. Ophthal Plast Reconstr Surg. 2012;28:389–92.
Rosenbaum JT, Choi D, Wilson DJ, et al. Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling. Exp Mol Pathol. 2015;99:271–8.
Pakrou N, Selva D, Leibovitch I. Wegener’s granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum. 2006;35:284–92.
Taylor SR, Salama AD, Joshi L, et al. Rituximab is effective in the treatment of refractory ophthalmic Wegener’s granulomatosis. Arthritis Rheum. 2009;60:1540–7.
McNab AA. Orbital inflammation in Churg-Strauss syndrome. Orbit. 1998;17:203–5.
Heine A, Beck R, Stropahi G, et al. Inflammatory pseudotumour of the anterior orbit. A symptom in allergic granulomatous angiitis (Churg-Strauss syndrome). Ophthalmologe. 1995;92:870–3.
Pradeep TG, Prabhakaran VC, McNab A, et al. Diffuse orbital inflammation in Churg-Strauss syndrome. Ophthal Plast Reconstr Surg. 2010;26:57–9.
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.
Deshpande V, Zen Y, Chan JKC, et al. Consensus statement on the pathology of IgG4-related disease. Hum Pathol. 2012;25:1181–92.
Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.
Goto H, Takahira M, Azumi A, Japanese Study Group for IgG4-Related Ophthalmic Disease. Diagnostic criteria for IgG4-related ophthalmic disease. Jpn J Ophthalmol. 2015;59:1–7.
Chang SY, Keogh KA, Lewis JE, et al. IgG4-positive plasma cells in granulomatosis with polyangiitis (Wegener’s): a clinicopathological and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases. Hum Pathol. 2013;44:2432–7.
McNab AA, McKelvie P. IgG4-related ophthalmic disease. Part I: background and pathology. Ophthal Plast Reconstr Surg. 2015;31:83–8.
McNab AA, McKelvie P. IgG4-related ophthalmic disease. Part II: clinical aspects. Ophthal Plast Reconstr Surg. 2015;31:167–78.
Aziz HA, Villa-Forte A, Plesec TP, et al. Isolated conjunctival inflammation suggestive of IgG4-related disease. Ocul Oncol Pathol. 2015;2:51–3.
Watanabe T, Fujinaga Y, Kawakami S, et al. Infraorbital nerve swelling associated with autoimmune pancreatitis. Jpn J Radiol. 2011;29:194–201.
Hardy TG, McNab AA, Rose GE. Enlargement of the infraorbital nerve: an important sign associated with orbital reactive lymphoid hyperplasia or IgG4-related inflammation. Ophthalmology. 2014;121:1297–303.
Cheuk W, Yuen HKL, Chan ACL, 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;32:1159–67.
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.
Ebbo M, Grados A, Guedj E, et al. Usefulness of 2-[18F]-flouro-2-deoxy-D-glucose-positron emission tomography/computed tomography for staging and evaluation of treatment response in IgG4-related disease: a retrospective multicenter study. Arthritis Care Res (Hoboken). 2014;66:86–96.
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.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
McNab, A.A. (2019). Specific Orbital Inflammatory Diseases. In: Hwang, C.J., Patel, B.C., Singh, A.D. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-13558-4_6
Download citation
DOI: https://doi.org/10.1007/978-3-030-13558-4_6
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-13557-7
Online ISBN: 978-3-030-13558-4
eBook Packages: MedicineMedicine (R0)