Skip to main content

Advertisement

Log in

Location and frequency of lesions in patients with IgG4-related ophthalmic diseases

  • Oculoplastics and Orbit
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Background

It is well-known that the lacrimal gland (LG) may be affected in IgG4-related ophthalmic disease (IgG4ROD). Recently, IgG4-related ophthalmic lesions other than those of the lacrimal gland have been reported. However, no study to date has revealed the details of these lesions. This study was conducted to evaluate the location and frequency of lesions found in conjunction with IgG4ROD using radiological imaging.

Methods

Radiological images and clinical records of 65 patients collected from seven institutions in Japan were reviewed retrospectively. All patients had been pathologically diagnosed with IgG4ROD. Patients of mucosa-associated lymphoid tissue lymphoma associated with IgG4-related lesions were excluded. Orbital magnetic resonance imaging or computed tomography findings were evaluated.

Results

Of the 65 patients, 31 (47.7 %) had lesions involving the LG alone, whereas 34 (52.3 %) had lesions involving the areas other than LG, including eight patients who had lesions without any LG involvement. IgG4-related ophthalmic lesions included LG enlargement in 57 patients (87.7 %), trigeminal nerve branch enlargement in 25 (38.5 %), extraocular muscle enlargement in 16 (24.6 %), diffuse orbital fat lesions in 15 (23.1 %), orbital mass lesions in 11 (16.9 %), eyelid lesions in eight (12.3 %), and nasolacrimal duct lesion in one (1.5 %). Six patients (9.2 %) presented with visual disturbance due to optic nerve disturbance, eight (12.3 %) with a restriction of ocular movement, and 19 (29.2 %) with exophthalmos.

Conclusions

Thirty-four (52.3 %) of the 65 IgG4ROD patients had lesions in areas other than LG. Lesions were found in the trigeminal nerve branch including pterygopalatine fossa, extraocular muscles, orbital fat, eyelid, and nasolacrimal duct.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H, Kiyosawa K (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403–1404

    Article  PubMed  Google Scholar 

  2. Cheuk W, Chan JKC (2010) IgG4-related sclerosing disease. A critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol 17:303–332

    Article  CAS  PubMed  Google Scholar 

  3. Zen Y, Nakanuma Y (2010) IgG4-related disease. A cross-sectional study of 114 cases. Am J Surg Pathol 34:1812–1819

    Article  PubMed  Google Scholar 

  4. Umehara H, Okazaki H, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, Hamano H, Kamisawa T, Shimosegawa T, Shimatsu A, Nakamura S, Ito T, Notohara K, Sumida T, Tanaka Y, Mimori T, Chiba T, Mishima M, Hibi T, Tsubouchi H, Kazuo Inui K, Ohara H (2012) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22:21–30

    Article  CAS  PubMed  Google Scholar 

  5. Yamamoto M, Harada S, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, Imai K (2005) Clinical and pathological differences between Mikulicz’s disease and Sjögren’s syndrome. Rheumatology (Oxford) 44:227–234

    Article  CAS  Google Scholar 

  6. Takahira M, Kawano M, Zen Y, Minato H, Yamada K, Sugiyama K (2007) IgG4-related chronic sclerosing dacryoadenitis. Arch Ophthalmol 125:1575–1578

    Article  PubMed  Google Scholar 

  7. Sato Y, Ohshima K, Ichimura K, Sato M, Yamadori I, Tanaka T, Takata K, Morito T, Kondo E, Yoshino T (2008) Ocular adnexal IgG4-related disease has uniform clinicopathology. Pathol Int 58:465–470

    Article  CAS  PubMed  Google Scholar 

  8. Kubota T, Moritani S, Katayama M, Terasaki H (2010) Ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder. Arch Ophthalmol 128:577–584

    Article  PubMed  Google Scholar 

  9. Takahira M, Ozawa Y, Kawano M, Zen Y, Hamaoka S, Yamada K, Sugiyama K (2012) Clinical aspects of IgG4-related orbital Inflammation in a case series of ocular adnexal lymphoproliferative disorders. Int J Rheumatol. doi:10.1155/2012/635473

    PubMed Central  PubMed  Google Scholar 

  10. Higashiyama T, Nishida Y, Ugi S, Ishida M, Nishio Y, Ohji M (2011) A case of extraocular muscle swelling due to IgG4-related sclerosing disease. Jpn J Ophthalmol 55:315–317

    Article  PubMed  Google Scholar 

  11. Shin YU, Oh YH, Lee YJ (2012) Unusual involvement of IgG4-related sclerosing disease in lacrimal and submandibular glands and extraocular muscles. Korean J Ophthalmol 26:216–221

    Article  PubMed Central  PubMed  Google Scholar 

  12. Wallace ZS, Khosroshahi A, Jakobiec FA, Deshpande V, Hatton MP, Ritter J, Ferry JA, Stone JH (2012) IgG4-related systemic disease as a cause of “idiopathic” orbital inflammation, including orbital myositis, and trigeminal nerve involvement. Surv Ophthalmol 57:26–33

    Article  PubMed  Google Scholar 

  13. Mehta M, Jakobiec F, Fay A (2009) Idiopathic fibroinflammatory disease of the face, eyelids, and periorbital membrane with immunoglobulin G4-positive plasma cells. Arch Pathol Lab Med 133:1251–1255

    PubMed  Google Scholar 

  14. Watanabe T, Fujikawa Y, Kawakami S, Hatta T, Hamano H, Kawa S, Kadoya M (2011) Infraorbital nerve swelling associated with autoimmune pancreatitis. Jpn J Radiol 29:194–201

    Article  PubMed  Google Scholar 

  15. Katsura M, Morita A, Horiuchi H, Ohtomo K, Machida T (2011) IgG4-related inflammatory pseudotumor of the trigeminal nerve: another component of IgG4-related sclerosing disease? AJNR Am J Neuroradiol 32:E150–E152

    Article  CAS  PubMed  Google Scholar 

  16. Ohshima K, Sogabe Y, Sato Y (2012) The usefulness of infraorbital nerve enlargement on MRI in clinical diagnosis of IgG4-related orbital disease. Jpn J Ophthalmol 56:380–382

    Article  PubMed  Google Scholar 

  17. Inoue D, Zen Y, Sato Y, Abo H, Demachi H, Uchiyama A, Gabata T, Matsui O (2012) IgG4-related perineural disease. Int J Rheumatol. doi:10.1155/2012/401890

    PubMed Central  PubMed  Google Scholar 

  18. Katsura M, Mori H, Kunimatsu A, Sasaki H, Abe O, Machida T, Ohtomo K (2012) Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology 54:873–882

    Article  PubMed  Google Scholar 

  19. Sogabe Y, Miyatani K, Goto R, Ishii G, Ohshima K, Sato Y (2012) Pathological findings of infraorbital nerve enlargement in IgG4-related ophthalmic disease. Jpn J Ophthalmol 56:511–514

    Article  PubMed  Google Scholar 

  20. Ohno K, Sato Y, Ohshima K, Takata K, Ando M, Al-Kader LA, Iwaki N, Takeuchi M, Orita Y, Yoshino T (2012) IgG4-related disease involving the sclera. Mod Rheumatol. doi:10.1007/s10165-012-0758-y

    Google Scholar 

  21. Suzuki M, Mizumachi T, Morita S, Kubota K, Iizuka K (2011) A case of immunoglobulin 4-related disease with bilateral mass-forming lesions in the nasolacrimal ducts. J Clin Rheumatol 17:207–210

    Article  PubMed  Google Scholar 

  22. Stone JH, Khosroshahi A, Deshpande V, Chan JKC, Heathcote JG, Aalberse R, Azumi A, Bloch DB, Brugge WR, Carruthers MN, Cheuk W, Cornell L, Castillo CFD, Ferry JA, Forcione D, Klöppel G, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Masaki Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani D, Sato Y, Smyrk T, Stone JR, Takahira M, Umehara H, Webster G, Yamamoto M, Yi E, Yoshino T, Zamboni G, Zen Y, Chari S (2012) Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 64:3061–3067. doi:10.1002/art.34593

    Article  CAS  PubMed  Google Scholar 

  23. Yamada K, Ohta T, Miyamoto T (1992) Bilateral trigeminal schwannomas associated with von Recklinghausen disease. AJNR Am J Neuroradiol 13:299–300

    CAS  PubMed  Google Scholar 

  24. Rootman J, Goldberg C, Robertson W (1982) Primary orbital schwannomas. Br J Ophthalmol 66:194–204

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  25. Rose GE, Wright JE (1991) Isolated peripheral nerve sheath tumours of the orbit. Eye (London) 5:668–673

    Article  Google Scholar 

  26. Barbagallo GMV, Russo A, Mendoza ND (2004) Isolated, benign, intraorbital schwannoma arising from the supraorbital nerve. Case report and review of the literature. J Neurosurg Sci 48:75–80

    CAS  PubMed  Google Scholar 

  27. Guibord N, Chalk C, Wein F, Richardson J, Snipes GJ, Del Carpio R (1998) Trigeminal nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 51:1459–1462

    Article  CAS  PubMed  Google Scholar 

  28. Ojiri H (2006) Perineural spread in head and neck malignancies. Radiat Med 24:1–8

    Article  PubMed  Google Scholar 

  29. McKinney AM, Short J, Lucato L, Santa Cruz K, McKinney Z, Kim Y (2006) Inflammatory myofibroblastic tumor of the orbit with associated enhancement of the meninges and multiple cranial nerves. Am J Neuroradiol 27:2217–2220

    CAS  PubMed  Google Scholar 

  30. Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, Takahashi H, Shinomura Y, Imai K, Saeki T, Azumi A, Nakada S, Sugiyama E, Matsui S, Origuchi T, Nishiyama S, Nishimori I, Nojima T, Yamada K, Kawano M, Zen Y, Kaneko M, Miyazaki K, Tsubota K, Eguchi K, Tomoda K, Sawaki T, Kawanami T, Tanaka M, Fukushima T, Sugai S, Umehara H (2009) Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 68:1310–1315

    Article  CAS  PubMed  Google Scholar 

  31. Tsuzaka K, Matsumoto Y, Sasaki Y, Abe T, Tsubota K, Takeuchi T (2007) Down-regulation of Fas-ligand mRNA in Sjögren’s syndrome patients with enlarged exocrine glands. Autoimmunity 40:497–502

    Article  CAS  PubMed  Google Scholar 

  32. Bartley GB, Fatourechi V, Kadrmas EF, Jacobsen SJ, Ilstrup DM, Garrity JA, Gorman CA (1996) Clinical features of Graves’ ophthalmopathy in an incidence cohort. Am J Ophthalmol 121:284–290

    CAS  PubMed  Google Scholar 

  33. Kubota T, Kano H (2007) Assessment of inflammation in idiopathic orbital myositis with fat-suppressed T2-weighted magnetic resonance imaging. Am J Ophthalmol 143:718–720

    Article  PubMed  Google Scholar 

  34. Cheuk W, Yuen HKL, Chan ACL, Shih L, Kuo T, Ma M, Lo Y, Chan W, Chan JKC (2008) Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 32:1159–1167

    Article  PubMed  Google Scholar 

  35. Oyama T, Takizawa J, Nakamura N, Aoki S, Aizawa Y, Abe H (2011) Multifocal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: a case report. Jpn J Ophthalmol 55:304–306

    Article  PubMed  Google Scholar 

  36. Kubota T, Moritani S, Yoshino T, Nagai H, Terasaki H (2010) Ocular adnexal marginal zone B cell lymphoma infiltrated by IgG4-positive plasma cells. J Clin Pathol 63:1059–1065

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yuka Sogabe.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sogabe, Y., Ohshima, Ki., Azumi, A. et al. Location and frequency of lesions in patients with IgG4-related ophthalmic diseases. Graefes Arch Clin Exp Ophthalmol 252, 531–538 (2014). https://doi.org/10.1007/s00417-013-2548-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-013-2548-4

Keywords

Navigation