Location and frequency of lesions in patients with IgG4-related ophthalmic diseases
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.
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.
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.
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.
KeywordsIgG4-related disease MRI Orbital tumor Orbital inflammation Trigeminal nerve Extraocular muscles
Conflict of interest
The authors declare that they have no conflict of interest.
- 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 22:21–30PubMedCrossRefGoogle 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 PubMedCrossRefGoogle 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 68:1310–1315PubMedCrossRefGoogle Scholar