Long-term outcomes of ocular adnexal lesions in IgG4-related ophthalmic disease.
AIMS
To determine the long-term outcomes of ocular adnexal lesions in immunoglobulin G4-related ophthalmic disease (IgG4-ROD).


METHODS
This retrospective, non-randomised exploratory study included 82 patients with ocular adnexal lesions. We evaluated the long-term outcomes in 71 patients during the median follow-up period of 30 months, who underwent either watchful waiting (n=20; range 12-90 months) or systemic corticosteroid treatment, delivered according to consensus guidelines (n=51; range 9- 115 months). We also analysed factors that might contribute to recalcitrance to treatment.


RESULTS
Of 82 patients, 40 (49%) were male, and the median patient age was 60 years old. Twenty-one (26%) patients with extraocular muscle (EOM) and/or trigeminal nerve branch (CN V) enlargements had a significantly high frequency of multiple ocular adnexal lesions (p<0.0001, Fisher's exact test). In addition, two patients developed EOM and/or CN V enlargements de novo over time. Twenty patients with solitary lacrimal gland enlargements preferred watchful waiting, due to mild symptoms. Of these, 18 (90%) lesions remained dormant throughout a median follow-up of 27 months. Among 51 patients treated with corticosteroids, 31 (61%) experienced relapses after treatment and required systemic low-dose maintenance treatment. A multivariate analysis indicated that EOM and/or CN V enlargements comprised a risk factor for relapse (HR 2.7; 95% CI 1.1 to 6.7).


CONCLUSIONS
This exploratory study showed that different types of ocular adnexal lesions in IgG4-ROD displayed distinct proliferative activities. Our results suggested that EOM and/or CN V enlargements might be secondary lesions that confer refractoriness to systemic corticosteroid treatment recommended by consensus guidelines.
['Kubota|Toshinobu|T|0000-0003-0653-0267', 'Katayama|Masao|M|', 'Nishimura|Reiko|R|', 'Moritani|Suzuko|S|']
D000328:Adult D000368:Aged D000369:Aged, 80 and over D005260:Female D005500:Follow-Up Studies D005938:Glucocorticoids D006801:Humans D000077733:Immunoglobulin G4-Related Disease D007766:Lacrimal Apparatus Diseases D008297:Male D008875:Middle Aged D016727:Orbital Pseudotumor D011379:Prognosis D012189:Retrospective Studies D014057:Tomography, X-Ray Computed D057832:Watchful Waiting
lacrimal gland orbit