Ocular adnexal lesions found in some inflammatory eye diseases—including extraocular muscle and trigeminal nerve branch enlargements—show distinct proliferative activity, and patients with these findings are often resistant to traditional corticosteroid treatment, an exploratory study in the British Journal of Ophthalmology reports.

Ocular adnexal tissues, such as lacrimal glands, extraocular muscles, trigeminal nerve branches and orbital fat, are affected by systemic immunoglobulin G4-related conditions, and systemic corticosteroids are often used as the first-line treatment. However, several previous studies reported risk factors related to relapse after treatment. Recently, Japanese researchers sought to determine the long-term outcomes of the lesions in this patient population.

The investigators evaluated 71 patients with an approximate follow-up of 30 months. The study divided the subjects into two groups: watchful waiting (20 subjects) and systemic corticosteroid treatment (51 patients).

The researchers reported 21 patients (26%) with extraocular muscle and/or trigeminal nerve branch enlargements had a significantly high frequency of multiple ocular adnexal lesions. In addition, two patients developed new extraocular muscle and/or trigeminal nerve branch enlargements. Twenty patients with solitary lacrimal gland enlargements preferred watchful waiting due to mild disease symptoms. Of these, 90% of the lesions remained dormant throughout a median follow-up of 27 months. Among 51 patients treated with corticosteroids, 61% experienced relapses after treatment and required low-dose maintenance treatment.

 The results suggest extraocular muscle and trigeminal nerve branch enlargements may be secondary lesions that are resistant to systemic corticosteroid treatment, the study noted. The researchers also reported patients with extraocular muscle and/or trigeminal nerve branch enlargements appeared to be associated with elevated serum immunoglobulin G4 levels and blood eosinophilia.

Kubota T, Katayama M, Nishimura R, Moritani S, et al. Long-term outcomes of ocular adnexal lesions in IgG4-related ophthalmic disease. B J Ophthalmol. July 4, 2019. [Epub ahead of print].