Background: the term scleritis refers to the inflammation of the sclera, the white outer type I collagen tunic of the eye (1). It may be rarely found in pediatric patients, with the incidence of pediatric scleritis accounting for about 1.2% of all scleritis cases (2). An early diagnosis and an adequate treatment are mandatory to avoid irreversible ocular sequelae (3). In this regard, the experience of biotechnological agents in patients with pediatric scleritis is quite limited at current.

Objectives: To assess the effectiveness of biotechnological agents in terms of scleritis control and glucocorticoid dosage tapering in patients with pediatric-onset scleritis; b) to assess the safety of the biotechnological agents employed and their ability in avoiding new ocular disease complications.

Methods: patients with pediatric-onset scleritis resistant to the conventional therapy and treated with biotechnological agents were retrospectively enrolled and prospectively followed-up. Demographic, clinical, ophthalmologic and therapeutic data were collected. The endpoints of the study consisted in the description of the therapeutic details gathered at the start of the biotechnological agents compared to what was observed at the 6-month, 12-month and last follow-up visits.

Results: 5 patients (3 males; 2 females) treated with adalimumab, infliximab, abatacept secukinumab were consecutively enrolled. A remarkable clinical efficacy was observed in 5/5 cases during a 10-to-54 months follow-up period; complete and persistent suppression of ocular inflammation was pointed out in 2/2 patients with active disease at the start of the treatment, while a clinically relevant decrease of scleritis relapses was observed in 5/5 patients after the start of biotechnological treatment, compared to the past years of scleritis activity. In particular, the 5 patients suffered from a total number of 17 scleritis relapses during the 12 months preceding the start of the biologic treatment; the same patients suffered from 2 scleritis relapses within the first 12 months from the start of the treatment and 4 scleritis relapses during the entire follow-up period (median time of 28 months, interquartile range=118 months). These results were obtained despite the progressive and persistent glucocorticoid sparing effect, with a median daily dosage of prednisone or equivalent ranging from 15 (interquartile range =21.75) mg/day at the start of treatment to 2.5 (interquartile range =8.75) mg/day at the 3-month assessment, 2.5 (interquartile range=0) mg/day at 6 month visit, and 0.0 (interquartile range =2.5) mg/day at the last assessment. Regarding the safety profile, one out of five patients experienced an adverse event represented by oral candidiasis, presumably related to the concomitant use of glucocorticoids.

Conclusion: biotechnological agents have shown to be highly effective in controlling pediatric-onset scleritis, allowing a remarkable glucocorticoids sparing effect in patients previously resistant to conventional treatment and recalcitrant to a glucocorticoid-sparing approach. The safety profile was excellent.

REFERENCES:

[1]Yanoff M, Duker JS. Ophthalmology 5th ed . New York: Elsevier; 2019.

[2]Majumder PD, Ali S, George A, et al. Clinical Profile of Scleritis in Children. Ocul Immunol Inflamm 2019;27:535-539. doi: 10.1080/09273948.2017.

[3]Murthy SI, Sabhapandit S, Balamurugan S, et al. Scleritis: Differentiating infectious from non-infectious entities. Indian J Ophthalmol 2020; 68:1818-1828. doi: 10.4103/ijo.IJO_2032_20.

Disclosure of Interests: None declared