A case report of pediatric-onset MS associated uveitis – Journal of Ophthalmic Inflammation and Infection

Pediatric MS, also referred to as pediatric-onset MS, early-onset MS or juvenile MS, challenges the management of pediatric IU. Less than 10% of patients with MS are under the age of 16, and 1% are even under the age of 10 [8].

The clinical characteristics of MS in children differ from that in adults; as the patients generally experience a more aggressive disease onset and course [9]. The diagnosis can be challenging, and many different diagnostic criteria have been proposed. The criteria developed by the Pediatric International Study Group have been employed by most researchers as well as our neurology colleagues in our institution [10].

The same problem exists for the diagnostic criterion for POMS-associated IU. However, it seems that the standardization of uveitis nomenclature (SUN) working group criteria for adult MS-associated IU can be used with considerations [11].

It should be pointed out that there are currently no distinct clinical or imaging findings that can accurately identify patients with IU who are at a higher risk of developing MS later on. As a result, routine brain MRI is not recommended for patients with IU unless they already display neurological symptoms or signs, or have additional neuro-ophthalmologic signs such as optic neuritis [12].

Acute pediatric demyelination is typically treated with first-line pulse steroid therapy (20 to 30 mg/kg/day (up to 1 g/day) for 3 to 5 days). Second-line therapies such as intravenous immunoglobulin (IVIG- 2 g/kg given over 2–5 days) or plasma exchange (to remove the pathological antibodies, cytokines, and other circulating inflammatory components from the bloodstream) are considered in patients with severe events or with incomplete or no improvement after receiving high-dose pulse steroid treatment [13, 14]. Considering that frequent relapses are correlated with poor prognosis, early institution of disease-modifying therapies for POMS is highly recommended [9]. According to certain specialists, it is advocated that potent immunosuppressant medications be administered initially to halt the progression of the disease and restore the functioning of the immune system. For patients with active disease and a higher likelihood of early disability, more intensive immunosuppressant treatment should be contemplated for a limited duration to attain disease control. Subsequently, a maintenance therapy involving lower-risk therapies should be implemented [15].

The presence of IU in patients with MS does not necessarily warrant the initiation of disease-modifying drugs (DMDs). Instead, the management approach should be customized to address the specific requirements for uveitis treatment. Nevertheless, for patients with MS who are already receiving DMDs, it is advisable to opt for local therapies to address ocular inflammation associated with IU. This approach helps minimize the risk of additional systemic side effects that may arise from immunomodulatory therapy. Of note, treatment with IFNβ, glatiramer acetate, mycophenolate mofetil, natalizumab, alemtuzumab, and anti-CD20 agents is effective in the management of both MS and IU [12].

Managing IU in pediatric patients with MS poses distinct challenges that hinder the establishment of a standardized guideline. The potential systemic side effects of medications on cognitive and physical development cannot be overlooked. Conversely, the utilization of local steroid injections, which are frequently administered in adults to mitigate systemic side effects, may pose the potential risk of glaucoma and cataract in pediatric patients. Moreover, the examination and monitoring of these young patients can be more intricate. Therefore, ophthalmologists must carefully evaluate each patient’s unique circumstances and tailor their treatment approaches accordingly.

We successfully managed our patient with active POMS-associated IU who had received high dose systemic steroid therapy, by adjuvant periocular steroid injection. We believe local steroid therapies could be administered to minimize the systemic side effects associated with systemic therapies in pediatric patients who are cooperative during examinations to detect potential ocular side effects such as glaucoma. Nevertheless, it is advisable to adopt a multidisciplinary approach involving both a neurologist and an ophthalmologist to ensure optimal treatment outcomes.