Central Nervous System Demyelination in Paediatric Pars Planitis.

Abstract

Purpose: Intermediate uveitis (IU) can occur secondary to systemic autoinflammatory disorders, such as juvenile idiopathic arthritis and multiple sclerosis. In contrast, pars planitis (PP) specifically refers to an idiopathic form of IU, characterized by the absence of any identifiable underlying systemic condition. The frequency of the association between PP and CNS demyelination is unknown in children.

Methods: A retrospective analysis of paediatric PP patients' clinical and brain magnetic resonance imaging (MRI) data was conducted to investigate the coexistence of PP and CNS demyelinating pathologies in children.

Results: The cohort comprised 65 paediatric patients with PP, mean age 13.25 ± 3.1, (range 6-18, median 13) years, 41.5% female, who had at least one brain MRI. The mean follow-up was 4.02 ± 2.76 (range 0.5-11, median 4) years. Demyelinating lesions on MRI were visible in 5/65 (7.7%) patients. None reported neurological symptoms nor had abnormal findings on neurological examination. Three patients who had been undergoing adalimumab (ADA) treatment for a period of between three months and four years had their ADA therapy stopped when a demyelinating lesion was observed on MRI. Additionally, mycophenolate mofetil or methotrexate treatment was maintained as a maintenance therapy. The other two patients had not received any immunomodulatory treatment when demyelinating lesions were identified, and MRI findings were taken into consideration when treatment was planned.

Conclusions: Demyelinating lesions of the CNS can be detected in paediatric PP patients at a rate similar to adults. Clinicians should be aware of the presence of silent demyelination in PP and plan the anti-inflammatory treatment accordingly.