[Clinical and epidemiological features in Neuromyelitis Optica Spectrum Disorder]. / Comparaison clinico-épidémiologique des pathologies du spectre des neuromyélites optiques.

INTRODUCTION: Neuromyelitis optica spectrum disorder (NMO-SD) has been recognized for the past decade. Biomarkers such as anti-Aquaporin 4 antibodies (AQP4) and anti-Myelin Oligodendrocyte Glycoprotein (MOG) have been able to classify NMO-SD into several groups. METHODS: A retrospective study was performed in the Strasbourg University Medical Center among patients with AQP4+, MOG+ and double-seronegative NMO to compare their clinical, epidemiological and paraclinical features. RESULTS: Thirty-two patients with NMO were included. The AQP4+ NMO patients had a median of age of 45 years, with associated myelitis in 62.5% of cases and other autoantibodies in 37.5% of cases. The mean number of relapses by clinical history was 3. The mean initial visual acuity during an exacerbation was 0.3 LogMAR, and the visual acuity after an exacerbation was 0.1 LogMAR. MOG+NMO patients had a median age of 23 years, with severely impaired initial visual acuity (0.6 LogMAR) but better recovery (0 LogMAR); optic disc edema was present in 80% of cases; the mean number of relapses on clinical history was 1. AQP4-/MOG- NMO's were more common in women (70%) and were bilateral in 40% of cases. CONCLUSION: The diagnostic characteristics of NMO-SD are becoming increasingly differentiated, with a positive impact on functional prognosis and long-term progression. Other biomarkers have yet to be identified to improve the diagnosis and treatment of these disorders.

[Clinical and epidemiological profile of pediatric uveitis, course of inflammatory uveitis treated with anti-TNF alpha]. / Profil clinique et épidémiologique des uvéites pédiatriques, évolution des uvéites inflammatoires sous anti-TNF alpha.

INTRODUCTION: Uveitis is the leading cause of acquired childhood blindness with a prevalence of 30 cases per 100,000 inhabitants. There are multiple causes ; nevertheless, there is no standardized etiological assessment. The goal of our study is to define an epidemiological and clinical profile of uveitis diagnosed in a university hospital and their course when treated with anti-tumor necrosis factor (TNF) α. PATIENTS AND METHODS: All cases of uveitis under 18 years old, from 1994 to 2016, were included. Post-traumatic, post-surgical, pseudo-uveitis and retinopathy of prematurity were excluded. Demographic data, patient history, initial ophthalmological status, etiologic assessment data and treatments already underway were collected. RESULTS: Ninety cases of pediatric uveitis were included, among which were 16.7 % infectious uveitis, 38.9 % inflammatory uveitis and 44.4 % idiopathic uveitis. Etiologic investigations were considered incomplete in 45 % of idiopathic uveitis cases. Treatment with anti-TNFα was selected for 15.5 % of patients. In total, 33 % of patients treated with etanercept required other anti-TNFα drugs due to a lack of control of inflammation. Infliximab and adalimumab successfully managed to control inflammation in 28.6 % of cases each. DISCUSSION: Diagnostic criteria based adult systemic disease are sometimes inappropriate for children. The advent of anti-TNFα appears to improve the visual prognosis of inflammatory uveitis resistant to conventional immunosuppressant therapy, but we still need to perfect protocols for their use. CONCLUSION: There are neither standardized etiological assessment nor clear diagnostic and therapeutic protocols for children. TNFα inhibitors are more effective in controlling inflammation in severe pediatric uveitis.