AimsThe optimal immunosuppressive therapy (IST) in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) remains uncertain. This study aimed to observe the disease course of MOG-ON and evaluate the therapeutic efficacy and tolerability of conventional immunosuppressants through Chinese cohort analysis.MethodsThis bidirectional cohort study included 121 patients with MOG-ON between January 2015 and December 2018. The clinical features and annualised relapse rate (ARR) of patients with and without IST were analysed.ResultsThe median age at onset was 17.5 years, and the sex ratio (F:M) was 1.24. Of 121 patients, 77 patients relapsed and 61 patients were younger than 18 years at disease onset. The overall median ARR of 63 patients in the non-IST group was 0.5, with 46.0% patients showing relapse at a median follow-up of 33.5 months. In the IST group, the ARR decreased from 1.75 pre-IST to 0.00 post-IST in 53 patients who received IST exceeding 6 months, with 20.8% patients showing relapse at a median follow-up of 23.8 months. The relapse rates of patients treated with rituximab (RTX) and mycophenolate mofetil (MMF) were not statistically different, but the rate of discontinuation was significantly lower in the RTX-treated group (18.2% vs 57.7%, p=0.0017).ConclusionThis study provides Class III evidence that both MMF and RTX may lower disease activity in patients with MOG-ON, and RTX showed better tolerability than MMF. However, observation after a single attack remains a good option because less than half of patients not on treatment suffered a relapse.

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BackgroundDifferent glial-autoantibodies-related paediatric optic neuritis (ON) are associated with different clinical characteristics and prognosis that require different treatments. Because glial autoantibody detection is not available in some parts of the world and there is often a delay in obtaining results, clinical factors that can be used to predict the subtype of paediatric ON are needed.MethodsThis was a single-centre retrospective cohort study. Children who presented with their first ON attack and with complete clinical data were included in the analysis. Single and multiple parameters for predicting paediatric myelin oligodendrocyte glycoprotein immunoglobin-associated ON (MOG-ON) and aquaporin-4 immunoglobin-related ON (AQP4-ON) were calculated.Results78 paediatric patients had their first ON attack from January 2016 to December 2019, of whom 69 were included in the final analysis, including 33 MOG-ON cases, 17 AQP4-ON cases and 19 Seronegative-ON cases. For predicting paediatric MOG-ON, the most sensitive predictors were ‘male or optic disc swelling (ODS) or bilateral’ (sensitivity 0.97 (95% CI 0.82 to 1.00)) and ‘follow-up visual acuity (VA) ≤0.1 logMAR or ODS’ (sensitivity 0.97 (95% CI 0.82 to 1.00)), and the most specific factor was ‘Age ≤11 y and simultaneous CNS involvement’ (specificity 0.97 (95% CI 0.84 to 1.00)). For predicting paediatric AQP4-ON, the most sensitive predictor was ‘Female or without ODS’ (sensitivity 1.00 (95% CI 0.77 to 1.00)), and the most specific factors were Neurological history (sensitivity 0.94 (95% CI 0.83 to 0.98)) and follow-up VA >1.0 logMAR (sensitivity 0.96 (95% CI 0.86 to 0.99)).ConclusionAccording to our data from a Chinese paediatric cohort, using multiple parameters increases the sensitivity and specificity of diagnosing paediatric MOG-ON and AQP4-ON. These can assist clinicians in diagnosing and treating paediatric ON when glial autoantibody status is not available.

Background There are no systematic reviews yet that evaluated the effects of PE/IA in patients with optic neuritis (ON) in demyelinating diseases. A meta-analysis of available study is needed to further explore the value of plasma exchange (PE) or immunoadsorption (IA) in treating ON in demyelinating diseases. Methods All relevant articles published on PubMed, Web of Science, Cochrane Library, Embase, China National Knowledge Infrastructure (CNKI), VIP Database, Wanfang, Sinomed and ophthalmology professional websites were searched. Study characteristics, demographic characteristics, clinical features and outcome measures were extracted. Response rate, adverse events (AE) rate, serious adverse event (SAE) rate, the log of the minimum angle of resolution (logMAR), visual outcome scale (VOS) and expanded disability status scales (EDSS) were evaluated using a random-effects model. Results 35 studies were included between 1985 and 2020, containing 1191 patients. The response rates of PE and IA in acute attack of ON were 68% and 82% respectively. LogMAR (−0.60 to − 1.42) and VOS (−1.10 to −1.82) had been significantly improved from within 1 month to more than 1 month after PE treatment. Besides, we found that logMAR improved 1.78, 0.95 and 0.38, respectively ,when the time from symptom onset to the first PE/IA was less than 21 days, 21–28 days, and more than 28 days. The pooled mean difference of EDSS was −1.14.Adverse effects rate in patients with PE or IA were 0.20 and 0.06, respectively. Conclusion The meta-analysis provided evidence that PE/IA treatment was an effective and safe intervention, and it is recommended that early initiation of PE/IA treatment is critical.

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Background HIF-1α is relevant to inflammation and fibrosis in hepatitis B virus (HBV)-related liver diseases. Thus, we designed a predictive model for decompensated cirrhosis. Methods Peripheral plasma HIF-1α levels were measured in 52 subjects, including 20 patients with HBV-related-compensated-cirrhosis (HBV-CC), 20 patients with HBV-related-decompensated-cirrhosis (HBV-DC) that underwent transjugular intrahepatic portosystemic shunt (TIPS), and 12 healthy controls (HC). Portal plasma HIF-1α levels were detected in HBV-DC patients. The correlation between clinical data and HIF-1α levels was assessed, logistic regression and nomogram were used to develop prediction model. Results Plasma HIF-1α levels were significantly higher in HBV-DC patients than that in HBV-CC patients and healthy controls (DC: 656.34±417.96, CC: 294.23±138.03, HC: 194.63±54.14, pg/ml; P = 0.0004). Plasma HIF-1α levels were positively correlated with total bile acid, total bilirubin, APRI, FIB-4, and MELD scores, and negatively correlated with albumin and platelets. Multivariate logistic regression manifested that total bilirubin (OR = 19.439; 95% CI: 1.486–254.320, P = 0.024), spleen thickness (OR = 75.144; 95% CI: 4.157–1358.440, P = 0.003) and HIF-1α concentrations above 341.78 pg/ml (OR = 23.580; 95% CI: 1.842–301.781, P = 0.015) were markedly associated with HBV-DC and thus included in the nomogram. The terrific cut-off value for the probability of HBV-DC was > 45%, and area under the curve was 0.954 (P < 0.001), with 95% sensitivity and specificity. Conclusions HIF-1α is related to biochemical liver parameters, cirrhosis grade, and progression to HBV-DC. Our model has preferable predictive value for HBV-DC.