Immunoglobulin a vasculitis with central nervous system involvement: analysis of 10 cases

Abstract

Immunoglobulin A vasculitis (IgAV) is a systemic inflammatory disease that affects small blood vessels. Central nervous system (CNS) involvement in IgAV is rare. This study analyzed the clinical characteristics of IgAV patients combined with CNS damage in children. Furthermore, the study made a comparison between the characteristics of IgAV patients with and without CNS damage, and initially explored the potential predictors for IgAV patients with CNS damage. A retrospective analysis was conducted on a cohort of 50 children diagnosed with IgAV and admitted to Beijing Children's Hospital from 2016 to 2019. The study encompassed a review of the clinical presentations, laboratory test results, imaging findings, therapeutic interventions, and prognoses of 10 children with IgAV who exhibited CNS involvement. These 10 cases were then compared with a group of 40 children with IgAV without CNS involvement. The prevalence of IgAV with CNS manifestations was 0.2%. The median age was 11.6 years, with a male-to-female ratio of 7:3. All CNS symptoms appeared after the purpuric rash. The mean period from IgAV onset to the development of neurological symptoms was 12.2 days (range: 1-27 days). Seizures were the most common neurological manifestation, with impaired consciousness and predominant convulsions. Other symptoms included headache, visual impairment, dysarthria, dyskinesia, and emotional irritation. The main abnormalities found on brain magnetic resonance imaging (MRI) were unilateral or bilateral abnormal focal signals, cortical and subcortical white matter edema, and thrombosis of the venous sinus. Glucocorticoid therapy and intravenous immunoglobulins were used to treat CNS damage caused by IgAV. All patients showed clinical improvement without recurrent neurological symptoms or sequelae. Statistically differences were identified in in terms of age, gastrointestinal damage, WBC count, NLR, ALB, C3 levels, and the CD4/CD8 ratio in IgAV patients with CNS damage when compared to those without CNS damage. Multivariable logistic regression analysis shows that age, NLR and C3 Levels are predictors of IgAV with CNS damage. CNS involvement in IgAV is a rare complication. Its clinical manifestations are diverse and vary in severity, and its diagnosis is exclusionary. Brain MRI is beneficial for diagnosis and follow-up. Steroid therapy is important for treating IgAV-associated CNS involvement. Age, NLR and C3 Levels are predictors of IgAV with CNS damage.

Keywords: Central nervous system involvement; Immunoglobulin A vasculitis; Intravenous Immunoglobulins; Methylprednisolone; Potential predictors; Seizure.