Growing evidence demonstrates the association of encephalitis, meningoencephalitis or encephalomyelitis, with SARS-CoV-2 infection. This study aims to determine the profile and possible mechanisms behind CNS inflammatory diseases in the context of Covid-19. We conducted a systematic review of case reports on Covid-19-related encephalitis, meningoencephalitis, acute necrotizing encephalitis, and acute disseminated encephalomyelitis in adults, published before January 2021. A total of 182 cases (encephalitis = 109, meningoencephalitis = 26, acute disseminated encephalomyelitis = 35, acute necrotizing (hemorrhagic) encephalitis = 12) were included. While cerebrospinal fluid (CSF) pleocytosis and increased protein level was present in less than 50%, magnetic resonance imaging (MRI) and electroencephalogram (EEG) were abnormal in 78 and 93.2% of all cases, respectively. Viral particles were detected in cerebrospinal fluid of only 13 patients and autoantibodies were present in seven patients. All patients presented with altered mental status, either in the form of impaired consciousness or psychological/cognitive decline. Seizure, cranial nerve signs, motor, and reflex abnormalities were among associated symptoms. Covid-19-associated encephalitis presents with a distinctive profile requiring thorough diagnosis and thereby a comprehensive knowledge of the disease. The clinical profile of brain inflammation in Covid-19 exhibits majority of abnormal imaging and electroencephalography findings with mild/moderate pleocytosis or proteinorrhachia as prevalent as normal cerebrospinal fluid (CSF). Oligoclonal bands and autoantibody assessments are useful in further evaluating neuro-covid patients, as supported by our pooled evidence. Despite the possibility that direct viral invasion cannot be easily estimated, it is still more likely that immune-mediated or autoimmune reactions play a more important role in SARS-CoV-2 neuroinflammation.

Background: Inborn errors of immunity are a growing group of disorders with a wide spectrum of genotypic and phenotypic profiles. CARMIL2 (previously named RLTPR) deficiency is a recently described cause of immune dysregulation, mainly presenting with allergy, mucocutaneous infections, and inflammatory bowel disease. CARMIL2 deficiency is categorized under diseases of immune dysregulation with susceptibility to lymphoproliferative conditions. Case Presentation: Here we describe a 29-years-old male from a consanguineous family, with food and sting allergy, allergic rhinitis, facial molluscum contagiosum (viral infection of the skin in the form of umbilicated papules), eosinophilia and highly elevated serum IgE level. Whole exome sequencing revealed numerous homozygous variants, including a CARMIL2 nonsense mutation, a gene regulating actin polymerization, and promoting cell protrusion formation. Conclusion: The selective role of CARMIL2 in T cell activation and maturation through cytoskeletal organization is proposed to be the cause of immune dysregulation in individuals with CARMIL2 deficiency. CARMIL2 has an important role in immune pathways regulation, through cell maturation and differentiation, giving rise to a balance between Th1, Th2, and Th17 immune response. This case can improve the understanding of the different impacts of CARMIL2 mutations on immune pathways and further guide the diagnosis of patients with similar phenotypes.

This study aims to identify the patterns and risk factors of uveitis in a cohort of Iranian children referred to a tertiary pediatric rheumatology clinic. A retrospective analysis was performed using the medical records of all patients with uveitis of any cause who were referred to a tertiary pediatric rheumatology clinic, along with subsequent juvenile-onset Behçet's disease and juvenile idiopathic arthritis (JIA) cases as controls. Causes of uveitis included JIA (n = 24), Behçet's disease (n = 15), idiopathic uveitis (n = 14), Sarcoidosis/Blau syndrome (n = 3), systemic lupus erythematosus (n = 2) and reactive arthritis (n = 1). Anterior and panuveitis were the most common anatomical subtypes. Retinal vasculitis was found in 28.8% of patients and 57.6% had bilateral uveitis. Complications occurred in 32% of cases, persistent visual impairment in 23% and legal blindness in none. The median "time-to-uveitis" ranged from 1 year in oligoarticular JIA to 6.5 years in the polyarticular group, with no significant difference in cumulative prevalence. Risk factors of persistent/recurrent uveitis were a lower age at onset of uveitis (p = 0.010) and retinal vasculitis (p = 0.049). This study presents a distinct pattern of uveitis among different etiological groups and describes three rare causes of uveitis. JIA-associated uveitis has a late-onset peak in the polyarticular group. This study adds to the existing evidence on the importance of long-term screening for uveitis and early treatment in pediatric rheumatic diseases.

This report includes a retrospective analysis of 20506 children aged under 6 years old admitted with Coronavirus Disease of 2019 in Iranian hospitals. The total mortality rate was 2.9%, and 5.7% required mechanical ventilation. We demonstrate a higher mortality rate in comparison with existing studies as well as identifying clinical predictors of survival.