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Purpose: This study analyzes the efficacy and safety of tofacitinib in pediatric patients presenting with treatment-resistant uveitis and scleritis. Method: Retrospective Chart Review Result: Nine children diagnosed with uveitis and one with scleritis received oral tofacitinib treatment. The median age of these patients was 9 years, with bilateral involvement observed in nine of them. Juvenile idiopathic arthritis was the most identifiable cause of uveitis, with anterior uveitis (50%) being the most frequent subtype of inflammation among these children. The median duration of immunosuppressive treatment before switching to tofacitinib was 18 (16–49) months. Remission of uveitis was achieved in all but two children, who experienced recurrence – manifesting as anterior uveitis. The median duration of follow-up in these children after tofacitinib treatment was 277.5 (183–549) days. At the end of follow-up, topical steroids could be withdrawn in six children, and two children were on topical steroids once a day. None of the children developed any systemic side-effect during the follow-up period. The mean BCVA at presentation was 0.62 ± 0.55, which improved to a mean of 0.27 ± 0.325 at the final follow-up (p = 0.0014) Conclusion: Treatment of pediatric uveitis with tofacitinib can be a valuable second-line treatment option and useful alternative in low- and middle-income countries. [ABSTRACT FROM AUTHOR]

Background: X-linked agammaglobulinemia, a primary immunodeficiency, can present with musculoskeletal manifestations. Case characteristics: A 4-year-old boy, diagnosed as systemic juvenile idiopathic arthritis at the age of 3 years and treated with biological agents, presented with fever, dyspnea and chest pain. Blood culture and pericardial fluid culture revealed Achromobacter xylosoxidans. Outcome: Investigation revealed normal serum ferritin but low levels of serum immunoglobulins. Further immunological work-up revealed diagnosis of X-linked agammaglobulinemia. Child improved on antibiotic therapy; treatment with steroids and biological was discontinued. Message: Underlying immunodeficiency disease must be looked for in children suspected to have juvenile arthritis, more so if they develop unusual serious infection in response to immunomodulatory therapy. [ABSTRACT FROM AUTHOR]

Blau syndrome (BS) is a rare autoinflammatory disorder characterized by the clinical triad of arthritis, uveitis, and dermatitis due to heterozygous gain-of-function mutations in the NOD2 gene. BS can mimic juvenile idiopathic arthritis (JIA)-associated uveitis, rheumatoid arthritis, and ocular tuberculosis. We report a family comprising a mother and her two children, all presenting with uveitis and arthritis. A NOD2 mutation was confirmed in all the three patients - the first such molecularly proven case report of familial BS from India. [ABSTRACT FROM AUTHOR]

Background and Aim: pGALS (pediatric Gait Arms Legs Spine) is a useful screening tool in identifying musculoskeletal problems in children. Although musculoskeletal problems are common in children, only a small fraction of them have an underlying serious pathology. There is limited data on this subject from north India but none from south India. Our aim was to identify the prevalence of musculoskeletal problems in school going boys in the local community. Methods: We conducted a prospective cross-sectional study among school boys 7–17 years of age from September 2017 to July 2019, in Chennai. The parents or the boys were asked pGALS screening questions and this was followed by the extended pGALS examination including Beighton's scoring for hypermobility. If abnormalities in the screening tool were found, the subjects were then referred to a pediatric rheumatologist for further evaluation. Results: A total of 1543 boys were included in this study. Of these 37% of the study group were found to be hypermobile. According to our study, the prevalence of growing pains was 4.6%. Of 2 boys with suspected pathology 1 boy had enthesitis-related arthritis and another had slipped upper femoral epiphysis (SUFE). The estimated prevalence of juvenile idiopathic arthritis (JIA) was 65/100000. According to our study 12.3% of the children were obese. Conclusion: pGALS is a useful tool to screen for musculoskeletal problems in school going boys. The estimated prevalence of JIA was 65/100000. Of them, 37% of the children had hypermobility. Obese children were more likely to complain of musculoskeletal pain (P value 0.00). [ABSTRACT FROM AUTHOR]