Background Uveitis is a known cause of blindness in the developed world, where non-infectious diseases dominate the spectrum of underlying aetiologies. However, data from sub-Saharan Africa is lacking. Here we aim to describe the diseases associated with non-infectious uveitis and the impact of currently available treatment in this setting. Methods A retrospective observational analysis of children with non-infectious uveitis from January 2010 to December 2017, attending the tertiary paediatric rheumatology and ophthalmology referral units in Cape Town was conducted. Statistical analysis utilising STATA13 software was performed with p < 0.05 considered significant. Results Twenty-nine children were identified with a median age at first visit of 74 months (IQR 49–86 months), female to male ratio of 0.9:1, predominantly of mixed race (72.4%). Juvenile idiopathic arthritis associated uveitis (JIAU) (48.3%) was the most frequent diagnosis. All children with JIAU had chronic anterior uveitis and 3 (21.4%) presented with uveitis before arthritis. There were no differences between children with uveitis and those with arthritis only, for gender (p = 0.68) and race (p = 0.58) but significantly, children with uveitis presented at an overall younger age (p = 0.008), with antinuclear antibody positive (p < 0.001) oligo-articular JIA (p = 0.01) and older age appeared to be protective (p = 0.01 OR1.0 CI 0.6-1.7). Children with idiopathic uveitis (41.4%) were predominantly male (66.6%), of mixed race (75%), with chronic anterior uveitis (41.7%) and presented with cataracts (100%). Less commonly, sarcoidosis (6.9%) and Behcet's disease (3.5%) were diagnosed. 55.2% had complications at presentation, predominantly cataracts (87.5%). 19 children (65.5%) had inactive disease at 12 months from diagnosis and remission as assessed at the last clinical visit, was achieved in 58.6% on standard initial therapy and in 75% of those on tumour necrosis factor inhibitors. Surgery was needed in 41.4%, primarily in the idiopathic group. Visual acuity improved or was maintained on treatment. Conclusion The spectrum and characteristics of immune mediated non-infectious uveitis are comparable to that reported in developed countries. Current practice detects children with potentially sight-threatening disease and access to tumour necrosis factor inhibitors has improved outcomes in refractory cases.

Study rationale: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory myopathy of childhood with an incidence of 1.9-3.2 per million. The aetiology of JDM is uncertain but may result from immune dysregulation triggered by environmental factors in genetically susceptible children. The demographic and clinical characteristics of JDM may thus differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. There is need for further studies for better understanding of the epidemiology, clinical characteristics and outcome of patients with JDM from the continent. Methods: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004-2013 in Red Cross, Groote Schuur and Tygerberg hospitals in Cape Town. Data was analyzed using R version 3.1.0 (2014-04-10). Results: Twenty-five cases were identified: 16 female and 9 male. Thirteen (52%) of the cases were of indigenous African, eleven (44%) mixed and one (4%) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0-9.7) and 7.9 (range 3.4-9.75) years respectively. Muscle weakness and characteristic cutaneous manifestations occurred in all the 25 patients while 24 had elevated muscle enzymes. All the patients received corticosteroids, seventeen (73.9%) received methotrexate and four received rituximab. Eleven patients had calcinosis during the disease course [median follow up period of 50 (range 0.5-159) months]. The mortality was 2/25 (8%) while only 40% of the patients had clinically inactive disease by PRINTO criteria. There was no difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p -value = 0.75) between patients who had clinically active and inactive disease. Discussion: The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. The median delay in diagnosis (4 months) was not longer than that reported in most other studies. However, some children had prolonged delay of up to 7 years due to misdiagnosis that denied them appropriate treatment in a timely manner. Majority (60%) of the patients also remained with clinically active disease, which put them at risk of further disease complications including calcinosis. Even though the mortality rate was low (8%) this was still more than double that reported in most recent large studies especially from the resource rich countries. Conclusions: Long-term follow up of JDM patients is advisable since majority of patients seem to have clinically active disease many years after disease onset despite treatment. Formulation and use of appropriate treatment guidelines and protocols may aid in the early diagnosis and appropriate management for optimum outcomes.

Objective: To systematically review and summarize the available literature regarding the effectiveness and safety of biologics in the treatment of juvenile-onset systemic lupus erythematosus. Methods: PubMed was systematically searched for relevant literature (2012–2017 inclusive) using the following criteria: (1) patients diagnosed with juvenile-onset systemic lupus erythematosus (≤18 years at diagnosis); (2) treatment with any biological agent; and (3) outcome measures assessing effectiveness and safety. Systematic literature reviews, meta-analyses, randomized controlled trials, cohort studies, case control studies, cross sectional surveys and case-series with ≥3 patients were included. Independent extraction of articles by two authors using predefined criteria was performed. The quality of each study was assessed using CASP tools and Oxford CEBM Levels of Evidence. Results: Nine articles met inclusion criteria: six cohort studies, two case series and one pilot study, totalling 230 patients. All but one article reported the effects of rituximab, the other those of belimumab. Overall, patients had active disease refractory to standard of care regimens using corticosteroids and immunosuppressants. Available evidence for rituximab demonstrated improvements in disease activity, complement levels and anti-dsDNA titres accompanying a steroid-sparing effect. Conclusion: Rituximab can be considered an effective treatment in juvenile-onset systemic lupus erythematosus patients with severe disease manifestations and/or refractory disease. Based on current evidence, use of belimumab in juvenile-onset systemic lupus erythematosus patients cannot be recommended. The long-term safety of these biological agents remains uncertain. Further prospective studies, ideally robust randomized controlled trials, are urgently needed to obtain more accurate data on the effectiveness and long-term safety of rituximab, belimumab and other biologics in juvenile-onset systemic lupus erythematosus.