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Background Rituximab is an anti-CD20 chimeric monoclonal antibody that depletes pre-plasma B cells. It is used in paediatric rheumatology for conditions including juvenile systemic lupus erythematosus (jSLE), juvenile dermatomyositis (JDM), vasculitides and refractory juvenile idiopathic arthritis (JIA). We aim to describe the cohort of patients treated with rituximab in the Scottish Paediatric and Adolescent Rheumatology Network (SPARN) in a 5-year period, and assess the need for a network guideline. Methods A review of paper and electronic case records. Patients seen within the SPARN network who received Rituximab between 2014 and 2018 were included. Results Vasculitis Rituximab was given to 16 patients in 5 different SPARN centres between 2014 and 2018. Male: Female ratio was 1:4.3. Mean age at first dose of rituximab was 12.75 years. Mean disease duration pre rituximab was 1 year. 8 (50%) patients had jSLE, 7 multisystem and 1 isolated lupus nephritis. 3 had ANCA-positive vasculitis and 5 had other conditions. (1 had RF+ve JIA; 1 severe panuveitis; 1 had an unspecified autoinflammatory condition; 1 had COPA syndrome with severe interstitial lung disease and 1 had autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Documentation of pre-rituximab checks was poor. Baseline bloods were done in all 16 patients. 11 patients had lymphocyte subsets and 13 immunoglobulins documented. Disease-specific antibodies were measured in all patients in whom applicable (11). 11 had a chest x-ray prior to rituximab. In 9 cases a pre-rituximab pregnancy test was indicated: none were documented. In only 3 patients was a pre-rituximab history of recent infections documented. In 8 patients a discussion regarding treatment risks and benefits was documented. Post-infusion lymphocyte subset monitoring was variable but documented in 15. 1 patient was never B cell deplete. 9 never fully reconstituted. RTX dose information was available for 14. All received doses of 750mg/m2 with 6 having the dose capped at 1g. Dose frequency information was available for all. The majority (9) received 2 doses. Of the rest, 1 patient received a single dose. 3 received 3 doses, 2 had 4 and 1 had 5. All patients in whom information was available, received pre-medication with paracetamol, chlorphenamine and methylprednisolone (n = 14). 4 patients had side effects noted (rash/itch in 1, anaphylaxis in 1, facial flushing in 1 and post-infusion infection in 1). Clinical response was clearly documented in 8 patients, 7 of whom showed clinical improvement (2 had systemic jSLE, 2 had ANCA +ve vasculitis, 1 had isolated lupus nephritis, 1 had COPA syndrome and 1 had RF+ JIA). Conclusion Rituximab is used for a range of conditions and in a number of centres within SPARN. This review highlights variation in and poor documentation of pre-rituximab discussions and investigations, prescribing, monitoring and response. A SPARN rituximab protocol is being developed to address this. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

Background Although juvenile idiopathic arthritis (JIA) occurs early in life, patients can require long-term treatment into adulthood due to active disease or flares. However, most available studies show that there are gaps in care during and after the transition to adult care. Transition has been associated with higher dropout rates, and there is limited evidence on treatment persistence in JIA patients. The current analysis evaluates etanercept bio-original (ETNBo) persistence up to 5 years and reports diagnostic patterns in early adolescence and young adults in Germany. Methods Patients with JIA (ICD10: M08) between 2-30 years of age with a first recorded ETNBo prescription between 1-January-2008 and 31-December-2012 were retrospectively identified in German health insurance claims data. Patients were followed until 31-December-2015 or end-of-record, whichever came first. Index date was set at the first ETNBo prescription after a 12-month etanercept-free look-back period. Patient characteristics, concomitant treatments and prior use of other biologic Disease-modifying antirheumatic Drugs (bDMARD) were evaluated in the 12-month look-back period. The 5-year etanercept treatment persistence, defined as median (95% confidence interval [CI]) months between the index date and a discontinuation (≥90-day treatment gap after last etanercept prescription), was estimated using Kaplan-Meier analysis, overall and for bio-naïve patients. Etanercept WHO defined daily dose (DDD) was adjusted by age; for patients 2-6, 7-10, 11-14 and >15 daily doses were defined as 1.4mg, 3.6mg, 5.4mg and 7.0mg, respectively. The proportion of patients receiving an adult rheumatic diagnosis after the age of 11 was described. Results We identified 125 patients diagnosed with JIA (median age: 15 years; 30% male). At index quarter 56% of patients were in the 11-20 age group. Most patients (94%) were bio-naïve and 46%, 34%, and 17% had concomitant use of NSAIDS, other DMARDS, and corticosteroids, respectively. Overall median persistence was 13 months (95%CI: 10-16; IQR: 5-27). Persistence for the bio-naïve patients was 50% (95%CI: 41-57), 27% (95%CI: 20-34), 16% (95%CI: 10-23), 10% (95%CI: 5-17) and 9% (95%CI: 4-15) at 12, 24, 36, 48, and 60 months respectively. The majority of patients (68%) received an additional diagnosis of rheumatoid arthritis after age 11, while 15% were given other adult rheumatic diagnoses. Only 9% of JIA patients had received no additional diagnosis to JIA. Conclusion This analysis provides important understanding of care in young JIA patients. We observed a decreasing persistence of etanercept therapy as patients progressed in time through the transition period into early adulthood. Importantly, we found that the majority of JIA patients received additional adult diagnoses, which suggests the transition could impact on the clinical management of this disease. Diagnostic patterns, clinical and patient circumstances during this critical period and their impact on persistence requires further analysis. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]