BackgroundAnti-phospholipid antibody associated chorea is a rare cause of this movement disorder, with a higher incidence in females and children. Other causes include Wilson’s disease, Sydenham’s chorea and ataxia-telangiectasia. Aetiology is uncertain, but may involve anti-phospholipid mediated dysfunction of the basal ganglia. We report an unusual case of anticardiolipin antibody related chorea in an adolecent male presenting to secondary care services.Case reportOur patient is a 14 year old male who became unwell in December 2016 with gradual onset of uncontrolled movements in his arms, and behavioural changes. There was a notable history of minor head injury, preceding sore throat and recurrent tonsillitis. Over the next six months, he reported worsening of choreoathetoid movements affecting his gait and upper limb function.He presented in April 2017 with acute slurring of speech three weeks after starting baclofen. He was then referred to tertiary paediatric neurology services. No features of Lupus were present on examination. His functioning was monitored after baclofen was stopped, and he progressed to treatment with high dose steroids, penicillin V and sodium valproate. He improved following significant rehabilitation with multidisciplinary involvement from occupational therapy, physiotherapy and the school team. His final diagnosis was revised to anticardiolipin antibody related chorea following results of immunological tests and consideration of the chronic progressive history. He was treated successfully with aspirin and mycophenolate mofetil, and has been discharged home with ongoing clinc review.Results12 lead ECG, echocardiogram and MRI brain normal.ASO Titre: 400 units/ml (50–200)Anti-DNAse B: 100 units/ml (<240 units/ml)Anti-Mitochondrial Ab: positiveAnti-Nuclear Ab titre: 1:320 (Positive)Anti-cardiolipin IgG Ab 1184.2 units/ml (0–19.9)Anti-B2-GP-1 IgG 4269.7 units/ml (<20)ConclusionAs a patient’s disease continues to evolve, so should our diagnostic approach. Atypical progession of disease should prompt review. Generally, treatment of anticardiolipin related chorea falls into 2 pathways; anticoagulation and immunosuppression, with evidence limited to case reports and small case series.Acknowledgements to the local medical illustration team for performing serial videos showing improvement in function.

Background:Belimumab (BEL) is a human monoclonal antibody that specifically inhibits B-cell activating factor (BAFF). PLUTO is an ongoing trial evaluating efficacy and safety of intravenous (IV) BEL in children ≥5 years of age with cSLE. Efficacy, and safety endpoints of PLUTO have been reported;1briefly, numerically more BEL vs PBO pts met the primary and major secondary efficacy endpoints. We present patient (pt) response to BEL over time.Objectives:To evaluate changes in SLE Responder Index (SRI) 4 and SRI6 responses, and disease activity over 52 weeks, in paediatric pts receiving BEL, or placebo (PBO), plus standard SLE therapy (SST).Methods:PLUTO (GSK Study BEL114055,NCT01649765) is a Phase 2, randomised, double-blind, placebo-controlled study. Pts 5–17 years of age with active cSLE were randomised to monthly BEL 10 mg/kg IV, or PBO, plus SST. Endpoints assessed: SRI4 and SRI6 response rate, mean percentage and absolute change from baseline in Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLE Disease Activity Index (SLEDAI) and Physicians’ Global Assessment (PGA) scores, and percentage of pts with no new British Isles Lupus Assessment Group (BILAG) 1A/2B organ domain scores compared with baseline, all by study visit. The last-observation-carried-forward (LOCF) principle (missing values imputed using the last available non-missing value) was applied to pts who withdrew or received protocol-prohibited medication or a dose of allowable medication that resulted in treatment failure prior to the Week (Wk) 52 visit. Descriptive statistics were used.Results:A total of 93 pts (94.6% female, mean [SD] age 14.0 [2.49] years) were randomised for the intention-to-treat (ITT) population: 53 to BEL and 40 to PBO. Mean (SD) BEL and PBO baseline scores were 10.3 (3.34) and 10.4 (3.63) for SELENA-SLEDAI and 1.3 (0.43) and 1.4 (0.42) for PGA, respectively. Pt number with at least BILAG 1A/2B organ domain involvement at baseline was 37 (69.8%) for BEL and 29 (72.5%) for PBO. SRI4 and SRI6 responses over 52 weeks were mostly numerically higher with BEL than PBO; more BEL than PBO pts were SRI4 and SRI6 responders at Wk 52 (Figure 1). Unadjusted mean (SE) percentage changes from baseline over time in SELENA-SLEDAI and PGA scores generally favoured BEL over PBO, as did unadjusted mean (SE) absolute changes (Figure 2). Wk 52 adjusted mean (95% CI) percentage treatment difference vs PBO was -4.0% (-21.8, 13.9) for SELENA-SLEDAI and -6.1% (-23.9, 11.7) for PGA, while Wk 52 adjusted mean (95% CI) treatment difference vs PBO was -0.7 (-2.4, 1.1) for SELENA-SLEDAI and -0.1 (-0.3, 0.1) for PGA. Over the study duration, numerically more BEL than PBO pts had no new BILAG 1A/2B organ domain scores (Figure 2).Figure 1.SRI4 and SRI6 response by study visitFigure 2.SELENA-SLEDAI and PGA score mean percentage and absolute change from baseline, and no new BILAG 1A/2B organ domain scores compared with baseline, all by study visitConclusion:In line with the main analyses performed at Wk 52,1further analyses of responses over time in SRI4, SRI6 and disease activity generally favoured BEL over PBO. Combined, these results continue to support the efficacy profile of IV BEL in the treatment of children with cSLE.References:[1]Brunner HI,et al.Arthritis Rheumatol.2018;70(59): 3224–5, Abst. 2867Acknowledgments:We acknowledge all PLUTO investigators (PRINTO, PRCSG and otherwise affiliated). Study funding: GSK.Disclosure of Interests:Nicolino Ruperto Consultant of: Ablynx, AbbVie, AstraZeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GSK, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, Sanofi, Servier, Sinergie, Sobi and Takeda, Liza McCann: None declared, Syuji Takei Grant/research support from: Eisai, Consultant of: Novartis, Bristol-Myers Squibb, Speakers bureau: GSK, Sanofi, Tanabe-Mitsubishi, Novartis, Chugai, Ono, Abbvie, Eli-Lilly, Bristol-Myers Squibb, Clarissa Pilkington: None declared, Damon Bass Shareholder of: GSK, Employee of: GSK, Beulah Ji Shareholder of: GSK, Employee of: GSK, Anne Hammer Shareholder of: GSK, Employee of: GSK, Mohamed Okily Shareholder of: GSK, Employee of: GSK, Gina Eriksson Shareholder of: GSK, Employee of: GSK, Holly Quasny Shareholder of: GSK, Employee of: GSK, Hermine Brunner Consultant of: Hoffman-La Roche, Novartis, Pfizer, Sanofi Aventis, Merck Serono, AbbVie, Amgen, Alter, AstraZeneca, Baxalta Biosimilars, Biogen Idec, Boehringer, Bristol-Myers Squibb, Celgene, EMD Serono, Janssen, MedImmune, Novartis, Pfizer, and UCB Biosciences, Speakers bureau: GSK, Roche, and Novartis

Background/Aims Some studies have shown that ultrasound can be more sensitive than clinical examination in detection of synovitis and improves diagnostic accuracy when clinical examination is in doubt. This study aimed to review if ultrasound results correlate with the clinical impression in patients with juvenile idiopathic arthritis (JIA) and determine if imaging helps to change management and assist in the optimisation of radiology resources, by improving our knowledge of when ultrasound is suitable. Methods We designed a retrospective study to review cases of JIA aged 1-18 years who have had an ultrasound requested from clinic between September 2021 - April 2022. Patients were excluded from study if; diagnosis was uncertain, new patients with JIA and patients with arthritis as part of a systemic inflammatory disorder. Data was collected from the radiology database and electronic patient record (MEDITECH). Results A total of 40 patients matched eligibility criteria with ultrasound performed on average 4 weeks after request. 13 (32.5%) male and 27 (67.5%) female with an average age of 11.7 years (3 -18 years). Of these patients 16 (40%) failed to demonstrate any active clinical signs of arthritis or tenosynovitis in clinic. The most common reasons for scan in this cohort included parental concern (43.75%) and difficulty of examination (12.5%). Other reasons included: patient reporting pain or morning stiffness in the absence of clinical signs during the consult. In these patients 13 (81.25%) were normal and 3 (18.75%) demonstrated chronic changes. Ultrasound in these patients did not lead to any change in treatment. 22 (55%) of patients demonstrated clinical signs of arthritis in clinic. 7 (31.2%) of these demonstrated active arthritis or tenosynovitis and subsequently had treatment changes in the form of a steroid injection (n = 5) or change in systemic medication (n = 5). Of those that had clinical signs but no active arthritis on ultrasound, 8 (36.4%) scans were found to be non-diagnostic / demonstrated chronic synovial thickening and 7 scans were normal (32%). 2 (5%) patients were seen in virtual clinic and not examined; both had a normal scan. Small joints of the ankles (26%) and hands (23%) are the most common joints scanned. Conclusion The use of ultrasound demonstrates benefit when clinical findings are present, and the scan allows clinicians to differentiate between active synovitis, tenosynovitis and chronic damage. In patients whose clinical examination does not show signs of inflammation, there is less evidence in this study for the use of ultrasound, and we should trust our clinical acumen. Disclosure L.A. Stead: None. N. Malde: None. L. McCann: None. N. Barnes: None. C. Landes: None. K. Gargh: None.

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