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Purpose To report the clinical outcomes of adalimumab in treating refractory Behcet’s disease (BD)-related uveitis in paediatric or adolescent patients. Methods Retrospective review of five paediatric or adolescent patients with BD-related uveitis with a minimum follow-up of 24 months. Results Disease quiescence was observed in 9 (90%) of 10 eyes at 12 months. The mean number of relapses per year per patient was 5 (range, 3–7) before initiation of adalimumab treatment. This was reduced to 0.2 relapse per patient per year among the five patients during the first 24 months after starting adalimumab treatment. At baseline, 5 eyes had active retinal vasculitis. Retinal vasculitis resolved in all cases (100%) after starting adalimumab. The mean time to complete resolution of inflammation was 3.4 weeks. The mean ± standard deviation logMAR best-corrected visual acuity was 0.711 ± 0.63 at baseline and improved to 0.172 ± 1.04 at 12 months (P < 0.001). None of the patients developed any adverse events associated with adalimumab treatment. Conclusion Adalimumab was effective in preventing irreversible sight-threatening BD-related uveitis in paediatric or adolescent patients. Adalimumab appears to be a promising treatment option for young patients with recalcitrant BD-related uveitis and has a favourable safety profile.

Background Rheumatic diseases and or medications (for example methotrexate) used to treat the underlying condition are associated with the development of interstitial lung disease (ILD). Development of ILD in subjects with juvenile idiopathic arthritis (JIA) is assumed to be rare, however, severe respiratory complications are increasingly reported in JIA patients. Objectives To describe the prevalence of respiratory diagnoses and ILD in a group of Chinese JIA patients at a tertiary center. Methods Retrospective chart review of JIA patients attending rheumatology clinic during 2012 to 2019 Results Fifty-eight cases of JIA were identified. Thirty-three (57%) received DMARDs, majority were on methotrexate (MTx, 24, 73%) and sulphasalazine (SSZ, 14, 42%). Eleven (19%) were currently or previously on biologics (adalimumab 5, etanercept 5, anakinra 1, previous exposure: infliximab 1, etanercept and anakinra 1). Respiratory diagnosis was made in 11 (19%) patients. There were 2 (3.4%) cases of ILD diagnosed during the study period (see below). The rest were asthmatics. Among them, 9 were on DMARDs [MTx 8(89%), SSZ 4(44%), leflunomide 2(LEF), hydroxychloroquine 1(HCQ)] or biologics [Etanercept 3(33%), adalimumab 1, tocilizumab and anakinra 1(sequential use)]. Four subjects were on combination DMARDs [MTx&SSZ in 2, 1 each for MTx+LEF+HCQ+SSZ (sequential combination)& MTx+LEF]. Three received combination DMARDs and biologics (1 each for MTx+adalimumab, MTx+etanercept & MTx+anakinra). Cases of ILD One was a SJIA patient diagnosed at 2 years old. She failed methotrexate, etanercept & tocilizumab (infusion reaction). She was on low dose prednisolone, methotrexate and anakinra when she presented to us with acute right heart failure requiring ICU care 3 years after diagnosis. Advanced clubbing was noted (fig 1). HRCT thorax on presentation showed severe interstitial lung disease with marked fibrosis and traction bronchiectasis (fig 2). She was diagnosed acute on chronic cor pulmonale and pulmonary hypertension secondary to ILD. The other was an oligoarticular JIA patient, diagnosed at 2 years old. She was refractory to intra-articular steroid injections and combination DMARDs including MTx, SSZ, LEF & HCQ. Arthritis was controlled by Etanercept. At 12 years of age, she started to have chest pain. Lung function test showed restrictive lung pattern. HRCT thorax showed pulmonary fibrosis. Conclusion From this Chinese cohort we identified 2 cases of ILD. They did not present with any symptoms until advanced stage or when complications set in. Both were very young at diagnosis and were difficult to control, as evidenced by the use of multiple DMARDs and biologics including methotrexate, IL6 and IL1 blockade. The causes of ILD are believed to be multifactorial. For severe case, prognosis is poor1. Although rare, methotrexate induced pneumonitis do occur. Symptoms of ILD may not be obvious until late stage. Clinical examination alone is not sensitive enough to detect disease progression. Asthma is common, as in this cohort, and may further complicated the assessment. It is recommended in the recent SHARE initiative on JSLE to screen for lung disease2. We believe it should be performed in JIA patients as well. 6 minute walk test is a noninvasive way to assess cardiopulmonary functional status and is validated in children3. Patients with unexplained chest symptoms or impaired lung function should be referred to pulmonologist for further assessment. References [1] Kimura Y, et al. Pulmonary Hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res2013;65(5):856-52 [2] Groot N, et al. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative. Ann Rheum Dis2017;76:1788-96 [3] Li AM, et al. The six-minute walk test in healthy children: reliability and validity. Eur Respir J2005;25:1057-60 Disclosure of Interests None declared

ObjectiveTo investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA).MethodsIn a multicentre retrospective study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data.ResultsLD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymphopaenia preceded LD detection. The most prevalent chest CT pattern was septal thickening, involving the periphery of multiple lobes ± ground-glass opacities. The predominant pathology (23 of 36) was pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia (PAP/ELP), with atypical features including regional involvement and concomitant vascular changes. Apparent severe delayed drug hypersensitivity occurred in some cases. The 5-year survival was 42%. Whole exome sequencing (20 of 61) did not identify a novel monogenic defect or likely causal PAP-related or macrophage activation syndrome (MAS)-related mutations. Trisomy 21 and young sJIA onset increased LD risk. Exposure to IL-1 and IL-6 inhibitors (46 of 61) was associated with multiple LD features. By several indicators, severity of sJIA was comparable in drug-exposed subjects and published sJIA cohorts. MAS at sJIA onset was increased in the drug-exposed, but was not associated with LD features.ConclusionsA rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed.

We report the neurologic and radiologic manifestations of three adolescent girls with acute carbon monoxide poisoning. The girls were found collapsed and unconscious in a bathroom where liquid petroleum gas was being used as heating fuel. As hyperbaric oxygen therapy was not available locally, they only received oxygen supplementation via nasal cannula (4 L/minute) as treatment in the first 2 days. On transfer to a tertiary center in Hong Kong, evolving neurologic manifestations of visual acuity and field deficits, confusion, and focal motor weaknesses were observed. Focal infarctions were evident in cerebral computed tomography in one patient and cortical lesions on magnetic resonance imaging in all three patients. [18F]Fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed additional decreased metabolism in the basal ganglia in two patients, which was typical of carbon monoxide poisoning. The neurologic deficits resolved completely at 3 weeks after the exposure, but psychologic symptoms succeeded. This report serves to alert clinicians to the varied neuro-ophthalmologic manifestations and psychologic impairment even with the same duration of carbon monoxide poisoning. PET might be more sensitive in detecting cerebral injuries specific for carbon monoxide poisoning. (J Child Neurol 2006;21:737—741; DOI 10.2310/7010.2006.00172).

SLE patients are at risk of tuberculosis (TB) infection due to both secondary immunosuppression and intrinsic defects in innate immunity. On the other hand infection can also perpetuate the development of an autoimmunity [1,2].