The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood:A randomised, single Centre parallel group trial (The Bendy Study)

Introduction: Joint hypermobility is common in childhood and can be associated with musculoskeletal pain and dysfunction. Current management is delivered by a multidisciplinary team, but evidence of effectiveness is limited. This clinical trial aimed to determine whether a structured multidisciplinary, multisite intervention resulted in improved clinical outcomes compared with standard care. Method: A prospective randomised, single centre parallel group trial comparing an 8-week individualised multidisciplinary intervention programme (bespoke physiotherapy and occupational therapy in the clinical, home and school environment) with current standard management (advice, information and therapy referral if deemed necessary). The primary endpoint of the study was between group difference in child reported pain from baseline to 12 months as assessed using the Wong Baker faces pain scale. Secondary endpoints were parent reported pain (100 mm visual analogue scale), parent reported function (child health assessment questionnaire), child reported quality of life (child health utility 9-dimensional assessment), coordination (movement assessment battery for children version 2) and grip strength (handheld dynamometer). Results: 119 children aged 5 to 16 years, with symptomatic hypermobility were randomised to receive an individualised multidisciplinary intervention (I) (n = 59) or standard management (S) (n = 60). Of these, 105 completed follow up at 12 months. No additional significant benefit could be shown from the intervention compared to standard management. However, there was a statistically significant improvement in child and parent reported pain, coordination and grip strength in both groups. The response was independent of the degree of hypermobility. Conclusion: This is the first randomised controlled trial to compare a structured multidisciplinary, multisite intervention with standard care in symptomatic childhood hypermobility. For the majority, the provision of education and positive interventions aimed at promoting healthy exercise and self-management was associated with significant benefit without the need for more complex interventions. Trial registration: The trial was registered prospectively with the national database at the Clinical Research Network (UKCRN Portfolio 9366). The trial was registered retrospectively with ISRCTN (ISRCTN86573140)

Clinical and laboratory phenotypes in juvenile-onset Systemic Lupus Erythematosus across ethnicities in the UK.

Funder: LUPUS UKSystemic lupus erythematosus (SLE) is a systemic autoimmune/inflammatory disease. Patients diagnosed with juvenile-onset SLE (jSLE), when compared to individuals with adult-onset SLE, develop more severe organ involvement, increased disease activity and greater tissue and organ damage. In adult-onset SLE, clinical characteristics, pathomechanisms, disease progression and outcomes do not only vary between individuals and age groups, but also ethnicities. However, in children and young people, the influence of ethnicity on disease onset, phenotype and outcome has not been investigated in detail. In this study, we investigated clinical and laboratory characteristics in pediatric SLE patients from different ethnic backgrounds (White Caucasian, Asian, Black African/Caribbean) accessing data from a national cohort of jSLE patients (the UK JSLE Cohort Study). Among jSLE patients in the UK, ethnicity affects both the disease's clinical course and outcomes. At diagnosis, Black African/Caribbean jSLE patients show more "classical" laboratory and clinical features when compared to White Caucasian or Asian patients. Black African/Caribbean jSLE patients exhibit more renal involvement and more frequently receive cyclophosphamide and rituximab. Studies targeting ethnicity-specific contributors to disease expression and phenotypes are necessary to improve our pathophysiological understanding, diagnosis and treatment of jSLE

Clinical and laboratory phenotypes in juvenile-onset Systemic Lupus Erythematosus across ethnicities in the UK.

Funder: LUPUS UKSystemic lupus erythematosus (SLE) is a systemic autoimmune/inflammatory disease. Patients diagnosed with juvenile-onset SLE (jSLE), when compared to individuals with adult-onset SLE, develop more severe organ involvement, increased disease activity and greater tissue and organ damage. In adult-onset SLE, clinical characteristics, pathomechanisms, disease progression and outcomes do not only vary between individuals and age groups, but also ethnicities. However, in children and young people, the influence of ethnicity on disease onset, phenotype and outcome has not been investigated in detail. In this study, we investigated clinical and laboratory characteristics in pediatric SLE patients from different ethnic backgrounds (White Caucasian, Asian, Black African/Caribbean) accessing data from a national cohort of jSLE patients (the UK JSLE Cohort Study). Among jSLE patients in the UK, ethnicity affects both the disease's clinical course and outcomes. At diagnosis, Black African/Caribbean jSLE patients show more "classical" laboratory and clinical features when compared to White Caucasian or Asian patients. Black African/Caribbean jSLE patients exhibit more renal involvement and more frequently receive cyclophosphamide and rituximab. Studies targeting ethnicity-specific contributors to disease expression and phenotypes are necessary to improve our pathophysiological understanding, diagnosis and treatment of jSLE

Management and treatment of children, young people and adults with systemic lupus erythematosus: British Society for Rheumatology guideline scope

Lay Summary: Systemic lupus erythematosus (SLE) is a lifelong condition in which the immune system damages the body’s own tissues, causing various symptoms including rashes, hair loss, mouth ulcers, joint pain and overwhelming tiredness. It can also affect major organs including the kidneys, heart, lungs and brain. SLE can present during childhood, but most commonly affects young and middle-aged females. It is approximately nine times more common in females than males. The disease can lead to disability, poor quality of life and even death in severe cases. Treatments can often be difficult to tolerate and can cause both short- and long-term side effects. Guidelines developed by the British Society for Rheumatology aim to provide guidance for diagnosing and treating people with SLE. This is necessary to ensure that the most up-to-date approach is followed, utilising the safest and most effective treatments. This article describes the plan for a guideline in SLE that is being updated to cover new evidence that has been published since 2017 relating to the treatment and management of SLE. The guideline will take a whole life course approach, from childhood to adulthood, and is being undertaken by a working group consisting of paediatric and adult rheumatologists and nephrologists, SLE experts, general practitioners, specialist nurses and other healthcare professionals, together with people with SLE and representatives from patient organizations. The guideline will be developed using the methods and processes outlined in the British Society for Rheumatology document ‘Creating Clinical Guidelines: Our Protocol’

Carpal-tarsal osteolysis with elbow involvement

Carpal-tarsal osteolysis is a rare condition that manifests as the progressive resorption of carpal and tarsal bones in young children. The diagnosis of this condition is often difficult and delayed as the initial clinical presentation is non-specific. Radiographic changes occur gradually, are often not seen at presentation and depend on recognising loss of bone in the ossification centres of the carpus and tarsus. MRI demonstrates morphological abnormalities in the cartilaginous, as well as the osseous components, of the developing carpal and tarsal bones and therefore may be helpful in predating the radiographic changes. Ultrasound appears to contribute little to the diagnosis and may be misleading. Exclusion of other conditions, particularly juvenile idiopathic arthritis, is important in making the diagnosis. MRI can be useful in excluding an inflammatory arthropathy, and suggesting the diagnosis of carpal-tarsal osteolysis

Sleep disturbances in HIV-infected patients associated with depression and high risk of obstructive sleep apnea

Objective: To evaluate sleep disturbances in a diverse, contemporary HIV-positive patient cohort and to identify demographic, clinical, and immune correlates. Methods: A convenience sample of 176 patients from a racially and ethnically diverse HIV-positive patient cohort in an urban population. This was a cross-sectional, epidemiologic study. We surveyed participants using multiple standardized instruments to assess depression, sleep quality, and risk for sleep apnea. We analyzed demographic, behavioral, and clinical correlates. Results: A total of 56% of participants were female, 75% Black and 64% had heterosexual HIV risk. The median age was 49 years. Poor sleep quality (Pittsburgh Sleep Quality Index > 5) was reported by 73% of patients and 52% met insomnia diagnosis criteria. A single question about self-reported sleep problems predicted a Pittsburgh Sleep Quality Index > 5 with a sensitivity and specificity of 82% and 81%, respectively. Female sex was significantly associated with higher risk of poor sleep quality, depression, and insomnia, whereas higher risk of obstructive sleep apnea was significantly associated with older age, male sex, obesity (body mass index ⩾ 30 kg/m 2 ), and metabolic comorbidities. High risk for obstructive sleep apnea, high rate of depression, and poor sleep hygiene represent treatment targets for sleep problems in HIV patients. Conclusion: Sleep disturbances were common in this patient cohort, although largely undiagnosed and untreated. Sleep problems are linked to worse disease progression and increased cardiovascular mortality. Screening for sleep problems with a single question had high sensitivity and specificity. In those patients with self-reported sleep problems, screening for obstructive sleep apnea, depression, and sleep hygiene habits should be part of routine HIV care