The term ‘lupus erythematosus’ refers to a range of related disorders. As many as 70–80% of lupus patients will develop skin lesions (abnormal patches) at some point during the course of their disease. Types of lupus affecting the skin are collectively known as cutaneous lupus erythematosus (CLE). The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a tool for classifying how severe the skin symptoms are in people with CLE. Being able to accurately and reliably measure skin lesions is an important way to monitor any improvement or worsening of the disease, which is useful in clinical trials to see if a treatment is working. It is already known that the CLASI is a reliable (accurate) measure in adults, but researchers based in the USA conducted this study to validate the reliability of the CLASI in the pediatric (child) population, where CLE can appear differently than in adults. This validation will allow clinical trials to reliably assess treatment efficacy in CLE. The researchers recruited 11 pediatric patients with active CLE, 6 dermatologists, and 6 rheumatologists to attend a one-day event at the University of Pennsylvania. Physicians were trained to use the CLASI as well as another scoring system called the Physician Global Assessment (PGA) to allow for comparison. Physicians (doctors) individually rated all patients using both tools. Each physician reassessed two randomly selected patients. CLASI proved to be a reliable and valid measurement tool and superior to the PGA for pediatric CLE, and the researchers conclude that it can be used in future clinical trials.
The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a reliable outcome measure for cutaneous lupus erythematosus (CLE) in adults used in clinical trials. However, it has not been validated in children, limiting clinical trials for paediatric CLE. This study aimed to validate the CLASI in paediatrics. Eleven paediatric patients with CLE, six dermatologists and six rheumatologists participated. The physicians were trained to use the CLASI and Physician's Global Assessment (PGA), and individually rated all patients using both tools. Each physician reassessed two randomly selected patients. Within each physician group, the intraclass correlation coefficient (ICC) was calculated to assess the reliability of each measure. CLASI activity scores demonstrated excellent inter‐ and intrarater reliability (ICC > 0·90), while the PGA activity scores had good inter‐rater reliability (ICC 0·73–0·77) among both specialties. PGA activity scores showed excellent (ICC 0·89) and good intrarater reliability (ICC 0·76) for dermatologists and rheumatologists, respectively. Limitations of this study include the small sample size of patients and potential recall bias during the physician rerating session. CLASI activity measurement showed excellent inter‐ and intrarater reliability in paediatric CLE and superiority over the PGA. These results demonstrate that the CLASI is a reliable and valid outcome instrument for paediatric CLE.
The term ‘lupus’ refers to a range of related disorders. Discoid lupus erythematosus (DLE) is a form of lupus in the skin, which is rare in children. It can lead to disfiguring scarring. Some patients develop systemic lupus (SLE), a type of lupus that affects the whole body, which can lead to damage to multiple organs. Small studies have suggested that 25 to 30% of children with DLE develop SLE over time, but risk factors for this are not known. Early diagnosis and treatment of SLE are helpful. This study used a survey approach to compare practice patterns between two medical specialties, academic rheumatologists and dermatologists, in the United States and Canada, caring for children with DLE. The study aimed to identify areas of agreement but also practice-based differences between groups. Consensus (agreement) occurred when 70% or more of both specialties agreed. The authors found consensus that certain laboratory studies should be checked for all children at diagnosis of DLE. Both groups agreed that the presence of “other auto-antibodies besides ANA”, arthritis, or nephritis were high-risk features for SLE that should increase screening for systemic disease. There were no other agreed-upon risk factors for SLE, including those that have previously been shown in adults with DLE. Both groups agreed that first-line systemic therapy (whole body treatment) for widespread DLE should be hydroxychloroquine. The authors could not agree on which medications to use for resistant skin disease. Overall, the study found some areas of agreement but many areas of practice-based difference. More data in children with DLE is necessary to determine best practice approaches. The authors are completing a type of study called a retrospective cohort study that may help to fill these gaps.
Janus kinase inhibition (JAKi) has been proposed as a treatment for idiopathic inflammatory myopathies to target increased interferon signalling. Predominantly retrospective reports have demonstrated effectiveness of JAKi in refractory juvenile dermatomyositis (JDM). However, JAKi remains an off-label treatment for JDM and there may be variation in use worldwide. An international survey was conducted to investigate approaches to JAKi for JDM. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Therapeutics workgroup and core members of the Paediatric Rheumatology European Society (PReS) JDM working party devised an electronic survey to assess the use of JAKi in JDM. CARRA and PReS members were invited by email to complete the survey. There were 229 respondents (18%), with 50% from the US and 29% from Europe. 150 had used JAKi for over 450 patients with JDM; among them, 77% noted clinical improvement in most or all patients and 17% reported side effects. The highest ranked perceived barriers to JAKi use were lack of clinical data and inability to obtain insurance approval. The highest ranked clinical indications for starting JAKi were refractory skin disease, refractory muscle disease, inability to wean steroids, and intolerance to other steroid-sparing agents. Pediatric rheumatologists use JAKi off-label treatment for refractory JDM. Most providers noted clinical improvement in their patients. Barriers to JAKi use include lack of clinical data and insurance coverage. Clinical trials are needed to provide better data on the efficacy and safety of JAKi.
ABSTRACT Although several studies have provided positive evidence concerning the impacts of technology‐mediated language learning on learners’ vocabulary acquisition, few studies have investigated concept mapping in this field. This study intended to examine the impacts of guided and unguided mobile‐mediated concept mapping on learners’ vocabulary acquisition outside of English as a foreign language (EFL) classrooms. Moreover, the vocabulary acquisition strategies of students were examined. Sixty EFL learners in three intact classes were assigned to guided and unguided mobile‐mediated concept mapping conditions as well as one control group. The participants in the guided mobile‐mediated concept mapping group were provided with fill‐in‐blank concept mapping of words, and they were asked to fill in the nodes through their smartphones. The participants of the unguided mobile‐mediated concept mapping group were asked to draw concept maps of words via their mobile phones without receiving fill‐in‐blank concept mapping. The control group's participants were required to understand and memorize the words given to the experimental conditions conventionally, and they did not receive mobile‐mediated concept maps. Analyses of the learners’ post‐ and also delayed posttests indicated that both guided and unguided mobile‐mediated concept mapping were equally beneficial for increasing EFL learners’ vocabulary learning. Moreover, findings revealed that guided and unguided mobile‐mediated concept mapping promoted learners’ vocabulary learning strategy awareness outside of EFL settings.
Children with discoid lupus erythematosus (DLE) are at risk for disfigurement and progression to systemic lupus erythematosus (SLE). Consensus is lacking regarding optimal care for children with DLE. The aim of this study was to compare practice patterns among paediatric dermatologists/rheumatologists treating paediatric DLE. An online survey was sent to 292 paediatric rheumatologists in the Childhood Arthritis and Rheumatology Research Alliance and 200 paediatric dermatologists in the Pediatric Dermatology Research Alliance. Consensus was defined as ≥ 70% agreement. Survey response rates were 38% (76 of 200) for dermatology and 21% (60 of 292) for rheumatology. Both specialties agreed that screening labs should include complete blood counts with differential, urinalysis, complement levels, erythrocyte sedimentation rate, antinuclear antibody and other autoantibodies, hepatic function and renal function/electrolytes. Both specialties agreed that arthritis or nephritis should prompt intensified evaluation for SLE. No other patient features achieved consensus as disease‐modifying risk factors. Hydroxychloroquine was agreed upon as first‐line systemic therapy, but consensus was lacking for second‐ or third‐line treatment. We found few areas of consensus and significant practice differences between paediatric dermatologists and rheumatologists treating DLE. Knowledge gaps include risk factors for SLE, optimal screening and treatment of refractory skin disease.
Mental health conditions are common in youth with systemic lupus erythematosus (SLE), yet intervention strategies are understudied. We used a patient-engaged approach to investigate the mental health needs of youth with SLE.
Methods
An anonymous online survey examined beliefs and experiences with mental health for youth with SLE. Eligible youth ages 14–24 years had a diagnosis of SLE and reported specific treatment for the condition. Parents of youth 8–24 years meeting the above criteria were also eligible to participate. The survey was developed in collaboration with patient and parent advisors, the Childhood Arthritis and Rheumatology Research Alliance (CARRA), and the Patients, Advocates, and Rheumatology Teams Network for Research and Service (PARTNERS). Participants were recruited through the Lupus Foundation of America and CARRA clinics. We tabulated youth responses for i) self-reported prevalence of mental health problems, categorized into mutually exclusive clinician-diagnosed disorders and self-diagnosed symptoms, and ii) mean Likert ratings (0=low, 4=high) for the impact of disease related-factors on their mental health. We also compared youth and parent responses using regression models to examine comfort level with potential mental health providers.
Results
102 respondents included 59 patients (58%) and 43 (42%) parents. Youth had a mean age of 20.9 (standard deviation, SD=3.4) years, and mean disease duration of 6.9 (SD 4.0) years. History of a mental health problem was reported by 21 youth (36%), of which 66% said that their rheumatologist was unaware. Clinician-diagnosed anxiety was reported by 19%, depression by 12%, and adjustment disorders by 19%; another 17%, 8% and 10% had self-reported symptoms of these disorders, respectively. Mean Likert ratings by youth indicated that disease aspects most impacting mental health were worry about disease impact on the future at 3.0 (SD 1.2), worry about having a flare at 2.9 (1.2), and worry about medication side effects at 2.8 (1.3). Youth and parents felt most comfortable discussing mental health concerns with rheumatologists and primary care providers, and least comfortable with social workers and school counselors (figure 1).
Conclusions
Youth with SLE have high rates of diagnosed and undiagnosed mental health problems, which are impacted by their disease. Mental health intervention strategies in rheumatology settings may improve mental health education, screening and treatment for these youth.
Funding Source(s):
The Childhood Arthritis and Rheumatology Research Alliance
