2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non- Systemic Polyarthritis, Sacroiliitis, and Enthesitis

To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting as non- systemic polyarthritis, sacroiliitis, or enthesitis.Methods. The Patient/Population, Intervention, Comparison, and Outcomes (PICO) questions were developed and recined by members of the guideline development teams. A systematic review was conducted to compile evidence for the benefits and harms associated with treatments for these conditions. GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology was used to rate the quality of evidence. A group consensus process was conducted among the Voting Panel to generate the nal recommendations and grade their strength. A Parent and Patient Panel used a similar consensus approach to provide patient/caregiver preferences for key questions. Thirty- nine recommendations were developed (8 strong and 31 conditional). The quality of supporting evidence was very low or low for 90% of the recommendations. Recommendations are provided for the use of nonsteroidal antiinflammatory drugs, disease- modifying antirheumatic drugs, biologics, and intraarticular and oral glucocorticoids. Recommendations for the use of physical and occupational therapy are also provided. Specific recommendations for polyarthritis address general medication use, initial and subsequent treatment, and adjunctive therapies. Good disease control, with therapeutic escalation to achieve low disease activity, was recommended. The sacroiliitis and enthesitis recommendations primarily address initial therapy and adjunctive therapies. This guideline provides direction for clinicians, caregivers, and patients making treatment decisions. Clinicians, caregivers, and patients should use a shared decision- making process that accounts for patients' values, preferences, and comorbidities. These recommendations should not be used to limit or deny access to therapies.

Struggling in the Dark to Help My Child: Parents' Experience in Caring for a Young Child with Juvenile Idiopathic Arthritis.

PURPOSE: The purpose of this study is to describe parents' experiences in caring for 2-5-year-old children with juvenile idiopathic arthritis (JIA). DESIGN AND METHODS: A qualitative study using single-occasion in-depth interviews was conducted. Nine parents (eight mothers and one father) were interviewed in-person or via telephone. Data were analyzed using inductive content analysis. Methods used to protect the trustworthiness of study results included maintenance of an audit trail, peer debriefing, and member checks. RESULTS: The core construct Struggling in the Dark to Help My Child explained parents' experience in six domains: not knowing, trying to reach out in the dark, feeling my child's pain, working out the kinks to stay on top to manage, feeling drained by the whole process, and being hard on the entire household. Parents struggled with the unknown, searched for resources, witnessed their child's suffering without knowing how to help, and tried every possible way to stay on top of the child's illness and treatment, even when they felt drained physically and emotionally. JIA not only consumed their lives, but also affected the entire family, including the siblings and spouse, and the relationships among family members. CONCLUSION AND IMPLICATIONS: Findings highlight the day-to-day lived challenges parents face when caring for a young child with JIA. Healthcare providers including nurses need to assess the particular needs of an ill child and parents as well as the impact of the illness on the physical and psychosocial health of the entire family so that proper resources can be provided.

'My daughter's rheumatology nurse took away all of her fear'.

My daughter Isabella was diagnosed with juvenile arthritis at the age of one. She was unable to walk and in pain constantly.

The associations among economic hardship, caregiver psychological distress, disease activity, and health-related quality of life in children with juvenile idiopathic arthritis.

PURPOSE: To examine the associations among caregiver perceived economic hardship, psychological distress, children's disease activity, and health-related quality of life (HRQOL) in children with juvenile idiopathic arthritis (JIA). METHODS: Caregivers of 182 children with JIA (ages 2-18) attending the rheumatology clinics at the Montreal Children's Hospital and the British Columbia's Children Hospital completed a series of questionnaires on perceived financial hardship, caregiver psychological distress, and children's HRQOL at baseline, 6 and 12 months. Clinical information such as disease activity was obtained from medical charts. Statistical models were used to look at the significance of several factors of interest while controlling for possible confounders. RESULTS: Higher caregiver perceived economic hardship [(ß = 0.03, 95% CI = 0.005, 0.06), P = 0.02], psychological distress [(ß = 0.02, 95% CI = 0.006, 0.03), P = 0.004], and higher children's disease activity [(ß = 0.11, 95% CI = 0.07, 0.15), P < 0.0001] were associated with worse children's HRQOL. CONCLUSIONS: Findings suggest that caregiver financial hardship and psychological distress as well as children's disease activity may impact children's HRQOL. By providing psychological help to parents, offering information regarding financial resources in the community and by ensuring disease control, especially when the disease is severe, health providers may improve children's health outcomes.

Avaliação do estresse psicológico do cuidador primário do paciente com artrite idiopática juvenil / Evaluation of psychological stress in primary caregivers of patients with juvenile idiopathic arthritis

OBJETIVO: Avaliar o estresse psicológico do cuidador do paciente pediátrico com artrite idiopática juvenil (AIJ). MÉTODOS: Estudo observacional analítico transversal não controlado de 40 cuidadores de pacientes com AIJ, que foram avaliados pelo questionário Caregiver Burden Scale. Esta escala analisa cinco domínios do estresse: tensão geral, isolamento, decepção, envolvimento emocional e estresse causado por barreiras impostas pelo meio ambiente, graduando-os de 1 a 4. Os dados obtidos foram submetidos a análise estatística. RESULTADOS: Os cuidadores de pacientes com AIJ são principalmente do sexo feminino (87,5 por cento), casados (92,1 por cento) e com parentesco direto com o paciente (90 por cento). O grau de estresse é maior nos cuidadores de pacientes com AIJ forma poliarticular (p = 0,006), nos solteiros (p = 0,019) e naqueles do sexo feminino (p = 0,017). A dimensão analisada na qual se observou maior nível de estresse foi a de dificuldades relacionadas com o meio ambiente. CONCLUSÃO: Mulheres casadas e com parentesco direto com o paciente são os maiores cuidadores do paciente de AIJ. Cuidado com pacientes de forma poliarticular causa mais estresse do que o de pacientes de forma oligoarticular. Barreiras impostas pelo meio ambiente são responsáveis pelo maior índice de estresse nestes cuidadores.

OBJECTIVE: To assess psychological stress in primary caregivers of juvenile idiopathic arthritis (JIA) pediatric patients. METHODS: Uncontrolled cross-sectional analytical study of 40 caregivers of JIA patients. Caregivers were evaluated using the Caregiver Burden Scale, which analyzes five domains of stress on a scale of 1 to 4: general strain, isolation, disappointment, emotional involvement and strain caused by environmental barriers. The data were subjected to statistical analysis. RESULTS: Caregivers of JIA patients were mainly female (87.5 percent), married (92.1 percent) and close relatives (90 percent). Stress levels were higher in caregivers of polyarticular JIA patients (p = 0.006), single caregivers (p = 0.019) and female caregivers (p = 0.017). Environment-related difficulties were reported as the most stressful category by caregivers. CONCLUSION: Caregivers of JIA patients are usually married female relatives. Caring for polyarticular JIA patients is more stressful than caring for oligoarticular JIA patients. Strain caused by environmental barriers accounts for the highest levels of stress among the caregivers included in this study.

Evaluation of psychological stress in primary caregivers of patients with juvenile idiopathic arthritis.

OBJECTIVE: To assess psychological stress in primary caregivers of juvenile idiopathic arthritis (JIA) pediatric patients. METHODS: Uncontrolled cross-sectional analytical study of 40 caregivers of JIA patients. Caregivers were evaluated using the Caregiver Burden Scale, which analyzes five domains of stress on a scale of 1 to 4: general strain, isolation, disappointment, emotional involvement and strain caused by environmental barriers. The data were subjected to statistical analysis. RESULTS: Caregivers of JIA patients were mainly female (87.5%), married (92.1%) and close relatives (90%). Stress levels were higher in caregivers of polyarticular JIA patients (p = 0.006), single caregivers (p = 0.019) and female caregivers (p = 0.017). Environment-related difficulties were reported as the most stressful category by caregivers. CONCLUSION: Caregivers of JIA patients are usually married female relatives. Caring for polyarticular JIA patients is more stressful than caring for oligoarticular JIA patients. Strain caused by environmental barriers accounts for the highest levels of stress among the caregivers included in this study.

Sleep quality in children with juvenile rheumatoid arthritis.

Children with juvenile rheumatoid arthritis (JRA) report poor sleep quality, daytime sleepiness, fatigue, anxiety, and altered mood. Sleep disturbances in school-aged children are an issue of serious concern. Children are at an age when sleep is of primary importance to physical and intellectual growth, and sleep disturbances that begin in childhood may persist into adulthood. In this article we will review what is currently known about sleep in children with JRA, the influence of medications on sleep quality, the potential impact of poor sleep quality on daily life issues, and complementary/alternative modalities that may be effective in reducing sleep disturbances.

Prevention of nonadherence to nonsteroidal anti-inflammatory medications for newly diagnosed patients with juvenile rheumatoid arthritis.

Adherence to medications for chronic pediatric diseases decreases overtime. This randomized controlled trial evaluated a clinic-based, nurse-administered educational and behavioral intervention to prevent the anticipated drop in adherence to nonsteroidal medications among newly diagnosed patients with juvenile rheumatoid arthritis. Thirty-four participants completed the study (mean age = 8.44 years, SD = 3.96), including 19 in the experimental group and 15 in the standard-treatment (education) control group. There were significant group and Group x Time effects for adherence (assessed with an electronic monitor over a 13-month period) favoring the experimental group. In contrast, the groups did not differ significantly in disease activity or functional limitations. Factors that may have prevented detection of differences in these health parameters are dicussed.

Juvenile arthritis: a nursing perspective.

Juvenile arthritis is the fifth most common chronic illness in children. When a child is diagnosed with juvenile arthritis, the family must adjust in many ways in order to cope with the demands of this illness. This article provides an overview of the disease, presents the case history of two sisters with polyarticular arthritis, and discusses how nurses and other health team members can empower a family to manage more successfully with this disease and thereby enhance quality of life. Professional resources that aid families coping including educating the family about the disease, providing psychologic support, as well as knowledge and assistance for optimal physical functioning and maintaining energy reserves, and suggesting strategies to sustain self esteem, motivation and hope.

Enfermedad de Still en el adulto:Aspectos clínicos y evolución / Adult onset Still's disease

Se desciben las características clínicas, humorales y evolutivas de 7 casos de enfermedad de Still del adulto (ESDA), de los cuales cinco fueron mujeres,seguidos en prospectivo a fin de aportar datos sobre esta entidad inusual y señalar aspectos no suficientemente enfatizados.Criterios diagnósticos fueron los de Medsger y Cristy (Medecine Baltimore 63:82, 1984). Las edades de comienzo oscilaron entre los 18 y 48 años, con un seguimiento mínimo de dos años, excepto dos pacientes. Seis habían recibido tratamiento inicial con antibióticos y todos presentaron fiebre héctica, rash cutáneo,poliartritis,leucocitosiscon neutrofilia, anemia y eritrosedimentación elevada. Factor reumatoidea, anticuerpos antinucleares por inmunofluorescencia y crioglobulinas fueron negativos.Complemento hemolítico total fue normal. Los cambios radiológicos incluyeron lesiones no erosivas hasta la fecha.Seis presentaron lesiones no erosivas hasta la fecha.Seis presentaron hipergammaglobulinemia.Enzimas hepáticas fueron normales en tres.Seis presentaron faringitis y una serositis.Características inusuales fueron la objetivación de queratoconjuntivitis sicca en dos pacientes, otro con VDRL, reiteradamente positiva y dos con taquicardia sinusal persistente en ausencia de enfermedad o situación que la explicara.El curso de la enfermedad, al momento de inclusión en el trabajo fue: tres pacientes presentaban curso policíclico sitémico crónico articular,dos policíclico sistémico crónico articular,dos policíclico sistémico y dos monocíclico sistémico

Enfermedad de Still en el adulto:Aspectos clínicos y evolución / Adult onset Stills disease

Se desciben las características clínicas, humorales y evolutivas de 7 casos de enfermedad de Still del adulto (ESDA), de los cuales cinco fueron mujeres,seguidos en prospectivo a fin de aportar datos sobre esta entidad inusual y señalar aspectos no suficientemente enfatizados.Criterios diagnósticos fueron los de Medsger y Cristy (Medecine Baltimore 63:82, 1984). Las edades de comienzo oscilaron entre los 18 y 48 años, con un seguimiento mínimo de dos años, excepto dos pacientes. Seis habían recibido tratamiento inicial con antibióticos y todos presentaron fiebre héctica, rash cutáneo,poliartritis,leucocitosiscon neutrofilia, anemia y eritrosedimentación elevada. Factor reumatoidea, anticuerpos antinucleares por inmunofluorescencia y crioglobulinas fueron negativos.Complemento hemolítico total fue normal. Los cambios radiológicos incluyeron lesiones no erosivas hasta la fecha.Seis presentaron lesiones no erosivas hasta la fecha.Seis presentaron hipergammaglobulinemia.Enzimas hepáticas fueron normales en tres.Seis presentaron faringitis y una serositis.Características inusuales fueron la objetivación de queratoconjuntivitis sicca en dos pacientes, otro con VDRL, reiteradamente positiva y dos con taquicardia sinusal persistente en ausencia de enfermedad o situación que la explicara.El curso de la enfermedad, al momento de inclusión en el trabajo fue: tres pacientes presentaban curso policíclico sitémico crónico articular,dos policíclico sistémico crónico articular,dos policíclico sistémico y dos monocíclico sistémico