Engaging patients and parents to improve mental health intervention for youth with rheumatological disease.

BACKGROUND: Mental health disorders are common in youth with rheumatological disease yet optimal intervention strategies are understudied in this population. We examined patient and parent perspectives on mental health intervention for youth with rheumatological disease. METHODS: We conducted a mixed methods cross-sectional study, via anonymous online survey, developed by researchers together with patient/parent partners, to quantitatively and qualitatively examine youth experiences with mental health services and resources in North America. Patients ages 14-24 years with juvenile idiopathic arthritis, juvenile dermatomyositis, or systemic lupus erythematous, and parents of patients ages 8-24 with these diseases were eligible (not required to participate in pairs). Participants self-reported mental health problems (categorized into clinician-diagnosed disorders vs self-diagnosed symptoms) and treatments (e.g. therapy, medications) received for the youth. Multivariate linear regression models compared patient and parent mean Likert ratings for level of: i) comfort with mental health providers, and ii) barriers to seeking mental health services, adjusting for potential confounders (patient age, gender, disease duration, and patient/parent visual analog score for disease-related health). Participants indicated usefulness of mental health resources; text responses describing these experiences were analyzed by qualitative description. RESULTS: Participants included 123 patients and 324 parents. Patients reported clinician-diagnosed anxiety (39%) and depression (35%); another 27 and 18% endorsed self-diagnosed symptoms of these disorders, respectively. 80% of patients with clinician-diagnosed disorders reported receiving treatment, while 11% of those with self-diagnosed symptoms reported any treatment. Patients were less comfortable than parents with all mental health providers. The top two barriers to treatment for patients and parents were concerns about mental health providers not understanding the rheumatological disease, and inadequate insurance coverage. Over 60% had used patient mental health resources, and over 60% of these participants found them to be helpful, although text responses identified a desire for resources tailored to patients with rheumatological disease. CONCLUSION: Self-reported mental health problems are prevalent for youth in this sample with rheumatological disease, and obstacles to mental health treatment include disease-related and logistic factors. Strategies are needed to improve acceptance and accessibility of mental health intervention, including routine mental health screening and availability of disease-specific mental health resources.

Concern about malignancy contributes to the psychosocial toll of dermatomyositis.

Objective: Dermatomyositis (DM) is associated with increased morbidity and mortality, decreased quality of life, and an increased incidence of psychiatric illness. We performed a pilot study to investigate whether concern about malignancy contributes to the psychosocial toll of DM.Method: Patients with a recent DM diagnosis at our institution between 2013 and 2018 and no history of DM-associated malignancy completed standardized questionnaires and a novel survey generated by our group, and participated in focus groups.Results: Seventeen patients (14 females and three males) completed the surveys. The mean Dermatology Life Quality Index (DLQI) score was 2.75. The mean score on the Patient Health Questionnaire-9 (PHQ-9) was 5.35, with a range of 0-20. Our independent DM-specific questionnaire revealed a mean of 17.41 (range 2-40).Conclusion: Concern about the increased risk of malignancy contributes to the psychosocial toll of DM. Individual impacts are highly variable and patient specific. Clinicians should assess for depression and anxiety in patients with DM, understanding that concern for malignancy adds to the total psychosocial burden in some patients.

Parent Perspectives on Addressing Emotional Health for Children and Young Adults With Juvenile Myositis.

OBJECTIVE: To assess parent perspectives regarding the emotional health impact of juvenile myositis (JM) on patients and families, and to assess preferences for emotional health screening and interventions. METHODS: Parents of children and young adults with JM were purposively sampled for participation in focus groups at the Cure JM Foundation National Family Conference in 2018. Groups were stratified by patient age group (6-12, 13-17, and 18-21 years), and conversations were audiorecorded, transcribed verbatim, and co-coded via content analysis, with subanalysis by age group. A brief survey assessed preferences for specific emotional health interventions. RESULTS: Forty-five parents participated in 6 focus groups. Themes emerged within 2 domains: emotional challenges, and screening and interventions. Themes for emotional challenges comprised the impact of JM on: 1) patient emotional health, particularly depression and anxiety; 2) parent emotional health characterized by sadness, grief, anger, guilt, and anxiety; and 3) family dynamics, including significant sibling distress. Subanalysis revealed similar themes across age groups, but the theme of resiliency emerged specifically for young adults. Themes for emotional health screening and interventions indicated potential issues with patient transparency, several barriers to resources, the facilitator role of rheumatology providers, and preferred intervention modalities of online and in-person resources, with survey responses most strongly supporting child/parent counseling and peer support groups. CONCLUSION: JM is associated with intense patient and family distress, although resiliency may emerge by young adulthood. Despite existing barriers, increasing access to counseling, peer support groups, and online resources with rheumatology facilitation may be effective intervention strategies.

COVID-19 and myositis - unique challenges for patients.

OBJECTIVE: The COVID-19 pandemic and the subsequent effects on healthcare systems is having a significant effect on the management of long-term autoimmune conditions. The aim of this study was to assess the problems faced by patients with idiopathic inflammatory myopathies (IIM). METHODS: An anonymized eSurvey was carried out with a focus on effects on disease control, continuity of medical care, drug procurance and prevalent fears in the patient population. RESULTS: Of the 608 participants (81.1% female, median (s.d.) age 57 (13.9) years), dermatomyositis was the most frequent subtype (247, 40.6%). Patients reported health-related problems attributable to the COVID-19 pandemic (n = 195, 32.1%); specifically 102 (52.3%) required increase in medicines, and 35 (18%) required hospitalization for disease-related complications. Over half (52.7%) of the surveyed patients were receiving glucocorticoids and/or had underlying cardiovascular risk factors (53.8%), placing them at higher risk for severe COVID-19. Almost one in four patients faced hurdles in procuring medicines. Physiotherapy, critical in the management of IIM, was disrupted in 214 (35.2%). One quarter (159, 26.1%) experienced difficulty in contacting their specialist, and 30 (4.9%) were unable to do so. Most (69.6%) were supportive of the increased use of remote consultations to maintain continuity of medical care during the pandemic. CONCLUSION: This large descriptive study suggests that the COVID-19 pandemic has incurred a detrimental effect on continuity of medical care for many patients with IIM. There is concern that delays and omissions in clinical care may potentially translate to poorer outcomes in the future.

Being on the juvenile dermatomyositis rollercoaster: a qualitative study.

BACKGROUND: Juvenile Dermatomyositis is a rare, potentially life-threatening condition with no known cure. There is no published literature capturing how children and young people feel about their condition, from their perspective. This study was therefore unique in that it asked children and young people what is it like to live with Juvenile Dermatomyositis. METHODS: Data were obtained from fifteen young people with Juvenile Dermatomyositis, between eight and nineteen years of age from one Paediatric Rheumatology department using audio-recorded interpretive phenomenology interviews. Data were analyzed phenomenologically, using a process that derives narratives from transcripts resulting in a collective composite of participants shared experiences, called a 'phenomenon'. RESULTS: The overarching metaphor of a rollercoaster captures the phenomenon of living with Juvenile Dermatomyositis as a young person, with the ups and downs at different time points clearly described by those interviewed. The five themes plotted on the rollercoaster, began with confusion; followed by feeling different, being sick, steroidal and scared from the medications; uncertainty; and then ended with acceptance of the disease over time. CONCLUSION: Young people were able to talk about their experiences about having Juvenile Dermatomyositis. Our findings will aid clinicians in their practice by gaining a deeper understanding of what daily life is like and highlighting ways to enhance psychosocial functioning. Hopefully, this study and any further resulting studies, will raise understanding of Juvenile Dermatomyositis worldwide and will encourage health care professionals to better assess psychosocial needs in the future.

Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study / Análise da função sexual de pacientes com dermatomiosite e polimiosite através de questionários autoaplicados: um estudo transversal

Abstract Introduction: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM). Objective: To assess sexual function in female patients with DM/PM. Patients and methods: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Characteristics on sexual function were obtained by applying the questionnaires Female Sexual Quotient (FSQ) and Female Sexual Function Index (FSFI) validated for the Brazilian Portuguese language. Results: The mean age of patients was comparable to controls (32.7 ± 5.3 vs. 31.7 ± 6.7 years), as well as the distribution of ethnicity and socioeconomic class. As for gynecological characteristics, patients and healthy controls did not differ with respect to age at menarche and percentages of dysmenorrhea, menorrhagia, premenstrual syndrome, pain at mid-cycle, mucocervical secretion, and vaginal discharge. The FSQ score, as well as all domains of the FSFI questionnaire (desire, arousal, lubrication, orgasm and satisfaction), were significantly decreased in patients vs. controls, with 60.9% of patients showing some degree of sexual dysfunction. Conclusions: This was the first study to identify sexual dysfunction in patients with DM/PM. Therefore, a multidisciplinary approach is essential for patients with idiopathic inflammatory myopathies, in order to provide prevention and care for their sexual life, providing a better quality of life, both for patients and their partners.

Resumo Introdução: Até o presente momento, não há descrições na literatura da avaliação ginecológica e da função sexual em pacientes do sexo feminino com dermatomiosite (DM) e polimiosite (PM). Objetivos: Avaliar a função sexual em pacientes do sexo feminino com DM/PM. Casuística e métodos: Estudo transversal unicêntrico em que 23 pacientes (16 DM e sete PM), entre 18 e 40 anos, foram comparadas com 23 mulheres saudáveis, com a mesma faixa etária. As características sobre a função sexual foram obtidas por meio da aplicação dos questionários Female Sexual Quotient (FSQ) e Female Sexual Function Index (FSFI) validados para a língua portuguesa do Brasil. Resultados: A média de idade das pacientes foi comparável à dos controles (32,7 ± 5,3 vs. 31,7 ± 6,7 anos), assim como a distribuição de etnia e da classe socioeconômica. Quanto às características ginecológicas, pacientes e controles saudáveis não apresentaram diferenças em relação à idade na menarca e às porcentagens de dismenorreia, menorragia, síndrome pré-menstrual, dor no meio do ciclo, secreção mucocervical e corrimento vaginal. O escore de pontuação do FSQ, assim como todos os domínios do questionário do FSFI (desejo, excitação, lubrificação, orgasmo e satisfação), estavam significantemente diminuídos nas pacientes comparativamente com os controles, 60,9% das pacientes apresentavam algum grau de disfunção sexual. Conclusões: Este foi o primeiro estudo que identificou disfunção sexual nas pacientes com DM/PM. Assim, uma abordagem multidisciplinar é essencial para pacientes com miopatias inflamatórias idiopáticas para fornecer medidas de prevenção e cuidados para sua vida sexual e propiciar uma melhor qualidade de vida das pacientes e de seus parceiros.

Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study.

INTRODUCTION: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM). OBJECTIVE: To assess sexual function in female patients with DM/PM. PATIENTS AND METHODS: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Characteristics on sexual function were obtained by applying the questionnaires Female Sexual Quotient (FSQ) and Female Sexual Function Index (FSFI) validated for the Brazilian Portuguese language. RESULTS: The mean age of patients was comparable to controls (32.7±5.3 vs. 31.7±6.7 years), as well as the distribution of ethnicity and socioeconomic class. As for gynecological characteristics, patients and healthy controls did not differ with respect to age at menarche and percentages of dysmenorrhea, menorrhagia, premenstrual syndrome, pain at mid-cycle, mucocervical secretion, and vaginal discharge. The FSQ score, as well as all domains of the FSFI questionnaire (desire, arousal, lubrication, orgasm and satisfaction), were significantly decreased in patients vs. controls, with 60.9% of patients showing some degree of sexual dysfunction. CONCLUSIONS: This was the first study to identify sexual dysfunction in patients with DM/PM. Therefore, a multidisciplinary approach is essential for patients with idiopathic inflammatory myopathies, in order to provide prevention and care for their sexual life, providing a better quality of life, both for patients and their partners.

Work ability in patients with polymyositis and dermatomyositis: An explorative and descriptive study.

BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are rare, chronic inflammatory diseases leading to muscle weakness and low muscle endurance. The muscle weakness may lead to restrictions in daily activities and low health-related quality of life. OBJECTIVES: This study aimed to investigate the work situation, work ability, work-related risk factors, and influence of the physical and psycho-social work environment in patients with PM and DM. METHODS: Patients with PM/DM were assessed using the Work Ability Index (WAI), and the Work Environment Impact Scale (WEIS). RESULTS: Forty-eight patients (PM n = 25 and DM n = 23) participated (women/men: 29/19) with a mean age of 54 years (range 28-67 years, SD.10) and mean disease duration of nine years (SD.9). Forty-four percent worked full-time, 31% part-time and 25% were on full-time sick leave. More than 50% self-rated work ability as "poor" or "less good". Physically strenuous work components were present "quite to very often" in 23-79% and more in patients on sick leave ≥ 2 years. For those working, the interfering factors in the work environment concerned task and time demands. Supporting factors concerned meaning of work, interactions with co-workers and others. Self-rated work ability correlated moderately-highly positive with percentage of full-time employment, work-related risk factors and opportunities and constraints in the work environment. CONCLUSIONS: Poor self-rated work ability is common in patients with PM/DM indicating a need to identify interfering risk factors and support patients to enhance work performance.

Development of an internationally agreed minimal dataset for juvenile dermatomyositis (JDM) for clinical and research use.

BACKGROUND: Juvenile dermatomyositis (JDM) is a rare autoimmune inflammatory disorder associated with significant morbidity and mortality. International collaboration is necessary to better understand the pathogenesis of the disease, response to treatment and long-term outcome. To aid international collaboration, it is essential to have a core set of data that all researchers and clinicians collect in a standardised way for clinical purposes and for research. This should include demographic details, diagnostic data and measures of disease activity, investigations and treatment. Variables in existing clinical registries have been compared to produce a provisional data set for JDM. We now aim to develop this into a consensus-approved minimum core dataset, tested in a wider setting, with the objective of achieving international agreement. METHODS/DESIGN: A two-stage bespoke Delphi-process will engage the opinion of a large number of key stakeholders through Email distribution via established international paediatric rheumatology and myositis organisations. This, together with a formalised patient/parent participation process will help inform a consensus meeting of international experts that will utilise a nominal group technique (NGT). The resulting proposed minimal dataset will be tested for feasibility within existing database infrastructures. The developed minimal dataset will be sent to all internationally representative collaborators for final comment. The participants of the expert consensus group will be asked to draw together these comments, ratify and 'sign off' the final minimal dataset. DISCUSSION: An internationally agreed minimal dataset has the potential to significantly enhance collaboration, allow effective communication between groups, provide a minimal standard of care and enable analysis of the largest possible number of JDM patients to provide a greater understanding of this disease. The final approved minimum core dataset could be rapidly incorporated into national and international collaborative efforts, including existing prospective databases, and be available for use in randomised controlled trials and for treatment/protocol comparisons in cohort studies.

Improvement in the cutaneous disease activity of patients with dermatomyositis is associated with a better quality of life.

BACKGROUND: Cutaneous dermatomyositis (DM) disease activity is associated with decreased quality of life. OBJECTIVES: To assess if an improvement in quality of life, as measured by the Skindex-29 and patient-reported itch and pain on a 10-point visual analogue scale (VAS), correlated with an improvement in cutaneous DM disease activity. METHODS: Patients with a completed cutaneous DM disease area and severity index [Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI)] at two visits separated by at least 2 months were classified into responder (n = 15) and nonresponder (n = 30) groups according to the point change in the CDASI activity scores between visits. Responders had at least a four-point improvement in CDASI activity, indicating clinically relevant improvement. RESULTS: The change from baseline to the follow-up visit of the Skindex-29 subscale scores for the responders vs. the nonresponders were significantly different for emotions (P < 0·01), functioning (P < 0·01) and symptoms (P < 0·01). The change in VAS score between responders and nonresponders was also significant for itch (P = 0·01) and pain (P = 0·04). There was no significant difference between the groups in terms of disease subtype, sex, race, age, treatment for DM, smoking history or a history of malignancy within 5 years of a diagnosis of DM. CONCLUSIONS: This is the first study to demonstrate that the quality of life of patients with DM improved as their cutaneous disease activity decreased.

Aerobic training in persons who have recovered from juvenile dermatomyositis.

A recent study has shown that 36 persons who had recovered from juvenile dermatomyositis (JDM) have on average an 18% decrease in maximal oxygen uptake. The objective of this study was to investigate the effect of a 12-week aerobic training program in this group, and assess whether aerobic training can normalize aerobic capacity to the expected level for age and gender. The patients participating in the study, one male and nine females (16-42 years of age), were in remission from JDM, defined as no clinical or biochemical evidence of disease activity and no medical treatment for 1 year. The patients had a median disease duration of 3.4 years (1.4-10.3), a median treatment duration of 2.4 years (0.4-9.3) and a median duration of remission of 7.0 years (1.2-30.0). Patients trained at home on a cycle ergometer for 12 weeks at a heart rate interval corresponding to 65% of their maximal oxygen uptake (VO(2max)). VO(2max) and maximal workload (W(max)) were determined before and after the 12-week training period through an incremental cycling test to exhaustion. The patients served as their own controls. Eight patients with JDM in remission completed the 12-week exercise program; one patient completed 9 weeks out of the 12-week program and one dropped out of the study. Training increased VO(2max) and W(max) by 26% and 30% (P < 0.001). Creatine kinase (CK) levels were normal pre-training and did not change with training, reflecting no muscle damage. We also found that at a given workload, heart rate was lowered significantly after the 12-week training period, indicating an improvement in cardiovascular fitness. This study shows that 12 weeks of moderate-intensity aerobic training is an effective and safe method to increase oxidative capacity and fitness in persons who have recovered from JDM. The results indicate that the low oxidative capacity in JDM patients in remission is reversible and can be improved. Thus, we recommend frequent aerobic training to be incorporated into supervised physiotherapy sessions in the treatment of JDM patients in remission.

Introducing new tools for assessment of parent- and child-reported outcomes in paediatric rheumatology practice: a work in progress.

It is increasingly recognised that regular assessment of parent- and child-reported outcomes (PCROs) in routine paediatric rheumatology practice may help to increase the quality of care of children with rheumatic diseases. However, most of the instruments available for assessment of PCROs have remained essentially research tools and are not routinely administered in most centres. Recently, new multidimensional questionnaires for paediatric rheumatic diseases have been devised. These tools have been specifically designed for regular administration in a busy clinical setting and have the advantage over other clinical measures of incorporating all main PCROs in a single instrument. This review describes briefly the multidimensional questionnaires developed for the assessment of PCROs in children with juvenile idiopathic arthritis, juvenile dermatomyositis, and juvenile systemic lupus erythematosus and discusses the rationale underlying their creation. Furthermore, it illustrates the methodology and benefits related to the use of multidimensional questionnaires in the collection of standardised quantitative data.

[Prolonged corticosteroid-therapy and anxiety-depressive disorders, longitudinal study over 12 months]. / Corticothérapie prolongée et troubles anxieux et dépressifs. Étude longitudinale sur 12 mois.

UNLABELLED: To date, there is little data in the literature describing the anxiety and depressive disorders iatrogenic to corticosteroids. These disorders are common, underestimated, with potentially serious consequences that may jeopardize the patient's prognosis; their management is not consensual. OBJECTIVES: The objective of our work is to determine the prevalence of anxiety and depressive disorders induced by corticosteroids, assessing their accountability to the corticosteroids and studying their risk factors. METHODS: We conducted a prospective longitudinal study over 12months evaluating the prevalence of anxiety and depressive disorders in patients followed for chronic skin diseases treated with prolonged corticosteroid-therapy. Our patients were assessed using standardized instruments: the Mini International Neuropsychiatric Interview (MINI), the Hamilton Anxiety Scale (HAS) and the Beck Depression Inventory (BDI). RESULTS: Of 54 patients included, our study showed a high prevalence of anxiety and depressive disorders estimated at 27%. These disorders were divided into depressive disorder in 16% of cases, and anxiety disorder in 11% of cases. The early onset of these disorders was found during the first weeks of treatment. According to the Beck Depression Inventory (BDI), depression was moderate in 67% of cases; severe with suicide attempts in 22% of cases, and mild in 11% of cases. According to the Hamilton Anxiety Scale (HAS), anxiety was mild in 33% of cases and moderate in 67% of cases. The disorders observed were mainly distributed into: 33% deep pemphigus, 27% lupus, 13% bullous pemphigoid and 13% dermatomyositis. In this study the statistically significant risk factors are dose of corticosteroids and personal psychiatric history of the patient; in addition, there is a high prevalence of disorders in patients whose age exceeds 40 years, female gender, and patients treated for deep pemphigus. The evolution after pharmacological treatment and supportive psychotherapy was favorable in most patients. CONCLUSION: The psychiatric examination prior to prescription of long-term corticosteroid-therapy use should be standard practice to identify patients at risk, discuss the treatment modalities, and provide comprehensive care.

Exercise training in juvenile dermatomyositis.

OBJECTIVE: To investigate the effects of a supervised exercise training program on health parameters, physical capacity, and health-related quality of life in patients with mild and chronic juvenile dermatomyositis (DM). METHODS: This was a prospective longitudinal study following 10 children with mild and chronic juvenile DM (disease duration >1 year). The exercise program consisted of twice-a-week aerobic and resistance training. At baseline and after the 12-week intervention, we assessed muscle strength and function, aerobic conditioning, body composition, juvenile DM scores, and health-related quality of life. RESULTS: Child self-report and parent proxy-report Pediatric Quality of Life Inventory scores were improved after the intervention (-40.3%; P = 0.001 and -48.2%; P = 0.049, respectively). Importantly, after exercise, the Disease Activity Score was reduced (-26.9%; P = 0.026) and the Childhood Muscle Assessment Scale was improved (+2.5%; P = 0.009), whereas the Manual Muscle Test presented a trend toward statistical significance (+2.2%; P = 0.081). The peak oxygen consumption and time-to-exhaustion were increased by 13.3% (P = 0.001) and 18.2% (P = 0.003), respectively, whereas resting heart rate was decreased by 14.7% (P = 0.006), indicating important cardiovascular adaptations to the exercise program. Upper and lower extremity muscle strength and muscle function were also significantly improved after the exercise training (P < 0.05). Both the whole-body and the lumbar spine bone mineral apparent density were significantly increased after training (1.44%; P = 0.044 and 2.85%; P = 0.008, respectively). CONCLUSION: We showed for the first time that a 12-week supervised exercise program is safe and can improve muscle strength and function, aerobic conditioning, bone mass, disease activity, and health-related quality of life in patients with active and nonactive mild and chronic juvenile DM with near normal physical function and quality of life.

Quality of life in adults with juvenile-onset dermatomyositis: a case-control study.

OBJECTIVE: To compare quality of life in adults diagnosed with juvenile dermatomyositis (DM) with that of matched controls, and to analyze the association with other disease parameters in patients. METHODS: Thirty-nine patients with juvenile DM (ages ≥ 18 years) were clinically examined and compared with 39 age- and sex-matched controls. Global and health-related quality of life (HRQOL) were assessed by the Norwegian version of the Quality of Life Scale (QOLS-N) and the Short Form 36 (SF-36), respectively. For patients, disease parameters were assessed by the Disease Activity Score (DAS), Health Assessment Questionnaire (HAQ), and Myositis Damage Index (MDI). RESULTS: Compared to the controls, patients with juvenile DM assessed a median of 22.2 years (range 1.8-36.1 years) after disease onset had reduced HRQOL in general health (P = 0.009) measured by the SF-36. In patients, a moderate correlation was found between the physical component summary (PCS) score and the DAS (r(s) = -0.422) and MDI (r(s) = -0.381), and a strong correlation was found between the PCS score and the HAQ (r(s) = -0.516). There were no differences between patients and controls in the SF-36 mental component summary scores. Patients and controls had similar total scores of the QOLS-N, but differences existed within certain items. CONCLUSION: Adult patients with juvenile DM had, compared to controls, reduced HRQOL in general health measured by the SF-36, but not in the other subscales of the SF-36 or in global quality of life measured by the QOLS-N. An association was found between disease parameters and reduced HRQOL in the physical domains.