The COVID-19 Pandemic Highlights An Unmet Need To Improve Remote Assessment of Disease Activity in Rare Diseases, Juvenile-Onset Systemic Lupus Erythematosus And Juvenile Dermatomyositis

Here we report the results of a pilot young patient survey which targeted patients with juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM), which explored wellbeing, resilience and general concern about the COVID-19 pandemic. The survey was completed anonymously by patients who have been approached via the automatically generated hospital data base between June-December 2020. This pilot study found that generalised concerns regarding the COVID-19 pandemic were signicantly higher in females, although their self-reported disease activity scores were comparable with male respondents. Our ndings highlight a need to improve remote assessments to facilitate triaging for face-to-face visits in the situation of future pandemics associated with disruption of clinical services, as well as further research in this area of young patient-reported outcomes outside hospital visits.


Abstract
Here we report the results of a pilot young patient survey which targeted patients with juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM), which explored wellbeing, resilience and general concern about the COVID-19 pandemic. The survey was completed anonymously by patients who have been approached via the automatically generated hospital data base between June-December 2020. This pilot study found that generalised concerns regarding the COVID-19 pandemic were signi cantly higher in females, although their self-reported disease activity scores were comparable with male respondents. Our ndings highlight a need to improve remote assessments to facilitate triaging for face-to-face visits in the situation of future pandemics associated with disruption of clinical services, as well as further research in this area of young patient-reported outcomes outside hospital visits. Background: The impact of the COVID-19 pandemic on the delivery of healthcare for adolescents and young people should not be underestimated, even though this population is less clinically vulnerable to COVID-19 infection. Physiological, psychological and social-role changes during this period heavily in uence future well-being and health. The additional consequences of disrupted access to education, work and healthcare, and social and nancial stresses during the pandemic have had signi cant impact on mental health and well-being(1). Young people with chronic health conditions, already face a disproportionate psychosocial burden compared with their peers, and are likely to face additional worries and concerns regarding COVID-19, including heightened health-related anxiety, disrupted routines, reduced access to physical and psychosocial support (2).
As clinicians working in a specialist centre, we aimed to evaluate the impact of the COVID-19 pandemic in adolescents and young adults with juvenile-onset systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM). These are rare, complex autoimmune connective tissue disorders with a female preponderance, associated with multiple organ involvement and signi cant impact on quality of life. Here we report the results of a pilot survey, which explored wellbeing, resilience and concern about the COVID-19 pandemic in patients with JSLE and JDM.

Patient Cohort And Methods:
The survey was completed anonymously by patients between June-December 2020. The hospital digital database generated a complete list of patients with JDM and JSLE, who were under the care of Department for Adolescent Rheumatology at University College London Hospital in 2020. Automatic emails were sent to registered patient emails available in the database and patients of age 16 and over (who were legally able to consent at the time of inclusion in this exploratory survey) were invited to complete the survey anonymously accessed via a link to a RedCap questionnaire database. None of the patients have been approached to complete the questionnaire when attending clinics in person to minimise the potential selection bias. The survey has been approved by the local ethics committee (Ref. 20/HRA/2565).
Results were analysed using descriptive statistics (using R). The survey (available as Supplementary le) contained questions about patient's demographics (age, ethnicity, gender), employment and education status, disease duration, age at onset, as well as well-being or resilience, using the validated mood and feeling questionnaire (MFQ)(3), resilience scale(4) and the Warwick-Edinburgh mental well-being scales (5). The patients were also required to complete a VAS 0-100 scale to rate their general concerns regarding the general impact of COVID-19 on their lives.

Findings:
There were 63 respondents (53 females; 10 males) who answered the survey during the recruitment period. The survey had a 47% response rate, which is not unsurprising considering that patients have not been approached in person as many of the routine clinical assessments were conducted virtually. Re ecting the signi cant gender bias in JSLE and the higher prevalence of JSLE compared to JDM, there was a predominance of young females completing the survey (84%). There were no signi cant gender differences in the disease duration, age at diagnosis, disease duration, age of responders or immunosuppressive medication according to self-reported gender ( Table 1). The majority of patients were living outside London as we are a tertiary centre for adolescent and young adult rheumatology. Generalised concerns regarding the COVID-19 pandemic were signi cantly higher in females (p=0.007), although their self-reported disease activity scores were comparable with male respondents (p=0.205) [ Table 1, Figure 1A]. The self-reported reported resilience was almost identical in young men and women (p=0.99).
Linear regression analysis, adjusted for age, ethnicity and gender demonstrated that self-reported disease activity (using a global visual analogue scale) was the strongest determinant of concerns associated with COVID-19 (p<0.0003) ( Figure 1B), which was evident in both females (p= 0.003) and males (p=0.004). Longer disease duration was associated with general concerns regarding COVID-19 pandemic in males only (p=0.018).There was no association between COVID-19-associated concerns and employment and education status, or well-being or resilience, using the validated mood and feeling questionnaire (MFQ)(3), resilience scale(4) and the Warwick-Edinburgh mental well-being scales(5).

Conclusion:
To our knowledge, this is the rst focused evaluation of the impact of the COVID-19 pandemic on patients with JSLE and JDM. Our results demonstrate that gender differences should be considered when assessing patients, and that concerns regarding COVID-19 strongly correlate with patient-reported disease activity when accounting for other confounders. We were unable to compare patient-reported and physician-assessed disease activity, given restricted access to clinic appointments during the height of the pandemic. Previous psychology research has identi ed gender differences in wellbeing in healthy young people(6), while the most important predictors of well-being in emerging adulthood were higher self-con dence and lower negative self-evaluation (7), aspects that are likely to be affected by having to live with a chronic condition such as JSLE or JDM. In addition, well-being at the age of emerging adulthood is recognised as having signi cant impact on further psychological well-being (8); therefore addressing additional psychological stressors, such as general concern related to COVID-19 pandemic in young people with chronic conditions could have long term bene ts.
The main limitations of this exploratory survey are related to the relatively small sample size of this single centre study, which does not allow for generalization of results, and inability to establish any causal relationship between the outcomes measured. There are still uncertainties about the outcomes of COVID-19 infection/vaccination in children and young people with chronic in ammatory conditions, despite the re-assuring signals to date (9)(10)(11)(12); this is likely to be re ected in their general COVID-19associated concern.
Telemedicine gained traction during the pandemic and is likely to have a more permanent place in mainstream healthcare provision hereafter. Virtual consultations are particularly relevant for continuity of routine care for rare diseases in high-risk groups, including our cohort of JSLE and JDM, as patients often have to travel considerable distances to obtain tertiary expertise and may be shielding due to inherent and iatrogenic immunocompromise. There are recognised advantages and disadvantages of care delivery via virtual consultations for young people (13,14), in addition to the challenges of suffering from a complex autoimmune condition such as JSLE and JDM which have to be taken into account when designing virtual clinical services. Our ndings highlight a need to improve remote assessments to facilitate triaging for face-to-face visits, by developing standardised composite patient reported outcome measures for disease activity. Emerging digital technologies for self-assessment, monitoring and reporting of disease activity are likely to be important tools to empower young people and promote independence, as they transition to young adult services.   Figure 1 A First panel -Differences in COVID concern level (VAS 1-100, 100 being the highest concern regarding health-outcomes during COVID-19 pandemic) according to gender in patients with JSLE and JDM Second panel -Differences in self-reported disease activity (VAS 1-100, 100 being the highest self-perceived disease activity) according to gender in patients with JSLE and JDM B Linear regression analysis, adjusted for age, ethnicity and gender demonstrated that self-reported disease activity (using a global visual analogue scale) was the strongest determinant of concerns associated with COVID-19 pandemic.

Supplementary Files
This is a list of supplementary les associated with this preprint. Click to download. Surveyblank.pdf