Experiences and Perspectives of Children and Young People Living with Childhood-Onset Systemic Lupus Erythematosus—An Integrative Review

Childhood-onset systemic lupus erythematosus (cSLE) impacts the daily life of children and young people. This study aimed to describe the experiences and perspectives of children and young people living with cSLE. An integrative review guided by Whittemore and Knafl was conducted. Extant empirical research published in peer-reviewed journals from 2000 to 2021 on children’s self-reported experiences living with cSLE was identified from Scopus, CINAHL, Medline via PubMed, and PsycINFO via Ovid databases. Nineteen studies involving over 1400 participants were included. Four themes and fourteen sub-themes were identified: (1) challenging symptoms (disruptions to life and altered self, severity, fatigue, depression, and anxiety), (2) medicines and side effects (dreaded steroids, conflicting feelings, and medication adherence), (3) complicated life (school sports and social, giving things up, lack of understanding, and quality of life) and (4) ways of coping (family and friends, relationships with health providers, and maintaining positivity). While cSLE shares many similarities with adult-onset SLE, awareness of differences in experiences and perceptions of children and young people is crucial. The significant psychological and social impact of the disease and its treatments necessitates a comprehensive, holistic approach to managing cSLE that considers the unique needs of youth.


Introduction
Childhood-onset systemic lupus erythematosus (cSLE) is a rare multi-system autoimmune disease that is defined by disease onset before the age of 18 years [1]. It affects approximately 15-20% of SLE patients and has a variable presentation and clinical course [2,3]. Incidences of cSLE are reported as between 0.36 and 2.5 per 100,000 children, with prevalence ranging from 1.89 to 34.1 per 100,000 [4][5][6][7]. Similar to adult onset (aSLE), cSLE is clinically complex, heterogeneous, and characterized by periods of flare and remission [8]; however, cSLE is much more aggressive than aSLE with higher disease activity, more severe organ manifestations, and subsequently increased medication burden [9][10][11]. Childhoodonset systemic lupus erythematosus most commonly affects the skin (including malar and discoid rash), muscles, and joints; however, involvement of major organs is common, with renal inflammation occurring most frequently [2,12]. Other non-specific symptoms of fatigue, pain, weight loss, and fever are frequent and result in significant morbidity [10].
Research in aSLE has examined experiences and outcomes through the analysis of barriers and facilitators, exploration of beliefs, illness perception, and treatment adherence [13][14][15][16]. In addition, studies describe how disease activity, anxiety, social support, sleep quality, and fatigue negatively impact quality of life (QOL) in aSLE [17,18]. In cSLE, much of the focus has been on treatment targets [8], adherence [19,20], health literacy
Focus groups.
Body changes and uncertainty about progressions of disease most impactful. SLE impacts on daily life and relationships. Medication compliance is challenging. Feeling of responsibility for parents' wellbeing.
Limitations include a small sample with only 1 of the three families meeting our age criteria and poorly reported methodology, and unconventional approach to reporting. Strengths include rich data from narratives.
[38] USA Aim: To estimate the health-related quality of life (HRQOL) of children with childhood-onset systemic lupus erythematosus (cSLE) and compare it to that of normative cohorts; to assess the relationship of HRQOL with cSLE disease activity and damage; and to determine the effects of changes of disease activity on HRQOL. Quantitative study.
Health-related quality of life (HRQOL) Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), the Rheumatology Module (PedsQL-RM), the Child Health Questionnaire (CHQ), and the British Isles Lupus Activity Group Index (BILAG) was used to measure organ-system-specific disease activity. Physicians rated the course of cSLE between visits.
Active disease, the worsening of disease, and the presence of disease damage are all risk factors for poor HRQOL in cSLE. Children with cSLE have markedly lower CHQ-P50 scores than healthy children. Patients with cSLE have much lower scores on the PedsQL-GC.
Control of disease is an important way to improve HRQOL in cSLE. Disease activity has a differential effect on HRQOL in cSLE, depending on the organ system involved. Lower HRQOL with the presence of Raynaud's phenomenon.
Limitations include ratings of the change in cSLE patients' health consistently from the patient were not collected. Thus, for the analysis, these ratings were simply combined.  The Patient Health Questionnaire-9 (PHQ-9) measured depression, the Medication Adherence Self-Report Inventory (MASRI) measured medication non-adherence, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI).
Positive depression screen in this study population of patients with cSLE was high (59%); the high rate of reported suicidal ideation-Nearly one-quarter (23%) of participants in this study who had a positive depression screen reported suicidal ideation at the time of the screen; patients who reported medication non-adherence in this study were more likely to have longer disease duration than those who reported medication adherence; prevalence of medication non-adherence in this study population of patients with cSLE was also notable with 20% of the participants reporting less than 80% adherence.
Limitations include a small sample from one hospital. The tools used to define the exposure and outcome (PHQ-9 and MASRI, respectively) each had inherent limitations. The study has demonstrated the feasibility of administering. depression and medication non-adherence screens within a clinical setting. Using clinically relevant cut-offs for fatigue and depressive symptoms, patients were assigned to Low (n = 27) or High Risk (n = 23) groups. At visits 1 and 2, respectively, clinically relevant fatigue was present in 66% and 56% of patients. Clinically significant depressive symptoms in 26% and 24%; clinically significant anxiety in 34% and 28%. Poorer health-related quality of life at follow-up was significantly predicted by higher fatigue and depressive symptoms at the initial visit.
Limitations include that most participants were adolescents recruited from 1 hospital, which may limit generalizability of the findings for younger children, and correction for multiple comparisons in the data analysis was not undertaken. This was the first study to identify potentially modifiable predictors of impaired HRQoL in cSLE children/adolescents. Adolescent (n = 10) insurance cover-public cover (n = 6), private cover (n = 4). Adolescent PROMIS fatigue score (range) 57.5 (30.3-74.4). Adolescent Child Pain VAS mean (range) 3 (0-8). Adolescent number of medications (SD) 4.6 (2.5). Young adult (n = 12) insurance cover-public cover (n = 6), private cover (n = 6). Young adult PROMIS fatigue score (range) 57.4 (33.1-72.4). Young adult number of medications (SD) 3.5 (1.3). Themes included knowledge deficits about cSLE, symptoms limiting daily function, specifically mood and cognition/learning, barriers, facilitators of adherence, worry about the future, symptoms limiting daily functioning, pain/fatigue, self-care and management, impact on personal relationships, and health care provider communication and relationship.
Limitations include the sample being one of convenience and from 1 hospital and being predominantly Caucasian, which does not capture the ethnic groups with the highest morbidity. Strengths include using a design that used a quantitative and qualitative approach.  Adolescents with SLE ages 15-18 years (n = 9) (7 females and 2 males), from 1 hospital.
Varied interpretation and understanding about what lupus is/does to the body. Lupus and cancer seen as having similar consequences. Sense of guilt and self-blame re: diagnosis, blaming external factors for SLE such stress or food, invisible and visible forms of lupus, and taking responsibility and action for looking after self. Healthcare staff minimized symptomsinteractions and information sharing is key to learning and taking responsibility. Life with lupus is complicated and limiting-differences from others-but creates growth and sense of responsibility.
Limitations include a small sample size, and methodological underpinnings were poorly described. Strengths include the results being clearly presented with good use of quotes. [

45] China
Aim: To examine the relationship between physical appearance concern and psychological distress in female adolescent patients with systemic lupus erythematosus.
Quantitative study.
Appearance concern was assessed by the physical appearance domain of the multi-dimensional Self Perception Profile for Children (SPPC). The SLEDAI was used to assess disease activity. Depression-the CDI covers negative mood, interpersonal difficulties, negative self-esteem, ineffectiveness, and inability to experience pleasure.
CDI: Nearly all patients showed increased depressive symptoms, as indicated by the mean scores on the CDI. The total mean CDI was 18.5 ± 4.3, indicating that these patients were experiencing depressive symptoms. Furthermore, a total of 32 (38.1 %) patients had CDI larger than 19 points. Among the CDI subscales, negative mood, negative self-esteem, and anhedonia contributed to the total scores mostly. Physical Appearance: Regarding the concern about appearance, 77 (91.7 %) patients with SLE reported that they felt unattractive due to the disease, according to the questions from the SPPC. Among the SLE patients, the SPPC physical appearance score was 13 ± 2.8, indicating that the SLE adoles cents believe their appearances were severely impaired. Appearance concern was highly correlated with depression (r 0.758, p 0.001). Subsequently, age was moderately correlated with depression (r 0.468, p 0.001). Other variables, such as the number of admissions and disease duration, were partially correlated with depression.
Limitations include the use of a generic measure for physical appearance evaluation and is less robust than a more comprehensive one and less effective than a SLE-specific evaluation tool. The self-administered evaluation of depression through the CDI could have failed to capture the whole depressive disorders. The authors failed to evaluate appearance concerns and psychological conditions of the adolescents before the SLE onset because the patients who received these questionnaires had been treated with an average of 15.8 months, and male adolescents were excluded.  The BPI and pain-VAS were highly correlated and also featured similar correlations to the remaining variables. only. HRQOL measured by PedsQL-GC was significantly impacted by fatigue and pain (R 2 5 0.75, p, 0.001), with fatigue predicting 42% and pain predicting 33% of the model variance, but pain coping, anxiety, pain catastrophizing, and depression did not significantly predict PedsQL-GC scores. The PedsQL-RM was significantly impacted by pain, fatigue, and anxiety (R 2 5 0.71, p, 0.001), with pain predicting 33%, fatigue predicting 25%, and anxiety predicting 7% of model variance, but pain coping, pain catastrophizing, and depression did not significantly predict PedsQL-RM scores. Both support the notion that pain, fatigue, and to a lesser extent anxiety account for significantly diminished HRQOL observed in childhood-onset systemic lupus erythematosus patients.
Limitations include the majority of the patients being teenagers, the sample size being small, and the design being cross-sectional. Strengths include the patients studied were well-phenotyped and are representative of those followed at the hospital. In cohort 1 10 patients had elevated depression scores (26%), and three (8%) subjects had clinically significant depression scores (T score 65). No subject endorsed suicidality. In cohort 2, seven (44%) of the young adults had elevated depression scores (10), with two (12.5%) subjects scoring in the moderate to severe depressive symptoms range (>19). Similarly, no subject endorsed suicidality. Symptoms receiving the highest mean ratings by cohort one included fatigue, school problems, indecisiveness, despair, and sleep disturbances. The most severe individual depressive symptoms endorsed by cohort two included sleep disturbances, loss of energy, fatigue, changes in appetite, and indecisiveness.
Limitations include a small sample size, allowing for hypothesis generation and potentially limiting our ability to detect differences even if they were present and data collection was cross-sectional.
[ Mean school domain scores for children of the PedsQLTM generic report were lower compared with total and subscale scores. Patients reported difficulty with schoolwork, had problems with memory and concentration, and were sad about the effect of SLE on schoolwork and attendance. Eighty-three percent of patients felt that they would have done better in school if they did not have SLE. Moderate correlations (r = 0.3-0.4) were found between SMILEY_ total score and the following items: satisfaction with school performance, interest in schoolwork, remembering what was learned, and concentrating in class. Patients on intravenous chemotherapeutic medications missed more school days (p < 0.05) compared with patients on oral medications.
Limitations include potential recall bias among patients and the need for a larger sample so children's responses could be stratified by age. The strengths include data collected across two centers and the use of validated tools. Interviews and written responses.
Limitations of SLE are physical, social, and psychological. Impact on daily life, on social and family relationships. Impact on self-related to medication side effects and disease effects. Sadness about diagnosis-rationalizing and normalizing as coping mechanisms. Stress and anxiety about the future.
Limitations include a small sample, poorly described methodology, and data were collected from 1 center. The progressive decline of HRQL with increasing disease activity and accumulated damage effect more pronounced in both instances on the physical than in the psychosocial health domain. Greater impairment of HRQL occurred in patients with active disease in the central nervous, renal, and musculoskeletal systems. Active nephritis and seizure most significantly affected family life (PE, PT, and FA). Lupus headache was the only disease manifestation that impaired Mental health. Pleurisy and fever had a more significant impact, respectively, on BP and CH. Marked reduction of self-esteem associated with renal disease. HRQL abnormalities were more associated with clinical features than with laboratory abnormalities.
Limitations include the use of a cross-sectional design where it is difficult to determine a causal relationship since there is doubt about the timing of when a child is in remission or in the middle of an exacerbation of SLE.
[52] United Kingdom Aim: To explore in depth the views of CSLE patients and parents on potential treatment targets (e.g., LLDAS), outcome measures (e.g., HRQOL and fatigue measures), and study designs being considered by TARGET LUPUS in light of their previous treatment and care. Qualitative study.
Variation in treatment response. Symptoms that represented lupus low disease activity state (LLAS) were variable and different from adults. Intolerable visible signs of lupus-steroids are undesirable-symptoms-medicine side effects-fatigue is a significant symptom-wanted to minimize disruption from CSLE in all aspects of the patient's life and preferred treatment goals to include corticosteroid dose reduction, HRQOL, and fatigue in addition to the targeting of disease activity.
Strengths include the methods section being clear and concise. Good inclusion of quotes from participants and data collected from eight centers. Semi-structured interviews.
Adjusting and creating a new normal that included medicines-contending with the side effects of medications was challenging-understanding the 'why' encouraged adherence. Participants felt that viewing a graphical model of their blood test results over their disease courses provided clearer representations of how medications or the lack of such influenced their conditions over time. Avoiding hospitalization and being sick were also goals that the participants strived toward, further increasing their medication-taking motivations. Participants resented doctors' lack of transparency when they explained the medications' side effects. Taking steroids was of the greatest concern because these medications made them gain weight.
Limitations include inconsistencies with reporting participant numbers. Strengths include a methodologically sound study that was well described.
[54] Australia Aim: To describe the experiences, perspectives, and health care needs of adolescents and young adults diagnosed with SLE prior to age 18 years. Qualitative study-grounded theory.
Focus groups and interviews.
Being treated differently, reluctance to disclose. Poor self-image felt different from peers. Physical manifestations of disease and medication side effects impacted negatively on the image of self. Isolation and stigma. Symptoms impacted future aspirations (jobs, parenthood, study). Lack of age-appropriate information-uncertainty about the future-Knowledge of SLE was mixed. Desire for autonomy and developing self-reliance. Positive side to SLE: greater confidence and strength of character. Successful management relied on family and friends. Relationship with clinicians-important to have trusted relationships-some talked about being 'judged'.
Limitations include some methodological confusion in the description of the analysis approach undertaken. Peer victimization, self-esteem, depression, anxiety, and QOL levels of the patients with SLE were not worsened than those of the control group. Peer victimization and trait anxiety levels have roles in determining the QOL impairment of children and adolescents with SLE. The difference in levels of peer victimization between the patients and controls was not statistically significant. SLE patients with lower disease activity may have lower depression and anxiety levels compared with those with higher disease activity.
Limitations include data collection being from 1 hospital, a structured psychiatric interview was not performed, and a disease-specific questionnaire for measuring QOL was not used. We identified four themes and fourteen sub-themes: (1) challenging symptoms (disruptions to life and altered self, severity, fatigue, depression, and anxiety), (2) medicines and side effects (dreaded steroids, conflicting feelings, and medication adherence), (3) complicated life (school sports and social, giving things up, lack of understanding, and quality of life) and (4) ways of coping (family and friends, relationships with health providers, and maintaining positivity). See Figure 2. Key themes representing young people's experience of living with cSLE. Selected quotations to illustrate each sub-theme and the articles where the sub-themes were evident are provided in Table 3. Illustrative Quotes.

Challenging Symptoms
Disruption to Life and Altered Self. The journey to a diagnosis was the first disruption experienced in the lives of CYP and resulted in feelings of resentment and stress [39,44,54]. Several studies reported that the first symptoms experienced at diagnosis were particularly challenging, as was the period of waiting for a diagnosis and trying to find appropriate treatment [37,49,52,54]. Participants described how at the time of diagnosis, they experienced self-doubt and questioned whether they possibly imagined symptoms [39,43,44,54]. Once the diagnosis was made, CYP found their sense of normalcy disrupted, and although they had confirmation of their symptoms, sharing the diagnosis with others was difficult, with some CYP choosing to keep their condition hidden [39] or preferring not to be defined by it [54].
Skin rashes, joint pain, muscle aches or weakness, loss of mobility, hair loss, and fatigue severely impacted CYPsʹ self-perception [44,45,50,52] as they no longer saw themselves as young and healthy but instead sick and incapacitated. The severe emotional impact of cSLE resulted in feelings of grief and resentment towards the disease and a strong wish that cSLE had never entered their lives [50]. Two studies highlighted how symptoms severely influenced how CYP felt about their appearance, including making them feel unattractive [45], giving them a heightened sense of self-consciousness, and contributing to poor self-image [54]. Some CYP experienced being teased, described as a 'freak', or told that they looked 'weird' in school, which emphasized the feeling that they were different and not similar to their peers. This notion of being different was evident across other studies where CYP were acutely aware that the symptoms of cSLE imposed limitations on their ability to be normal [44,45,52,54].
Severity. The symptoms experienced by CYP were an individual and subjective experience [44,45,52,54]. CYP understood that cSLE had both minor and major symptoms, and although joint pain, muscle aches, weakness, and rashes were considered common, there was variability between 'levels of disease' and to what degree individuals were impacted [52,54]. Living with cSLE was therefore described as a very individual experience, with some participants 'feeling lucky' [54] about how it manifested in them, whereas others described their cSLE as more severe [44].

Medicines and Side Effects Dreaded steroids
It was really hard getting to school. I was on prednisone and got pretty fat, so I was getting bullied a lot. It was hard [54] It was just so defeating. No matter how hard I try now, I can't take off weight and I have this fat face. Who's going to want to date me? [37] I just wanted to come off them. Even when I was only on half a tablet, I didn't feel happy with being on them [52] [ 39,44,[52][53][54] Conflicting feelings

Relationships with health providers
It's really all about the connection with your doctor . . . I think it's just the relationship that you have with your doctor [39] I will still just take medicine cos I mean it's prescribed by the doctor . . . I trust that my doctor would know some of the side effects . . . make informed decision on dosage [54] [ 37,39,40,50,[52][53][54] Maintaining positivity I try to be optimistic and hope that if I make plans, I'll be healthy enough when that day comes. If I'm not, I hope others will understand [37] Having lupus helps you in many ways: makes you more responsible [44] [37, 44,54] * Authors explanations.

Challenging Symptoms
Disruption to Life and Altered Self. The journey to a diagnosis was the first disruption experienced in the lives of CYP and resulted in feelings of resentment and stress [39,44,54]. Several studies reported that the first symptoms experienced at diagnosis were particularly challenging, as was the period of waiting for a diagnosis and trying to find appropriate treatment [37,49,52,54]. Participants described how at the time of diagnosis, they experienced self-doubt and questioned whether they possibly imagined symptoms [39,43,44,54]. Once the diagnosis was made, CYP found their sense of normalcy disrupted, and although they had confirmation of their symptoms, sharing the diagnosis with others was difficult, with some CYP choosing to keep their condition hidden [39] or preferring not to be defined by it [54].
Skin rashes, joint pain, muscle aches or weakness, loss of mobility, hair loss, and fatigue severely impacted CYPs' self-perception [44,45,50,52] as they no longer saw themselves as young and healthy but instead sick and incapacitated. The severe emotional impact of cSLE resulted in feelings of grief and resentment towards the disease and a strong wish that cSLE had never entered their lives [50]. Two studies highlighted how symptoms severely influenced how CYP felt about their appearance, including making them feel unattractive [45], giving them a heightened sense of self-consciousness, and contributing to poor self-image [54]. Some CYP experienced being teased, described as a 'freak', or told that they looked 'weird' in school, which emphasized the feeling that they were different and not similar to their peers. This notion of being different was evident across other studies where CYP were acutely aware that the symptoms of cSLE imposed limitations on their ability to be normal [44,45,52,54].
Severity. The symptoms experienced by CYP were an individual and subjective experience [44,45,52,54]. CYP understood that cSLE had both minor and major symptoms, and although joint pain, muscle aches, weakness, and rashes were considered common, there was variability between 'levels of disease' and to what degree individuals were impacted [52,54]. Living with cSLE was therefore described as a very individual experience, with some participants 'feeling lucky' [54] about how it manifested in them, whereas others described their cSLE as more severe [44].
Fatigue. Eight of the nineteen studies addressed fatigue and described it as one of the most burdensome symptoms of cSLE [38,[41][42][43][44]46,52,54]. Two studies examined fatigue and its relationship to depression and poor health-related quality of life (HRQoL) [38,42]. Furthermore, fatigue was reported to impact school activities, limiting CYP's capacity to study and perform well at school [46,49,54]. When asked to comment on the strengths and weaknesses of the health-related quality of life/fatigue (Peds QL-FS) measure [52], CYP identified the need for a separate questionnaire to focus on fatigue as the 'main issue' with having cSLE [52].
Depression and Anxiety: Five studies reported depressive symptoms, anxiety, and suicidal ideation in CYP living with cSLE [41,42,[45][46][47]. Assessment of depression was conducted using the children depression inventory (CDI) [42,[45][46][47], the Patient Health Questionnaire-9 (PHQ-9) [41], and the Beck Depression Inventory (BDI-II) [47]. Three of these studies reported higher rates of depression and suicidal ideation among CYP living with cSLE compared to age and sex-matched healthy CYP and found links between depression, medication non-adherence, and appearance concerns [41,45,46]. In contrast, Donnelly et al. [42] and Kohut et al. [47] found near-normal values for both depressive symptoms and anxiety among CYP, noting an association between higher levels of depressive symptoms and reduction in HRQoL. Two studies assessed anxiety using the Screen for Child Anxiety Related Emotional Disorders (SCARED) questionnaire [42,46], where 50% of CYP in the Jones et al. [46] study and 34% of CYP in the Donnelly et al. [42] study reported clinically relevant anxiety levels that impacted on HRQoL.

Medicines and Side Effects
Dreaded Steroids. Medication regimes, especially the use of steroidal drugs, raised strong and opposing feelings among CYP [37,40,43,[52][53][54]. CYP understood and appreciated the effectiveness of steroids in treating their cSLE [37,52,54] but also loathed the unpleasant side effects of weight gain, swollen face, skin changes (such as acne, striae, and flushing), and increased appetite [39,50,53]. Although the physical side effects were the most distressing aspect of taking steroids, CYP also experienced anxiety, wakefulness, higher physical depressive symptoms, and lower self-esteem [37,47,54]. Ruperto et al. (2004) [51] postulated poorer HRQoL in CYP with active disease or accumulated damage in the renal, central nervous, and musculoskeletal systems, including impaired self-esteem due to changes in body image from the associated side effects of aggressive corticosteroid and immunosuppressive medications. The significant side effects of steroids heightened the sense of self-consciousness, awkwardness, and awareness of being different [44,50].
Conflicting Feelings. Four studies described how cSLE medications posed a paradoxical dilemma for CYP, where being dependent on medications for life, multiple times a day, to maintain their health was described as weird, stressful, and nonsensical [40,[52][53][54]. This was especially true when CYP believed their cSLE was too mild to warrant treatment or believed that it would not be a 'big deal' if they refrained from taking their medications [43,[52][53][54]. Because medications were linked to changes in their face and body, social aspects of their life were further impacted; hence medications were not looked upon favorably [44]. However, CYP also came to appreciate the positive aspect of their medications when they started to feel better, and the right medications finally helped mitigate their symptoms [40,52,54]. CYP felt that sometimes health professionals would state cSLE was the reason for their symptoms when in fact, they believed the medications were the cause which created a situation where CYP felt at odds with the treatment plan [54].
Medication Adherence. CYP stated they did not take their prescribed medications because of forgetfulness, the number of pills, the bad taste, and/or concern about side effects [39,41,43,45,53]. Some CYP doubted the need for medications because they deemed they were not sick enough or felt their cSLE 'wasn't that bad' [52,53], whereas others described a lack of perceived improvement in daily symptoms [43] as a barrier to adherence. Other common issues that impacted adherence were the logistics around taking medications, disruptions to normal activities, and sometimes business and/or getting out of the routine [39,43,53]. Two studies stated that adherence was influenced by a perceived lack of transparency from health professionals about the side effects [53,54]. Case et al. (2021) reported that a trusting patient-provider relationship positively impacted medication adherence, especially when CYP perceived healthcare professionals were working in their best interests. Other key factors that contributed to adherence were: recognizing the benefits through the acquisition of knowledge about cSLE; understanding how medication helped them control their disease, including reducing flares; and appreciating that medications enabled them to engage in activities to achieve their aspirations [40,43,53]. In addition, Tan et al. (2021) reported that parents' attitudes, involvement, and strict monitoring of medication were critical to adherence.

Complicated Life
School, Sports, and Social Activities. The burden of treatment, constant clinic appointments, side effects of medication use, and symptoms all impacted daily life, including school attendance, performance [43,49], and sports and leisure activities [44,54]. CYP described a desire to do well at school but conceded that the unpredictable nature of cSLE resulted in a lack of control over educational pursuits [37,49]. Being able to participate and being included in normal school activities was described as a key way of coping with cSLE in a social context [50].
Giving Things Up. The sense of having to 'give things up' or being limited in pursuing life's usual adventures, such as back packing or living overseas, was a source of frustration [44,50,54]. In addition, the prognostic uncertainty of cSLE led CYP to feel worried about their future and ability to achieve long-term goals [43,44,50,54]. This was reflected in their perceptions of limitations regarding educational and career aspirations, anxiety about the worsening or persistence of disease, concerns about a shorter lifespan, and the potential effect on their ability to have a family of their own [43,44,46,50,54].
Lack of Understanding.
Coming to understand what cSLE is and how they came to have it resulted in CYP making associations and interpretations based on: what they were told by health professionals [43,44], explanations from parents [37,44], and information sources from the internet [54]. Sometimes these information sources were deemed to be too 'sciencey' [43], whereas others claimed they wanted to know everything they possibly could [54]. Children and young people also felt adults did not always provide the full picture, whereas it was important for CYP to know that cSLE was not going to go away [39,54].
Misunderstanding the nature of cSLE, the aetiology, and how it impacted CYP's lives were commonly encountered [44,54]. There was a sense among CYP that family, friends, teachers, and even health professionals did not appreciate or understand the difficulty of living with cSLE, and consequently, empathy was lacking [39,43,44]. Misinformation created negative feelings, especially when it led to peers frantically wiping down surfaces for fear they would 'catch' cSLE [43,50]. This lack of understanding was deemed particularly important in relation to schools where CYP felt that education from health professionals to teachers might help with some of the challenges they faced [43]. More public exposure and the need for disease-specific information about cSLE were considered highly important [43,50,54].

Ways of Coping
Family and Friends. CYP described how talking to others with cSLE, either in person or online, was valuable for gaining a sense of belonging, being understood, and enhancing overall wellbeing [37,39,54]. Family members were deemed invaluable for providing emotional and practical support, access to treatment, encouraging medication adherence, and providing information about cSLE [37,39,43,44,50,53,54]. Several studies addressed the role that friends/peers played [39,53,54]. Relationships with peers provided invaluable emotional support that enabled CYP to cope and manage their life with cSLE [39,50]. Being included and encouraged to join normal activities [50] or being 'cheered up' about changes in appearance due to medication side effects [53,54] helped to combat feelings of being different from peers.
Relationships with Health Providers. Connectedness and collaborative relationships with health providers were key to coping with cSLE. Having trust and confidence in their health care provider enabled participants to manage cSLE and contributed to treatment adherence [39,43,53,54]. Studies highlighted how CYP highly valued health care professionals who were accommodating and took into consideration individual needs when developing treatment plans [39,43,53]. Connections and relationships with healthcare professionals were noted to be particularly important during the transition from pediatric to adult services [39,43,54]. The transition came with tension and unanticipated challenges for CYP; thus, willingness to transition was strongly influenced by the bonds they had with their first healthcare provider, which was usually the CYP's rheumatologist [39,54].
Maintaining Positivity. Despite the many challenges of living with cSLE, CYP strived to maintain a positive attitude by viewing cSLE as not merely a challenge to overcome but also a means of developing self-confidence, empathy, resilience, and self-care abilities [39,44,50,53,54]. Thus, important life skills such as coping, empathy, and resiliency were acquired [39]. Further, CYP gained self-management skills, such as learning about their body and how to take care of it [44]. Others described how being focused and positive about learning to self-manage their medications prevented their family members from worrying [53]. In addition, some CYP experienced a recalibration of mindset and considered themselves 'lucky' to have cSLE rather than something they deemed more serious, such as cancer [39,44,54]. Finding a way to focus on the positive aspects of life, being grateful for the little things, and re-evaluating priorities were described as ways of coping [44,49,53,54].

Discussion
It is evident within this review that CYP living with cSLE experience a wide range of visible and invisible symptoms, including pain, fatigue, rashes, depression, anxiety, and altered body image that disrupts their normal psychosocial, emotional, physical, and developmental growth, and places them at higher risk of bullying, and adverse mental health outcomes than their peers. These findings are similar to the experiences of CYP with other rheumatological diseases where fatigue, anxiety, and depression have been shown to have a significant impact not only on HRQoL but day-to-day emotional and psychosocial wellbeing [23,[55][56][57]. The impact and severity of cSLE were highlighted by this review as highly unique and, in the wider literature, influenced by individual demographic and illness characteristics, with a level of impact being strongly linked to disease activity and organ damage [2,58,59]. In addition, gender, age of diagnosis, type of immunosuppressive medication, number of flares, and ethnicity are factors that influence CYP's likelihood of increased mortality [58][59][60].
Children and young people stated that the first step into their cSLE journey was waiting for a diagnosis, having their symptoms confirmed, and commencing treatment which triggered emotional turmoil, feelings of relief, and significant frustration. The difficulty in making an initial diagnosis of cSLE in CYP has been attributed to its multi-system involvement, atypical symptoms, variability in clinical presentation [2,58,61], as well as other factors, including demographic, clinical, and socioeconomic characteristics [62]. Given the importance of prompt diagnosis to prevent disease-related morbidity, Hussain et al. (2022) recommended the need for screening guidelines and policies to improve healthcare outcomes for CYP.
Medications played an important part in the management of cSLE and CYP's experiences of living with it, including their self-perception, self-esteem, and relationships with healthcare providers. Children and young people described feeling anxious and worried about the side effects of medications particularly the Cushingoid features that accompanied the use of glucocorticoids. The changes to their body, mood, and sense of self related to steroid treatment represented a confronting and psychologically distressing aspect of cSLE. Glucocorticosteroids remain a foundational treatment in SLE [63]; however, it is increasingly recognized that the dose should be kept as low as possible in order to minimize both short and long-term side effects [64]. In addition, there is a recognized need to evaluate the side effects and benefits of steroid use from CYP's perspective [65]. Given that most CYP in this review were adolescents, concerns about physical appearance and disturbance in body image were not surprising. Children and young people are extremely sensitive to and focused on changes to their appearance and the opinions of peers [66]. Alterations to their self and body image and to the psychological distress that may accompany changes potentially place them at increased risk of depression when compared to their healthy peers [67,68] and those with adult-onset SLE [69]. This points to the need for routine psychosocial screening of CYP with cSLE by health professionals to ensure at-risk and psychologically compromised CYP are identified and supported [24,70]. In addition, there is a need for increased awareness in practice and further research to identify strategies for improving early intervention for depression and anxiety in CYP with cSLE [57].
The conflicting feelings surrounding medication adherence were related to the burden of having to take medications every day, the disruption to normal life, and the potential risk of side effects; however, positive effects were experienced when medication improved CYP's condition. Treatment adherence was influenced by having a trusting relationship with healthcare providers, feeling involved with decision-making about treatment, and developing personal knowledge and understanding about cSLE. These findings are consistent with adult studies and those related to other rheumatological conditions that describe the importance of resilience [71], the healthcare provider-patient relationship [72], and patient empowerment [73] as positively impacting medication adherence. Increasing CYPs' knowledge about cSLE and medications and addressing concerns about side effects have been described as important ways to improve communication between CYP and healthcare professionals and improve adherence [16,74,75]. For CYP, this may require consideration of different approaches and broader access to educational material, such as online or web-based interventions and the use of social media, shown to enhance adherence and support young people in managing their cSLE medications [76,77]. Many CYP living with cSLE will never achieve an inactive disease state; therefore, medication adherence is a particularly important aspect of care, especially when using high immunosuppressive treatments [58,60].
Disruptions and interruptions to normal activities due to the burden of treatment, clinic appointments, side effects of medications, and feeling restricted in their ability to achieve future goals and aspirations created a complicated life for CYP living with cSLE. A correlation between neuropsychiatric symptoms, cognitive disorders, and poor academic performance in maths, learning, attention, information processing, memory, and completing school or college degrees has been reported in the literature as a significant factor for CYP with cSLE being unable to reach academic outcomes and aspirations [49,78,79]. Many CYP in this review spoke about how they felt school, family, and friends did not fully understand or appreciate the difficulties they faced living with cSLE and expressed concern about the lack of public knowledge regarding their disease. This was echoed in a study from the United Kingdom, where CYP felt that greater public awareness and wider communication about rheumatic conditions were needed, believing this would enable them to manage their daily challenges more easily [80]. Children and young people also recommended that research should focus on the lived experience of CYP to gain a greater understanding of the psychosocial impact of the illness, with CYP being actively consulted, included, and viewed as expert informants (64).
Social support systems, including peers, family, and cSLE support groups, were found to be necessary and important for coping with cSLE by CYP in this review. Organized events, such as camps for CYP with cSLE, were beneficial in providing social support through shared experiences and helping young people to normalize their life [81,82]. Camps for CYP offer opportunities for peer acceptance and social inclusion, and these benefits have been similarly described in studies in other long-term conditions such as diabetes [83], celiac disease [84], bleeding disorders [85], and epilepsy [86]. It is reported in the literature that CYP with cSLE has shown high coping scores for emotion-focused coping strategies (0.6 ± 0.2) and socially supported coping strategies (0.5 ± 0.2) linked to high happiness scores on the Brief Coping Orientation to Problems Experienced (COPE) questionnaire and the Subjective Happiness Scale (SHS) [87]. Remaining grateful and building on one's self-confidence, resiliency, and self-care abilities in managing medications, treatment, and symptoms were some of the problem-focused coping strategies identified in this review. Similarly, CYP with cSLE had high self-reported coping scores for problem-focused coping (0.7 ± 0,2) associated with high levels of happiness (r = 0.564, p = 0.002) [87]. Despite these positive findings, CYP living with cSLE require individualized, multi-dimensional interventions and support to develop coping strategies to enhance their ability to respond to the multiple challenges imposed by cSLE. This includes the skills needed to effectively transition from CYP services to adult care [82,87,88].
This review highlighted that a multidisciplinary approach is needed that includes routine assessment of both physical and psychosocial symptoms as an essential approach to care, given that early intervention for depression has the potential to improve outcomes. Ongoing psychosocial support, health education, adherence interventions, and personalized treatment plans are necessary components of holistic care for CYP living with cSLE. In addition, there needs to be greater awareness and education to the public and general practitioners about the nature of cSLE, the early symptoms, complications, and treatment strategies. Future health care and research should focus on psychosocial and developmental wellbeing, resiliency, and practical strategies for managing and living with cSLE, fatigue, interventions to promote peer support networks, and medication adherence. Future research needs to be inclusive of CYPs' voice, including the voices of younger children, and conducted in collaboration with service providers to ensure the best outcomes for CYP living with cSLE.
Limitations. This study, as with all integrative reviews, is limited by search terms, selected databases, and applied search strategy methods. In addition, this review is limited by including only empirical peer-reviewed studies published in English. Most of the instruments were well described, and reliability and validity were reported; however, due to the range and variability of instruments used, a meta-analysis of results was not possible. Not all studies described participants' SLEDAI or SDI; therefore, it is not possible to draw comparisons between CYP's experiences in terms of disease activity scores. Finally, CYP living with cSLE were not consulted on the results of this integrative review, given that the project commenced during the COVID-19 pandemic. Despite these limitations, the procedure undertaken by the authors in this review was rigorous and followed a systematic process.

Conclusions
While cSLE shares many similarities with adult-onset SLE, there are essential differences in terms of morbidity, impact of symptoms, and medication side effects that disrupt CYP's day-to-day life. An awareness of the differences in experiences and perceptions of CYP is crucial. The significant psychological and social impact of cSLE and its treatment necessitates a comprehensive, holistic approach to managing care that considers the unique needs of CYP. Studies describe fatigue as the most common symptom among adults with SLE [89,90] and highlight the significant impact of depression and anxiety on quality and daily life [91,92]. There is an opportunity for children and young people to identify these symptoms earlier and potentially prevent these impacts as children grow into adulthood. The review highlights significant anxiety, fatigue, and depression among CYP due to cSLE and points to the need for further research and exploration to understand CYPs' experiences and their support needs to more fully direct practice, research, policy, and knowledge.