Comparing paediatric- and adult-onset linear morphoea in a large tertiary-referral scleroderma centre

Abstract

Background: Linear morphoea is a severe morphoea subtype associated with extracutaneous manifestations, potentially permanent disfigurement and functional impairment. Linear morphoea is more prevalent in paediatric patients, and knowledge of disease in adults is limited. The objective of this study was to compare paediatric- and adult-onset linear morphoea, in an exclusively adult population. Methodology: This was a retrospective cohort study of adult patients with linear morphoea seen over a 3-year period at a single-site adult tertiary-referral Connective Tissue Disease centre. Clinical markers of disease severity and course, including anatomical distribution, extracutaneous manifestations, cutaneous symptoms, associated autoimmunity, inflammatory blood parameters, Dermatology Life Quality Index scores, treatment requirements and modified Localised Scleroderma Activity Tool were assessed and compared in paediatric- and adult-onset linear morphoea. Results: Of 298 patients with morphoea seen during the study period, 135 had linear morphoea and 133 were included in the study. Most were female (78.9%), the mean age was 36.5 years and almost half (43.6%) had adult-onset disease. Disease was similarly severe between groups with regard to anatomical distribution, cutaneous symptoms (n = 89, 66.9%), extracutaneous manifestations (n = 76, 57.1%), antinuclear antibody–positivity (n = 40, 40.4%), raised erythrocyte sedimentation rate (n = 27, 25.0%) and associated autoimmune diagnoses (n = 15, 11.3%). Prescribed treatments were similar between groups; 73.7% receiving methotrexate and almost one-third (32.3%) requiring more than one steroid-sparing agent. Those with paediatric-onset had more disease-related damage, with a mean modified Localised Scleroderma Skin Damage Index score of 19.5 (95% confidence interval: 17.0–22.0) versus 8.1 (95% confidence interval: 4.4–11.8; p < 0.001). Significantly more patients with adult-onset linear morphoea had quiescent disease (p = 0.0332), and even after correcting for disease duration, paediatric-onset patients still had 2.6 times greater odds of active disease (odds ratio = 2.59, 95% confidence interval: 0.9–7.6; p = 0.083). Conclusion: Linear morphoea in adults can be a severe disease with extracutaneous, autoimmune and systemic features. Adults with paediatric-onset disease appear to have more severe cumulative damage, greater functional impairment and ongoing disease activity. This patient subgroup may require particularly close monitoring and more aggressive therapy.

Systematic Review of Health‐Related Quality of Life Impact in Juvenile Localized Scleroderma

“…Pediatric‐onset JLS was noted to have 2.6 odds of active disease compared with adult‐onset disease, with notable differences between damage scores (LoSDI 19.5 > 8.1). In this study, univariate analysis showed that children are at a higher risk of ongoing active disease, more cumulative damage, and potentially increased functional impairment 23 . In this cohort, 56.3% escalated therapy, and a third of these required more than one immunosuppressive agent.…”

“…In this study, univariate analysis showed that children are at a higher risk of ongoing active disease, more cumulative damage, and potentially increased functional impairment. 23 In this cohort, 56.3% escalated therapy, and a third of these required more than one immunosuppressive agent. These medications, as discussed below, add negatively to the patient and caregiver experience.…”

“…Any symptom correlates with deleterious QoL perception not only by the patient but also by their caregivers, 16 especially in those patients with compromised physical functioning 17 . Pediatric‐onset disease is associated with more severe cumulative damage, more active and ongoing disease, and an increased risk of functional impairment compared with adult‐onset disease 23 . Additionally, children appear to experience their disease differently than adults with LS or caregivers, and caregivers seem to be unaware of the level of discomfort experienced by their children in relation to ECMs 16,24 …”

“…Finally, the Localized Scleroderma Quality of Life Instrument (LoSQI) was recently validated in the SCORE study and its international validation is ongoing. 23 The LoSQI addresses some items, such as skin sensations, physical functioning, body image, social support, and medication side effects; however, this patientcentered questionnaire requires more detailed psychometric analysis before being used either clinically or for research purposes. Baseline HRQoL result analysis from this study are currently underway across different sites from the United States and Canada (unpublished data).…”

“…17 Pediatric-onset disease is associated with more severe cumulative damage, more active and ongoing disease, and an increased risk of functional impairment compared with adult-onset disease. 23 Additionally, children appear to experience their disease differently than adults with LS or caregivers, and caregivers seem to be unaware of the level of discomfort experienced by their children in relation to ECMs. 16,24 A subset of studies that employed the CDLQI questionnaire evaluated factors associated with decreased HRQoL (Table 3).…”