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Childhood lupus
Validation of the Cutaneous Lupus Erythematosus Disease Area and Severity Index and pSkindex27 for use in childhood-onset systemic lupus erythematosus
  1. Ashwaq AlE'ed1,
  2. Pinar Ozge Avar Aydin2,
  3. Nora Al Mutairi3,
  4. Alhanouf AlSaleem3,
  5. Hafize Emine Sonmez4,
  6. Michael Henrickson2,
  7. Jennifer L Huggins2,
  8. Seza Ozen4,
  9. Sulaiman M Al-Mayouf3 and
  10. Hermine I Brunner2
  1. 1 Department of Pediatrics, College of Medicine, Qassim University, Buraidah, Saudi Arabia
  2. 2 Department of Pediatrics, University of Cincinnati College of Medicine, Division of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
  3. 3 King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
  4. 4 Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey
  1. Correspondence to Professor Hermine I Brunner; hermine.brunner{at}cchmc.org

Abstract

Objective To determine the measurement properties of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the paediatric adaptation of the Skindex29 (pSkindex27) when used in childhood-onset SLE (cSLE).

Methods Patients with mucocutaneous involvement of cSLE were evaluated at the study entry and 6 months later. Besides the CLASI and pSkindex27, the Pediatric Quality of Life Inventory Generic Core scale (PedsQL-GC), its Rheumatology Module (PedsQL-RM), the SLE Disease Activity Index (SLEDAI) and the SLE Damage Index (SDI) were completed.

Results The CLASI and pSkindex27 had high internal consistency (both Cronbach α >0.82). Children were able to complete the pSkindex27, with self-report and caregiver proxy-reports showing excellent agreement (intraclass correlation coefficient=0.97). The CLASI Activity Score (CLASI-A) was strongly correlated with the mucocutaneous domain score of the SLEDAI as was the CLASI Damage Score (CLASI-D) with that of the SDI (both: Spearman correlation coefficients (rs) >0.68). pSkindex27 summary scores were moderately correlated with those of the PedsQL-GC and PedsQL-RM (all: rs >|0.51|), the CLASI-A and CLASI-D (both: rs > 0.64), respectively. Patients who experienced a >50% improvement of the CLASI-A between study visits had significantly higher PedsQL-GC and pSkindex27 scores than those without improvement of mucocutaneous features.

Conclusion Both CLASI and pSkindex27 are useful assessment tools in cSLE, active and chronic mucocutaneous lesions and their changes over time can be measured using the CLASI and the pSkindex27 can capture the impact of mucocutaneous involvement on patient health-related quality of life.

  • skindex
  • pediatrics
  • clasi
  • csle
  • skin

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0

https://doi.org/10.1136/lupus-2018-000275

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    Footnotes

    • AA'e and POAA contributed equally.

    • Contributors AAE: study design, data collection, manuscript preparation, final approval of manuscript. POAA: data collection, statistical analysis, manuscript preparation, final approval of manuscript. NAM: data collection, manuscript preparation, final approval of manuscript. AAS: data collection, manuscript preparation, final approval of manuscript. HES: data collection, manuscript preparation, final approval of manuscript. MH: data collection, manuscript preparation, final approval of manuscript. JLH: data collection, manuscript preparation, final approval of manuscript. SO: data collection, manuscript preparation, final approval of manuscript. SMA-M: data collection, manuscript preparation, final approval of manuscript. HIB: study design, data collection, statistical analysis, manuscript preparation, final approval of manuscript.

    • Funding AAE was supported by a research scholar award of the Saudi Department of Health. HIB is supported by an academic research committee award of the Cincinnati Children’s Hospital Medical Center. The CCTST at the University of Cincinnati is funded by the National Institutes of Health (NIH) Clinical and Translational Science Award (CTSA) program, grant 5UL1TR001425-03. The CTSA program is led by the NIH’s National Center for Advancing Translational Sciences (NCATS).

    • Disclaimer The content of this manuscript is solely the responsibility of the CCTST and does not necessarily represent the official views of the NIH.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Ethics approval The institutional review boards of the participating centres approved the study (reference IRB CCHMC: 2015-5063).

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data sharing statement No additional data are available.