Measuring Disease Damage and Its Severity in Childhood‐Onset Systemic Lupus Erythematosus

• DOI: 10.1002/acr.23531
• Publication Date: 2018-02-06T21:22:42Z
• Authors: Michael J. Holland; Michael W. Beresford; Brian M. Feldman; Jennifer Huggins; Ximena Norambuena; Clovis A. Silva; Gordana Susic; Flavio Sztajnbok; Yosef Uziel; Simone Appenzeller; Stacy P. Ardoin; Tadej Avcin; Francisco Flores; Beatrice Goilav; Raju Khubchandani; Marissa Klein‐Git...; Deborah Levy; Angelo Ravelli; Scott E. Wenderfer; Jun Ying; Nicolino Ruperto; Hermine I. Brunner
• Keywords: Male; Adolescent; Severity of Illness Index; 3. Good health; Cohort Studies; 03 medical and health sciences; 0302 clinical medicine; Rheumatology; Humans; Lupus Erythematosus, Systemic; Female; Child

Objective To describe the frequency and types of disease damage occurring with childhood‐onset systemic lupus erythematosus (SLE) as measured by 41‐item Systemic Lupus International Collaborating Clinics/American College Rheumatology Damage Index (SDI), to assess SDI's ability reflect severity. Methods Information for SDI was prospectively collected from 1,048 SLE patients. For a subset 559 patients, physician‐rated severity visual analog scale (MD VAS damage) also available. Frequency items association between summary scores MD were estimated. Finally, an international consensus conference, using nominal group technique, considered capture SLE–associated its Results After mean duration 3.8 years, 44.2% patients (463 1,048) already had score >0 (maximum 14). The most common scored proteinuria, scarring alopecia, cognitive impairment. Although there moderately strong (Spearman's r = 0.49, P < 0.0001) in (SDI >0), mixed‐effects analysis showed that only 4 items, each <2% overall, significantly associated damage. There among experts current form is inadequate estimating Conclusion Disease SLE, even relatively short durations. Given shortcomings SDI, need develop new tools estimate impact