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

the PRINTO and PRCSG Investigators

Research output: Contribution to journalArticle

Abstract

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

Original languageEnglish (US)
Pages (from-to)1621-1629
Number of pages9
JournalArthritis Care and Research
Volume70
Issue number11
DOIs
StatePublished - Nov 1 2018

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Systemic Lupus Erythematosus
Consensus
Aptitude
Alopecia
Visual Analog Scale
Proteinuria
Cicatrix
Physicians

ASJC Scopus subject areas

  • Rheumatology

Cite this

Measuring Disease Damage and Its Severity in Childhood-Onset Systemic Lupus Erythematosus. / the PRINTO and PRCSG Investigators.

In: Arthritis Care and Research, Vol. 70, No. 11, 01.11.2018, p. 1621-1629.

Research output: Contribution to journalArticle

the PRINTO and PRCSG Investigators. / Measuring Disease Damage and Its Severity in Childhood-Onset Systemic Lupus Erythematosus. In: Arthritis Care and Research. 2018 ; Vol. 70, No. 11. pp. 1621-1629.
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title = "Measuring Disease Damage and Its Severity in Childhood-Onset Systemic Lupus Erythematosus",
abstract = "Objective: To describe the frequency and types of disease damage occurring with childhood-onset systemic lupus erythematosus (SLE) as measured by the 41-item Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), and to assess the SDI's ability to reflect damage severity. Methods: Information for the SDI was prospectively collected from 1,048 childhood-onset SLE patients. For a subset of 559 patients, physician-rated damage severity measured by visual analog scale (MD VAS damage) was also available. Frequency of SDI items and the association between SDI summary scores and MD VAS damage were estimated. Finally, an international consensus conference, using nominal group technique, considered the SDI's capture of childhood-onset SLE–associated damage and its severity. Results: After a mean disease duration of 3.8 years, 44.2{\%} of patients (463 of 1,048) already had an SDI summary score >0 (maximum 14). The most common SDI items scored were proteinuria, scarring alopecia, and cognitive impairment. Although there was a moderately strong association between SDI summary scores and MD VAS damage (Spearman's r = 0.49, P < 0.0001) in patients with damage (SDI summary score >0), mixed-effects analysis showed that only 4 SDI items, each occurring in <2{\%} of patients overall, were significantly associated with MD VAS damage. There was consensus among childhood-onset SLE experts that the SDI in its current form is inadequate for estimating the severity of childhood-onset SLE–associated damage. Conclusion: Disease damage as measured by the SDI is common in childhood-onset SLE, even with relatively short disease durations. Given the shortcomings of the SDI, there is a need to develop new tools to estimate the impact of childhood-onset SLE–associated damage.",
author = "{the PRINTO and PRCSG Investigators} and Holland, {Michael J.} and Beresford, {Michael W.} and Feldman, {Brian M.} and Jennifer Huggins and Ximena Norambuena and Silva, {Clovis A.} and Gordana Susic and Flavio Sztajnbok and Yosef Uziel and Simone Appenzeller and Ardoin, {Stacy P.} and Tadej Avcin and Francisco Flores and Beatrice Goilav and Raju Khubchandani and Marissa Klein-Gitelman and Deborah Levy and Angelo Ravelli and Wenderfer, {Scott E.} and Jun Ying and Nicolino Ruperto and Brunner, {Hermine I.}",
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AU - the PRINTO and PRCSG Investigators

AU - Holland, Michael J.

AU - Beresford, Michael W.

AU - Feldman, Brian M.

AU - Huggins, Jennifer

AU - Norambuena, Ximena

AU - Silva, Clovis A.

AU - Susic, Gordana

AU - Sztajnbok, Flavio

AU - Uziel, Yosef

AU - Appenzeller, Simone

AU - Ardoin, Stacy P.

AU - Avcin, Tadej

AU - Flores, Francisco

AU - Goilav, Beatrice

AU - Khubchandani, Raju

AU - Klein-Gitelman, Marissa

AU - Levy, Deborah

AU - Ravelli, Angelo

AU - Wenderfer, Scott E.

AU - Ying, Jun

AU - Ruperto, Nicolino

AU - Brunner, Hermine I.

PY - 2018/11/1

Y1 - 2018/11/1

N2 - Objective: To describe the frequency and types of disease damage occurring with childhood-onset systemic lupus erythematosus (SLE) as measured by the 41-item Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), and to assess the SDI's ability to reflect damage severity. Methods: Information for the SDI was prospectively collected from 1,048 childhood-onset SLE patients. For a subset of 559 patients, physician-rated damage severity measured by visual analog scale (MD VAS damage) was also available. Frequency of SDI items and the association between SDI summary scores and MD VAS damage were estimated. Finally, an international consensus conference, using nominal group technique, considered the SDI's capture of childhood-onset SLE–associated damage and its severity. Results: After a mean disease duration of 3.8 years, 44.2% of patients (463 of 1,048) already had an SDI summary score >0 (maximum 14). The most common SDI items scored were proteinuria, scarring alopecia, and cognitive impairment. Although there was a moderately strong association between SDI summary scores and MD VAS damage (Spearman's r = 0.49, P < 0.0001) in patients with damage (SDI summary score >0), mixed-effects analysis showed that only 4 SDI items, each occurring in <2% of patients overall, were significantly associated with MD VAS damage. There was consensus among childhood-onset SLE experts that the SDI in its current form is inadequate for estimating the severity of childhood-onset SLE–associated damage. Conclusion: Disease damage as measured by the SDI is common in childhood-onset SLE, even with relatively short disease durations. Given the shortcomings of the SDI, there is a need to develop new tools to estimate the impact of childhood-onset SLE–associated damage.

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

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