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Organ system-involvement in SLE and relationship with demographic factors, disease duration and health-related quality of life in childhood SLE

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Pediatric Rheumatology201210 (Suppl 1) :A22

  • Published:


  • Arthritis
  • Systemic Lupus Erythematosus
  • Disease Duration
  • Domain Score
  • Damage Index


Damage in childhood systemic lupus erythematosus (SLE) affects ocular, musculoskeletal, neuropsychiatric, renal, cardiovascular, peripheral vascular, and skin domains and damage can affect their health related quality of life (HRQOL). Aggressive treatments have improved survival in childhood SLE, but the disease is still associated with significant morbidity. The objective of our multicenter study is to examine the organ system-involvement in childhood SLE and the relationship of damage with HRQOL, age, gender, ethnicity and disease duration.


In this cross-sectional study, children ≤18 years with SLE and parents completed the Simple Measure of the Impact of Lupus Erythematosus in Youngsters© (SMILEY©) and physicians measured Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI). SMILEY© is a new, brief, 24-item HRQOL assessment tool for pediatric SLE, that has recently been validated in US English. The four domains are: Effect on self, Limitations, Social and Burden of SLE. Responses are in the form of a 5-faces scale for easy comprehension. Higher percentage scores indicate better HRQOL. Contingent upon the data distribution of the above variables, we used student t-test, Mann-Whitney U test or the Kruskal-Wallis (KW) test to examine the relationship of SDI with age, gender, ethnicity, disease duration and HRQOL.


Out of a total of 169 children (17% male), 59 children (35%) had any damage (SDI score>0). Their age, damage and disease duration and specific organ-system involvement is given in table 1. Their ethnicities were: Black (39%), Asian or Pacific Islander (12%), Latino (27%), White (18%) and other (5%). Children predominantly had renal, neuropsychiatric, skin, and musculoskeletal involvement. Significant difference was found in damage with disease duration (p=0.005, Mann Whitney U test). There was no significant difference in damage in patients with different gender, ages or ethnicity. Parent SMILEY© total and all domain scores were decreased in patients with damage compared to patients without damage (table 2), but only the Effect on self domain scores was statistically significant (p=0.02). The child SMILEY© total, Limitation and Effect on self domain scores were lower in patients who had any damage.
Table 1

SDI scores and organ-system involvement, and HRQOL scores


Descriptives (n=169)

SDI total score


Mean ± SD (range)(n)

36±39 (1-183)


Median =24

Age (years)

15 ± 3 (6-18)

Disease duration (months)


Mean ± SD (range) (n)

36±39 (1-183)


Median =24

Renal %(n)

17 (29)

Neuropsychiatric %(n)

14 (24)

Skin %(n)

11 (18)

Musculoskeletal %(n)

6 (10)

Peripheral vascular %(n)

3 (5)

Ocular %(n)

2 (4)

Pumonary %(n)

2 (4)

Diabetes %(n)

2 (3)

Cardiovascular %(n)

2 (3)

Gastrointestinal %(n)


Premature gonadal failure %(n)

1 (2)

Malginancy %(n)

0 (0)

SMILEY total score


Mean ± SD (range) (n) Child

65 ± 14 (33-98) (166)


62 ± 15 (28-96) (153)

Table 2

Means of parent total and domain scores of SMILEY for patients with no damage (SDI=0) and patients with any damage (SDI>0)

Parent SMILEY scores

SDI score=0 (n)

SDI score >0 (n)

Effect on self domain

63±18 (96)

57±16 (55)

Limitation domain

62±19 (97)

59±16 (55)

Social domain

78±18 (97)

74±17 (55)

Burden of SLE domain

57±16 (96)

54±17 (55)

Total score

64±15 (97)

60±14 (55)


Damage in childhood SLE is significantly related to disease duration. Renal, neuropsychiatric, skin, and musculoskeletal systems are predominantly involved in our US cohort, which is similar to previous studies. The impact of damage on children’s HRQOL as perceived by parents may be different from the children’s perception and needs further examination.


Lakshmi N. Moorthy: Arthritis Foundation, 2; Maria J. Baratelli: Arthritis Foundation, 2; Margaret G.E. Peterson: Arthritis Foundation, 2; Afton L. Hassett: Arthritis Foundation, 2; Alexa B. Adams: None; Laura V. Barinstein: None; Emma J. MacDermott: None; Elizabeth C. Chalom: None; Karen Onel: None; Linda I. Ray: None; Jorge Lopez-Benitez: None; Christina Pelajo: None; Kathleen A. Haines: None; Daniel J. Kingsbury: None; Victoria W. Cartwright: None; Philip J. Hashkes: None; Nora G. Singer: None; Gina A. Montealegres: None; Ingrid Tomanova-Soltys: None; Andreas O. Reiff: None; Sandy D. Hong: None; Thomas J. A. Lehman: None.

Authors’ Affiliations

Childrens Hospital LA, Los Angeles, CA, USA
Hackensack University Medical Center, Hackensack, NJ, USA
Hospital for Special Surgery, New York, NY, USA
Legacy Emanuel Children's Hospital, Portland, OR, USA
Maimonides Medical Center, Brooklyn, NY, USA
MetroHealth Medical Center, Cleveland, OH, USA
Shaare Zedek Medical Center, Jerusalem, Israel, USA
St. Barnabas Medical Center, New Brunswick, NJ, USA
Tufts Medical Center, Boston, MA, USA
UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA
University Hospitals Case Medical Center, Cleveland, OH, USA
University of Chicago, Chicago, IL, USA
University of Iowa Children's Hospital, Iowa City, IA, USA
University of Medicine and Dentistry of NJ (UMDNJ)- Robert Wood Johnson Medical School, New Brunswick, NJ, USA
University of Michigan Medical School, Ann Arbor, MI, USA
University of Mississippi Medical Center, Jackson, MS, USA


© Moorthy et al; licensee BioMed Central Ltd. 2012

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.