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Use of immunosuppressive medications and prevalence of disability among patients with pediatric systemic lupus erythematosus: initial analysis of the CARRAnet registry
© Hersh et al; licensee BioMed Central Ltd. 2012
- Published: 13 July 2012
- Systemic Lupus Erythematosus
- Renal Biopsy
- Disease Feature
- Avascular Necrosis
- Disease Activity Index
To describe the demographics, disease features and treatment of participants with pediatric systemic lupus erythematosus (pSLE) enrolled to date in the CARRAnet registry.
Subjects were included in the CARRAnet registry, a national pediatric rheumatology database, if diagnosed with pSLE at ≤16 years of age and ≤ age 21 at enrollment. Demographics, disease features and prior and current treatments were collected, as well as the SLE disease activity index (SLEDAI), Health Related Quality of Life (HRQOL), ACR Functional Class (current and worst ever), Childhood Health Assessment Questionnaire (CHAQ), and Physician Global Assessment of disease activity (PGA). Associations between parameters were assessed using Spearman’s coefficient, and linear regression was used to determine predictors of intravenous (IV) cyclophosphamide (CYC) administration.
Prescribed treatments in the CARRAnet registry
Ever prescribed N (%)
Currently prescribed N (%)
Methotrexate (oral or injectable)
CYC use was not associated with gender, age at onset, CHAQ score, or ACR Functional Class; but was associated with renal biopsy (p<.001 [CI 4.14-30.6]). 56 participants (45.5%) were classified as ACR Functional Class III or IV at some point in their disease course, while14 (11.4%) were considered Class III or IV at the time of enrollment. 54 participants (43.9%) rated their HRQOL as “very good” or “excellent” but physical function was impaired with CHAQ>0 in 48% (n=59). The median score for PGA and SLEDAI was 2 (range 0-7) and 2 (range 0-38), respectively. There was a significant association between the CHAQ and SLEDAI scores (rho=0.19, p=0.03) or the PGA (rho=0.26, p=0.04).
In the initial analysis of pSLE subjects in the CARRAnet registry, most participants required steroids for disease control and the majority were on additional immunosuppressants. Furthermore, disability occurred in nearly half the cohort, indicating improved treatment strategies are needed to treat pSLE.
Aimee Hersh: None; Mary Beth F. Son: None; Hermine Brunner: None; Barbara Anne Eberhard: None; Emily Von Scheven: None; CARRAnet Investigators: None.
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 (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.