ACR Provisional Criteria: Global Flares in Childhood-Onset SLE

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The SLEDAI and BILAG threshold values were all >82% sensitive and specific for denoting the severity of patients’ flares.
The SLEDAI and BILAG threshold values were all >82% sensitive and specific for denoting the severity of patients’ flares.

Provisional criteria for global flares in patients with childhood-onset systemic lupus erythematosus (cSLE) are now available from the American College of Rheumatology to identify individuals who experience a flare. These criteria will allow for the differentiation of the severity of cSLE exacerbations, according to the results of an international consensus conference that were published in Arthritis Care & Research.

The investigators sought to validate the preliminary criteria for global flare in cSLE. Patient profile ratings were requested from 503 pediatric rheumatologists from Australia, Africa, Asia, Europe, and the Americas who were members of ≥1 of the following organizations: Pediatric Rheumatology Collaborative Group, the Childhood Arthritis Rheumatology Research Alliance, the Pan American League of Associations for Rheumatology, and the Pediatric Rheumatology European Society Juvenile Lupus Working Group.

 Interpretation of the flare or “true” disease course of a particular patient profile was established using  two approaches, resulting in 2 distinct datasets for the ensuing validation exercises.

With the use of patient profile ratings, the preliminary criteria for cSLE global flares were assessed for their ability to differentiate patients who experienced varying levels of flare — minor, moderate, or major.

A total of 268 pediatricians who were experienced in the care of patients with cSLE rated unique patient profiless. The results of standard cSLE laboratory testing and information about cSLE flare descriptors were presented as: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and  erythrocyte sedimentation rate (ESR).

Diagnostic accuracy of the two highest-ranked candidate criteria that take into account absolute changes in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) confirmed MD-global, PCR, and ESR. Regarding the SLEDAI-based criteria, flare scores of ≥6.4, ≥3.0, and ≥0.6 represented major, moderate, and minor flares, respectively.

With respect to the BILAG-based algorithm, flare scores of ≥7.4, ≥3.7, and ≥2.2 constituted major, moderate, and minor flares, respectively. The SLEDAI and BILAG threshold values were all >82% sensitive and specific for denoting the severity of patients' flares.

The investigators concluded that a methodologically stringent validation process has been used in order to calculate a flare score with the ability to interpret the course of cSLE over time with respect to the level of worsening that may have occurred.

According to the available data, however, these algorithms cannot be utilized to quantify potential improvement over time.

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Reference

Brunner HI, Holland M, Beresford MW, et al; Canadian early Arthritis Cohort Investigators. American College of Rheumatology provisional criteria for global flares in childhood-onset systemic lupus erythematosus [published online April 25, 2018].  Arthritis Care Res (Hoboken). doi:10.1002/acr.23557

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