Inactive Disease and Remission in Childhood-Onset Systemic Lupus Erythematosus

Cincinnati Children's Hospital Medical Center, University of Cincinnati, William S. Rowe Division of Rheumatology, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA.
Arthritis care & research 05/2012; 64(5):683-93. DOI: 10.1002/acr.21612
Source: PubMed


To define inactive disease (ID) and clinical remission (CR) and to delineate variables that can be used to measure ID/CR in childhood-onset systemic lupus erythematosus (cSLE).
Delphi questionnaires were sent to an international group of pediatric rheumatologists. Respondents provided information about variables to be used in future algorithms to measure ID/CR. The usefulness of these variables was assessed in 35 children with ID and 31 children with minimally active lupus (MAL).
While ID reflects cSLE status at a specific point in time, CR requires the presence of ID for >6 months and considers treatment. There was consensus that patients in ID/CR can have <2 mild nonlimiting symptoms (i.e., fatigue, arthralgia, headaches, or myalgia) but not Raynaud's phenomenon, chest pain, or objective physical signs of cSLE; antinuclear antibody positivity and erythrocyte sedimentation rate elevation can be present. Complete blood count, renal function testing, and complement C3 all must be within the normal range. Based on consensus, only damage-related laboratory or clinical findings of cSLE are permissible with ID. The above parameters were suitable to differentiate children with ID/CR from those with MAL (area under the receiver operating characteristic curve >0.85). Disease activity scores with or without the physician global assessment of disease activity and patient symptoms were well suited to differentiate children with ID from those with MAL.
Consensus has been reached on common definitions of ID/CR with cSLE and relevant patient characteristics with ID/CR. Further studies must assess the usefulness of the data-driven candidate criteria for ID in cSLE.

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    • "When used in children and adolescents, the SLE Responder Index has high specificity but only modest sensitivity in capturing cSLE improvement as rated by the treating physician or patient (parent) [51]. International consensus formation efforts resulted in several key criteria for clinical remission and inactive disease in cSLE [52] (Table 3). Similarly, criteria for global flares have been developed for cSLE and aSLE [53,54]. "
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