Background/Purpose:

Despite recent advances in identifying effective treatments for systemic Juvenile Idiopathic Arthritis (sJIA), many pediatric rheumatologists continue to use corticosteroids and methotrexate. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed standardized consensus treatment plans (CTPs) for sJIA with the goal of comparing their effectiveness using data collected for the CARRA Registry. Since physicians will select CTPs without randomization, each CTP must be used with sufficient frequency to allow for meaningful comparisons of efficacy. We aimed to ascertain the current anticipated frequency of CTP use by CARRA pediatric rheumatologists, and whether a clear standard of care exists for sJIA treatment.  

Methods:                                                                                                                           

An electronic survey was sent to voting members of CARRA regarding CTP choice for new-onset sJIA which has failed NSAID therapy alone. Respondents were asked to select one or more of the following CTPs for each clinical case scenario: (a) systemic corticosteroids (CS) only; or (b) methotrexate, (c) anti-IL1 or (d) anti-IL6 therapy, each with or without CS. Respondents could choose more than one CTP if factors such as insurance limitations or family preference might affect treatment. Features of the clinical case scenarios are summarized in the Table.

Table: Clinical Cases

Results:

134 of 247 (54%) CARRA members responded. The figure depicts treatment selections sorted by case, demonstrating wide variability in preferred treatment for new-onset sJIA. IL1 and IL6 inhibitors have become important treatment choices. Methotrexate use increases with more prominent arthritis features; however, methotrexate and CS usage are frequent regardless of presenting disease features. Overall, concurrent CS use was indicated by the majority of respondents across all CTPs (Case 1: 74%; Case 2: 87%, Case 3: 66%; Case 4: 91%).

Conclusion:

There is still significant variability in sJIA treatment approaches and no clear standard of care among CARRA members, with widespread use of methotrexate and CS. There is likely to be sufficient utilization of each of the CTPs for new-onset sJIA to establish comparative treatment effectiveness using the observational CARRA Registry.