TY - JOUR T1 - An Evidence-Based Guideline Improves Outcomes for Patients With Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome JF - The Journal of Rheumatology JO - J Rheumatol SP - 1042 LP - 1051 DO - 10.3899/jrheum.211219 VL - 49 IS - 9 AU - Maria L. Taylor AU - Kacie J. Hoyt AU - Joseph Han AU - Leslie Benson AU - Siobhan Case AU - Mia T. Chandler AU - Margaret H. Chang AU - Craig Platt AU - Ezra M. Cohen AU - Megan Day-Lewis AU - Fatma Dedeoglu AU - Mark Gorman AU - Jonathan S. Hausmann AU - Erin Janssen AU - Pui Y. Lee AU - Jeffrey Lo AU - Gregory P. Priebe AU - Mindy S. Lo AU - Esra Meidan AU - Peter A. Nigrovic AU - Jordan E. Roberts AU - Mary Beth F. Son AU - Robert P. Sundel AU - Maria Alfieri AU - Jenny Chan Yeun AU - Damilola M. Shobiye AU - Barbara Degar AU - Joyce C. Chang AU - Olha Halyabar AU - Melissa M. Hazen AU - Lauren A. Henderson Y1 - 2022/09/01 UR - http://www.jrheum.org/content/49/9/1042.abstract N2 - Objective To compare clinical outcomes in children with hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) who were managed before and after implementation of an evidence-based guideline (EBG).Methods A management algorithm for MAS-HLH was developed at our institution based on literature review, expert opinion, and consensus building across multiple pediatric subspecialties. An electronic medical record search retrospectively identified hospitalized patients with MAS-HLH in the pre-EBG (October 15, 2015, to December 4, 2017) and post-EBG (January 1, 2018, to January 21, 2020) time periods. Predetermined outcome metrics were evaluated in the 2 cohorts.Results After the EBG launch, 57 children were identified by house staff as potential patients with MAS-HLH, and rheumatology was consulted for management. Ultimately, 17 patients were diagnosed with MAS-HLH by the treating team. Of these, 59% met HLH 2004 criteria, and 94% met 2016 classification criteria for MAS complicating systemic juvenile idiopathic arthritis. There was a statistically significant reduction in mortality from 50% before implementation of the EBG to 6% in the post-EBG cohort (P = 0.02). There was a significant improvement in time to 50% reduction in C-reactive protein level in the post-EBG vs pre-EBG cohorts (log-rank P < 0.01). There were trends toward faster time to MAS-HLH diagnosis, faster initiation of immunosuppressive therapy, shorter length of hospital stay, and more rapid normalization of MAS-HLH–related biomarkers in the patients post-EBG.Conclusion While the observed improvements may be partially attributed to advances in treatment of MAS-HLH that have accumulated over time, this analysis also suggests that a multidisciplinary treatment pathway for MAS-HLH contributed meaningfully to favorable patient outcomes. ER -