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.

ObjectiveAlthough interleukin‐1 (IL‐1)/IL‐6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung disease during treatment. This study was undertaken to retrospectively evaluate incidence and risk factors for eosinophilia and describe lung disease outcomes in IL‐1/IL‐6 inhibitor–exposed patients with systemic JIA.MethodsAmong JIA patients at our institution exposed to interleukin‐1 (IL‐1)/IL‐6 inhibitors (1995–2022), we compared incidence rate of eosinophilia in systemic JIA compared to other JIA, stratified by medication class (IL‐1/IL‐6 inhibitors, other cytokine inhibitors, methotrexate). We used Cox models to identify predictors of eosinophilia during IL‐1/IL‐6 inhibitor use and summarized treatment changes and outcomes after eosinophilia, including lung disease. HLA typing was performed on a clinical or research basis.ResultsThere were 264 new medication exposures in 75 patients with systemic JIA and 41 patients with other JIA. A total of 49% of patients with systemic JIA with HLA typing (n = 45) were positive for HLA–DRB1*15 alleles. Eosinophilia was common during IL‐1/IL‐6 inhibitor use and did not differ by systemic JIA compared to other JIA (0.08 and 0.07 per person‐year, respectively; P = 0.30). Among systemic JIA patients, pretreatment macrophage activation syndrome (MAS) was associated with a higher rate of subsequent eosinophilia on biologic therapy (unadjusted hazard ratio 3.2 [95% confidence interval 1.2–8.3]). A total of 4 of 5 patients who switched therapy within 10 weeks of eosinophilia experienced disease flare compared to none of the patients who continued the original therapy. A total of 8 of 25 patients with pulmonary evaluations had lung disease, and all had severe manifestations of systemic JIA (MAS, intensive care unit stay). One death was attributed to systemic JIA–lung disease.ConclusionEosinophilia is common in JIA patients using IL‐1/IL‐6 inhibitors. Severe disease may be associated with eosinophilia and lung disease in systemic JIA.

Our rheumatology workforce is facing a moment of reckoning with the stark lack of racial and ethnic diversity. National events have called increasing attention to the multitude of barriers faced by people from groups who are underrepresented in medicine (URiM), defined as "racial and ethnic populations that are underrepresented in the medical profession relative to their numbers in the general population," including women, people with disabilities, and self-identified Black, Latinx, Native Hawaiian, Pacific Islander, American Indian, and Alaska Native individuals (1).This article summarizes key concepts from a virtual forum hosted by Brigham and Women's Hospital with Drs. Kerr, Stone, and Blanco on "Diversity in the Rheumatology Research Workforce." The forum focused on diversity, equity, and inclusion (DEI) within race and ethnicity, recognizing that these categories are social constructs that do not exist in isolation. Rather, as outlined in the theory of intersectionality, a person's self-identified race and ethnicity will intersect with other aspects of identity, social position, and processes and policies in complex ways that deserve further dedicated research (2). Herein, we will explore some of the barriers and biases encountered by URiM rheumatology professionals and outline changes that are required to recruit and retain a more diverse workforce.We must work to diversify the rheumatology workforce not only because it is the only right and equitable approach, but also because it is critical to the patients we serve and to the improvement of our field. URiM physicians have been shown to provide higher quality care to patients identifying as racial or ethnic minorities, and racial concordance with providers can improve communication and therefore care (3). This makes increasing URiM provider representation an important and necessary component of working toward equity in patient care and health outcomes. However, it is also an independent goal that will bring essential perspectives and experiences, which will impact the quality of the research questions we ask and the clinical care we provide, and thus requires independent attention and strategies (4). While the responsibility to recognize and dismantle structural racism in rheumatology is our shared responsibility, we can only work toward this if URiM voices are heard and valued.Recognizing these important needs, we will outline 1) the striking shortage of URiM professionals in rheumatology, 2) barriers faced by URiM applicants and professionals, 3) actionable strategies to increase URiM professional recruitment and retention, and 4) methods for teaching bias, structural racism, and structural competency. We will use terminology for race and ethnicity that reflects the cited studies (5). THE CRITICAL SHORTAGE OF URiM PROFESSIONALS IN RHEUMATOLOGYThe field of medicine struggles with a lack of diversity in many ways, which is reflected in suboptimal care for vulnerable patientThe content of this article is solely the responsibility of the authors and does not...