​​Standardizing Still’s Disease Research: Global Data Harmonization Needed Save

A recent study presented at #ACR24 convergence highlights significant gaps and inconsistencies in the research of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD). Analyzing 195 studies with over 18,000 patients, they found considerable variation in clinical and outcome measures between pediatric and adult cohorts.

Still’s disease, encompassing sJIA in children and AOSD in adults, is a rare inflammatory condition that can lead to severe complications including the most recently highlighted lung disease. Despite being part of the same disease spectrum, sJIA and AOSD are often studied and treated differently. The review revealed that sJIA studies predominantly used ILAR classification criteria (80%), while nearly all AOSD studies (98%) relied on Yamaguchi criteria. There was no consensus on defining or measuring macrophage activation syndrome (MAS), a severe complication, with various criteria like the 2016 MAS criteria and HLH-2004 criteria being used.

The study also pointed out a lack of demographic reporting, with only 12% of studies including data on race and ethnicity. Clinical features varied, with symptoms like pharyngitis, myalgias, arthralgias, pleuritis, and pericarditis more commonly reported in AOSD, while laboratory markers such as CRP, ESR, ferritin, and WBC were consistently measured in both groups. Outcome measures further illustrated this divide, with one-third of AOSD studies using the Pouchot scoring system and a similar proportion of sJIA studies employing the ACR criteria for inactive disease. Disease course was more frequently reported in AOSD studies than in sJIA studies.

The lack of uniformity in data collection and outcome measurement hampers researchers' ability to compare findings across studies and collaborate internationally. The study authors emphasized the need for a global consensus on a standardized minimal dataset to advance care for Still’s Disease. A unified approach would enable larger, more comprehensive studies, enhancing understanding of disease progression and complications like MAS, improving global recognition of rare complications, and guiding the development of targeted, evidence-based treatments.

The community needs to define a consensus-based dataset for Still’s Disease, which could harmonize data collection, streamline research efforts, and ultimately improve outcomes for patients worldwide. This study underscores the urgent need for a collaborative global framework to study Still’s Disease, bridging the gap between pediatric and adult care and ensuring better treatment strategies for all patients living with this rare condition.