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Publications

Journal Article Epublish

PubMed: 40269883 DOI: 10.1186/s12969-025-01092-5

Unmet needs and research gaps in Still's disease across ages: proceedings from a pediatric and adult joint expert panel.

Pediatr Rheumatol Online J 23(1):40

Authors: Claudia Bracaglia, Francesca Minoia, Sebastiaan J Vastert, Christoph Kessel, Lorenzo Dagna, Angelo Ravelli, Fabrizio De Benedetti

Affiliations

  • Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, ERN-RITA Centre, Piazza Sant'Onofrio 4, Rome, 00165, Italy.
  • Department of Paediatrics and Immuno-Rheumatology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Department of Pediatric Rheumatology & Immunology, Wilhelmina Children's Hospital, UMC Utrecht, Utrecht, Netherlands.
  • Department of Pediatric Rheumatology & Immunology, EULAR Centre of Excellence in Rheumatology, WWU Medical Center (UKM) Münster, Münster, Germany.
  • Unit of Immunology, Rheumatology, Allergology, and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
  • School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
  • IRCCS Istituto Giannina Gaslini, Genoa, Italy.
  • Department of Neuroscience, Ophthalmology, Genetics and Maternal and Child Sciences (DiNOGMI), University of Genoa, Genoa, Italy.
  • Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, ERN-RITA Centre, Piazza Sant'Onofrio 4, Rome, 00165, Italy.

Abstract

BACKGROUND

Still's disease (SD), including systemic juvenile idiopathic arthritis (sJIA) and adult-onset SD (AOSD), is an inflammatory condition typically characterized by daily fever, arthritis, and skin rash together with neutrophilic leukocytosis, thrombocytosis, and increased acute phase reactants. The reported differences between sJIA and AOSD appear to reflect variations along an inflammatory spectrum influenced by age, rather than differences in the underlying pathology.

METHODS

In February 2023, an expert meeting, including pediatric and adult rheumatologists, was held in Rome, Italy, with the aim of defining more precise and timely strategies for disease management. The following four topics were discussed: (1) early recognition and diagnosis of SD; (2) pathogenetic pathways and possible biomarkers for diagnosis and response; (3) refractory disease and risk factors, and (4) treatment of SD and its complications.

RESULTS

The development of improved diagnostic criteria and validation of biomarkers are important steps towards achieving early diagnosis, although several biomarkers remain to be universally validated and available for clinical practice. Additionally, awareness of important complications of SD, including macrophage activation syndrome and lung disease, is crucial for improving patient outcomes, alongside an improved understanding of risk factors for the development of refractory disease. While interleukin (IL)-1 and IL-6 inhibitors have improved the treatment landscape of SD, harmonizing the therapeutic approach across centers and countries, together with developing treatment strategies for refractory patients, still represents a challenge.

CONCLUSIONS

Here, we summarize the results of discussions among experts, supplemented by relevant literature, and highlight unmet needs in the diagnosis and management of SD.

MeSH

Arthritis, Exanthema, Biomarkers, Rheumatic Diseases, Risk Factors, Diagnosis, Research, Therapeutics