The Asia‐Pacific League of Associations for Rheumatology Consensus Recommendations on the Management of Systemic Juvenile Idiopathic Arthritis (Juvenile Still's Disease)
Thaschawee Arkachaisri, Kai Liang Teh, Soamarat Vilaiyuk, Sulaiman M. Al‐Mayouf, Swee Ping Tang, Takako Miyamae, Elizabeth Y. Ang, Vahid Ziaee, Narendra Kumar Bagri, Suma Balan, Christine B. Bernal, Winnie K. Y. Chan, Sirirat Charuvanij, Priscilla Campbell‐Stokes, Assunta C. H. Ho, Jayathri S. Jagoda, Rebecca A. James, Taqdees Khaliq, Saira Elaine Anwer Khan, Cherica A. Tee, Chao‐Yi Wu, Carla Marie Asis, Kosar Asnaashari, Pauline Chan, Marian Crisal M. Celindro‐Chan, Nathalie Gail U. Choi, Ma. Theresa Collante, Junjie Huang, Sern Chin Lim, Maria J. H. Lebrudo, Butsabong Lerkvaleekul, Watchareewan Sontichai, Mariel B. Valmonte, Leonila F. Dans, Sumaira FarmanABSTRACT
Background
The unique characteristics of the Asia Pacific called for the development of pragmatic and viable guidelines for the management of juvenile idiopathic arthritis (JIA). These consensus recommendations represent the second part of the guideline intending to offer an updated and regionally relevant framework for the management of systemic JIA (sJIA)/juvenile Still's disease.
Methods
A multidisciplinary task force of 35 members from 14 countries, including pediatric and adult rheumatologists as well as patient representatives, was convened. The guideline development followed the GRADE, ADAPTE, and AGREE II frameworks. Relevant international guidelines were critically appraised, and a systematic literature review was performed to address 10 PICO questions. Draft statements were discussed and voted upon using a modified Delphi process, with consensus defined as ≥ 80% agreement.
Results
Four overarching principles and eighteen statements, including five statements addressing macrophage activation syndrome (MAS) were formulated. The recommendations align with international guidelines, advocating early use of IL‐1/IL‐6 inhibitors, avoidance of glucocorticoid monotherapy in sJIA without MAS, promoting tapering of glucocorticoids once inactive disease is achieved, alongside class switching for biologic refractory cases and a treat‐to‐target approach akin to EULAR/PReS guideline. Additional features include recommending conventional synthetic DMARD when biologics are unavailable and listing the options, acknowledging regional health system heterogeneity in the Asia Pacific, mentioning tapering strategy, discouraging NSAIDs as initial monotherapy, highlighting non‐biologic salvage options, and placing less emphasis on sJIA‐associated lung diseases.
Conclusion
These recommendations provide direction for practitioners caring for sJIA patients in limited resource areas to avoid treatment delay, hence improve overall outcomes. A shared decision approach and treat‐to‐targets are emphasized.