2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis
Authors: Onel Karen B. , Horton Daniel B. , Lovell Daniel J. , Shenoi Susan , Cuello Carlos A. , Angeles‐Han Sheila T. , Becker Mara L. , Cron Randy Q. , Feldman Brian M. , Ferguson Polly J. , Gewanter Harry , Guzman Jaime , Kimura Yukiko , Lee Tzielan , Murphy Katherine , Nigrovic Peter A. , Ombrello Michael J. , Rabinovich C. Egla , Tesher Melissa , Twilt Marinka , Klein‐Gitelman Marisa , Barbar‐Smiley Fatima , Cooper Ashley M. , Edelheit Barbara , Gillispie‐Taylor Miriah , Hays Kimberly , Mannion Melissa L. , Peterson Rosemary , Flanagan Elaine , Saad Nadine , Sullivan Nancy , Szymanski Ann Marie , Trachtman Rebecca , Turgunbaev Marat , Veiga Keila , Turner Amy S. , Reston James T. 
Published: March 2022

Objective

To provide updated guidelines for pharmacologic management of juvenile idiopathic arthritis (JIA), focusing on treatment of oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA with and without macrophage activation syndrome. Recommendations regarding tapering and discontinuing treatment in inactive systemic JIA are also provided.

Methods

We developed clinically relevant Patient/Population, Intervention, Comparison, and Outcomes questions. After conducting a systematic literature review, the Grading of Recommendations Assessment, Development and Evaluation approach was used to rate the quality of evidence (high, moderate, low, or very low). A Voting Panel including clinicians and patients/caregivers achieved consensus on the direction (for or against) and strength (strong or conditional) of recommendations.

Results

Similar to those published in 2019, these JIA recommendations are based on clinical phenotypes of JIA, rather than a specific classification schema. This guideline provides recommendations for initial and subsequent treatment of JIA with oligoarthritis, TMJ arthritis, and systemic JIA as well as for tapering and discontinuing treatment in subjects with inactive systemic JIA. Other aspects of disease management, including factors that influence treatment choice and medication tapering, are discussed. Evidence for all recommendations was graded as low or very low in quality. For that reason, more than half of the recommendations are conditional.

Conclusion

This clinical practice guideline complements the 2019 American College of Rheumatology JIA and uveitis guidelines, which addressed polyarthritis, sacroiliitis, enthesitis, and uveitis. It serves as a tool to support clinicians, patients, and caregivers in decision‐making. The recommendations take into consideration the severity of both articular and nonarticular manifestations as well as patient quality of life. Although evidence is generally low quality and many recommendations are conditional, the inclusion of caregivers and patients in the decision‐making process strengthens the relevance and applicability of the guideline. It is important to remember that these are recommendations. Clinical decisions, as always, should be made by the treating clinician and patient/caregiver.