Karen B Onel1, Daniel B Horton2, Daniel J Lovell3, Susan Shenoi4, Carlos A Cuello5, Sheila T Angeles-Han3, Mara L Becker6, Randy Q Cron7, Brian M Feldman8, Polly J Ferguson9, Harry Gewanter10, Jaime Guzman11, Yukiko Kimura12, Tzielan Lee13, Katherine Murphy14, Peter A Nigrovic15, Michael J Ombrello16, C Egla Rabinovich6, Melissa Tesher17, Marinka Twilt18, Marisa Klein-Gitelman19, Fatima Barbar-Smiley20, Ashley M Cooper21, Barbara Edelheit22, Miriah Gillispie-Taylor23, Kimberly Hays24, Melissa L Mannion7, Rosemary Peterson25, Elaine Flanagan26, Nadine Saad27, Nancy Sullivan28, Ann Marie Szymanski29, Rebecca Trachtman30, Marat Turgunbaev31, Keila Veiga32, Amy S Turner31, James T Reston28. 1. Hospital for Special Surgery, New York, New York. 2. Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey. 3. Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, Ohio. 4. Seattle Children's Hospital and Research Center and University of Washington, Seattle. 5. McMaster University, Hamilton, Ontario, Canada. 6. Duke University, Durham, North Carolina. 7. University of Alabama at Birmingham. 8. The Hospital for Sick Children, Toronto, Ontario, Canada. 9. University of Iowa Carver College of Medicine, Iowa City. 10. Children's Hospital of Richmond at VCU, Richmond, Virginia. 11. BC Children's Hospital, Vancouver, British Columbia, Canada. 12. Hackensack Meridian School of Medicine, Hackensack, New Jersey. 13. Stanford University, Palo Alto, California. 14. Louisiana Department of Health, New Orleans. 15. Boston Children's Hospital and Brigham and Women's Hospital, Boston, Massachusetts. 16. NIH, Bethesda, Maryland. 17. University of Chicago, Chicago, Illinois. 18. University of Calgary and Alberta Children's Hospital, Calgary, Alberta, Canada. 19. Ann & Robert Lurie Children's Hospital of Chicago and Northwestern University, Chicago, Illinois. 20. Nationwide Children's Hospital, Columbus, Ohio. 21. Children's Mercy Hospital, Kansas City, Missouri. 22. Connecticut Children's Medical Center, Hartford. 23. Baylor College of Medicine, Houston, Texas. 24. Penn State Health Children's Hospital, Hershey, Pennsylvania. 25. Dell Children's Medical Center, Austin, Texas. 26. Emory University, Atlanta, Georgia. 27. University of Michigan, Ann Arbor. 28. ECRI Institute, Plymouth Meeting, Pennsylvania. 29. Johns Hopkins All Children's Hospital, St. Petersburg, Florida. 30. Icahn School of Medicine at Mount Sinai, New York, New York. 31. American College of Rheumatology, Atlanta, Georgia. 32. Maria Fareri Children's Hospital, Valhalla, New York.
Abstract
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.
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.