Sharon A Chung1, Mark Gorelik2, Carol A Langford3, Mehrdad Maz4, Andy Abril5, Gordon Guyatt6, Amy M Archer7, Doyt L Conn8, Kathy A Full9, Peter C Grayson10, Maria F Ibarra11, Lisa F Imundo2, Susan Kim1, Peter A Merkel12, Rennie L Rhee12, Philip Seo13, John H Stone14, Sangeeta Sule15, Robert P Sundel16, Omar I Vitobaldi17, Ann Warner18, Kevin Byram19, Anisha B Dua7, Nedaa Husainat20, Karen E James21, Mohamad Kalot22, Yih Chang Lin23, Jason M Springer4, Marat Turgunbaev24, Alexandra Villa-Forte3, Amy S Turner24, Reem A Mustafa25. 1. University of California, San Francisco. 2. Columbia University, New York, New York. 3. Cleveland Clinic Foundation, Cleveland, Ohio. 4. University of Kansas Medical Center, Kansas City. 5. Mayo Clinic, Jacksonville, Florida. 6. McMaster University, Hamilton, Ontario, Canada. 7. Northwestern University, Chicago, Illinois. 8. Emory University, Atlanta, Georgia. 9. Springfield, Illinois. 10. National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. 11. Children's Mercy Hospital, Kansas City, Missouri. 12. University of Pennsylvania, Philadelphia. 13. Johns Hopkins University, Baltimore, Maryland. 14. Massachusetts General Hospital, Boston. 15. Children's National Hospital, Washington, DC. 16. Boston Children's Hospital, Boston, Massachusetts. 17. Chicago, Illinois. 18. Saint Luke's Health System, Kansas City, Missouri. 19. Vanderbilt University, Nashville, Tennessee. 20. SSM Health-St. Mary's Hospital, St. Louis, Missouri. 21. University of Utah, Salt Lake City. 22. State University of New York at Buffalo. 23. University of South Florida, Tampa. 24. American College of Rheumatology, Atlanta, Georgia. 25. University of Kansas Medical Center, Kansas City, and McMaster University, Hamilton, Ontario, Canada.
Abstract
OBJECTIVE: To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN). METHODS: Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel. RESULTS: We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted. CONCLUSION: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.
OBJECTIVE: To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN). METHODS: Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel. RESULTS: We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted. CONCLUSION: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.
Authors: Tiago Rodrigues; Inês Aguiar-Ricardo; Miguel Nobre Menezes; Joana Rigueira; Rafael Santos; Valter Fonseca; Fausto J Pinto; Ana G Almeida Journal: JACC Case Rep Date: 2022-05-04