An International Consensus Exercise to Develop Candidate Items for Classification Criteria in Relapsing Polychondritis

Marcela A. Ferrada¹, Peter A. Merkel², Keith A. Sikora³, Robert Colbert⁴, Chikashi Terao⁵, Hajime Yoshifuji⁶, Toshiki Nakajima⁷, Aman Sharma⁸, Shirish Sangle⁹, Dax Rumsey¹⁰, Christian Pagnoux¹¹, Larry Young¹², Colleen K. Correll¹³, Fabien Maldonado¹⁴, Robert Lebovics¹⁵, Alexander Gelbard¹⁴, Philip Zapata¹⁶, Allen Clint¹⁷, Otis Rickman¹⁴, Nitin Seam¹⁷, Guillaume Moulis¹⁸, Nathalie Costedoat-Chalumeau¹⁹, Clement J. Michet Jr.²⁰, James D. Katz²¹ and Peter C. Grayson²¹, ¹NIAMS, National Institues of Health, Bethesda, MD, ²Division of Rheumatology, University of Pennsylvania, Philadelphia, MN, ³Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁵Departments of Genetics and Rheumatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, ⁶Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, ⁷Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ⁸Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁹Louise Coote Lupus Unit, Rheumatology and Lupus, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, United Kingdom, ¹⁰Stollery Children's Hospital, Edmonton, AB, Canada, ¹¹Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ¹²Medicine, University of Miami Miller School of Medicine, Miami, FL, ¹³University of Minnesota, Minneapolis, MN, ¹⁴Vanderbilt University Medical Center, Nashville, TN, ¹⁵Mt. Sinai St. Luke's Hospitals, New York, NY, ¹⁶The George Washington University School of Medicine and Health Sciences, Washington, DC, ¹⁷National Institutes of Health, Bethesda, MD, ¹⁸CIC 1436, Toiulouse, France, ¹⁹Service de médecine interne Pôle médecine, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares de l’île de France, Paris, France, ²⁰Division of Rheumatology, Mayo Clinic, Rochester, MN, ²¹National Institute of Arthritis, Musculoskeletal and Skin Disease (NIAMS), Bethesda, MD

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Miscellaneous

Session Information

Date: Tuesday, November 7, 2017

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster II

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

There are no validated classification criteria for relapsing polychondritis (RP). Given that some manifestations of RP are organ- or life-threatening, it is imperative that classification criteria in RP have adequate sensitivity to identify cases early during the disease. The objective of this study was to use consensus procedures to select a set of items with potential utility to classify RP, focusing on items related to disease activity and organ damage.

Methods:

An international, multidisciplinary group of physicians with experience managing RP was formed. Based on a systematic review of publications reporting on observational cohorts, case series, and case reports data in RP, combined with clinical experience, a list of potential candidate items for classification criteria was generated. All group members were invited to participate in an online Delphi exercise for item reduction. Survey participants rated each potential candidate item on a scale of 1-10 (ranging from 1=completely inappropriate to 10=completely appropriate for inclusion as a potential classification criteria item in RP). Items with a median response score of ≤4 were eliminated from further consideration. Items with a median score of ≥7 were retained. Items with a median score >4 and <7 were termed indeterminate and will be subject to 1-2 additional Delphi rounds

Results:

A list of 142 candidate items was generated related to patient-reported symptoms, physician-observed findings, laboratory, and imaging assessments in RP. The first round of the Delphi survey led to a response rate within 16 days of 81% (25/31). Respondents were from 6 countries (United States=15; France=3; Japan=3; Canada=2; United Kingdom=1; India=1) representing multiple subspecialties (Otolaryngology=4; Pulmonology=3; Adult Rheumatology=14; Pediatric Rheumatology=4). Based on survey responses, 49 items were retained, 34 were eliminated, and 59 were indeterminate. Most retained items were related to the respiratory, head/eyes/ears/nose/throat, and musculoskeletal systems (Figure). Items related to damage (e.g. deformed ear, tracheomalacia) were consistently rated higher than features of active disease (but non-damage) (e.g. red ear, anterior neck tenderness). The only laboratory items retained were a negative test for anti-neutrophil cytoplasmic antibody and a positive test for anti-collagen II antibody.

Conclusion:

This consensus exercise generated a preliminary set of candidate items for use in developing classification criteria for RP through prospective data collection. The final rounds of the Delphi will help refine what will be an extensive set of candidate items related to disease activity or damage. Development of classification criteria that represent the broad spectrum of clinical presentations in RP will facilitate the design and implementation of clinical trials in this complex disease.

Disclosure: M. A. Ferrada, None; P. A. Merkel, Actelion, Alexion, Boston Pharm., Bristol-Myers Squibb, ChemoCentryx, Genzyme/Sanofi, GlaxoSmithKline, Genentech/Roche, InflaRx, PrincipioBio, Proteon, Seattle Genetics, 5,Actelion, Bristol-Myers Squibb, CaridianBCT, Celgene, ChemoCentryx, Genentech/Roche, GlaxoSmithKline, Kypha, MedImmune/AstraZeneca, 2,American College of Rheumatology European League Against Rheumatism National Institutes of Health: NHLBI, NIAMS, NIAID, NCATS, ORDR US Food and Drug Administration The Patient-Centered Outcomes Research Institute The Vasculitis Foundation, 2; K. A. Sikora, None; R. Colbert, None; C. Terao, None; H. Yoshifuji, None; T. Nakajima, None; A. Sharma, None; S. Sangle, None; D. Rumsey, None; C. Pagnoux, None; L. Young, None; C. K. Correll, None; F. Maldonado, None; R. Lebovics, None; A. Gelbard, None; P. Zapata, None; A. Clint, None; O. Rickman, None; N. Seam, None; G. Moulis, None; N. Costedoat-Chalumeau, None; C. J. Michet Jr., None; J. D. Katz, None; P. C. Grayson, None.

To cite this abstract in AMA style:

Ferrada MA, Merkel PA, Sikora KA, Colbert R, Terao C, Yoshifuji H, Nakajima T, Sharma A, Sangle S, Rumsey D, Pagnoux C, Young L, Correll CK, Maldonado F, Lebovics R, Gelbard A, Zapata P, Clint A, Rickman O, Seam N, Moulis G, Costedoat-Chalumeau N, Michet CJ Jr., Katz JD, Grayson PC. An International Consensus Exercise to Develop Candidate Items for Classification Criteria in Relapsing Polychondritis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/an-international-consensus-exercise-to-develop-candidate-items-for-classification-criteria-in-relapsing-polychondritis/. Accessed .

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