Comparison of Clinicopathologic and Imaging Features Between Chronic Nonbacterial Osteomyelitis and Its Mimickers: A Multi-national 450 Case-Control Study

Yongdong Zhao¹, Raymond Naden², Melissa Oliver³, Zhaoyi Wang⁴, Eveline Wu⁵, Cassyanne Aguiar⁶, Jonathan Akikusa⁷, Ozge Basaran⁸, Kevin Cain¹, Martina Capponi⁹, Nathan Donaldson¹⁰, Emily Fox¹¹, Antonella Insalaco¹², Annette Jansson¹³, Ummusen Kaya Akca¹⁴, Tzielan Lee¹⁵, Edoardo Marrani¹⁶, Kamran Mahmood¹⁷, Elizabeth Murray¹⁸, Farzana Nuruzzaman¹⁹, Karen Onel²⁰, Manuela Pardeo¹², Lauren Potts²¹, Nathan Rogers¹⁰, Anja Schnabel²², Gabriele Simonini²³, Jennifer Soep¹⁰, Sara Stern²⁴, Alexander Theos²⁵, Yujuan Zhang²⁶, Polly Ferguson²⁷, Christian Hedrich²⁸, Fatma Dedeoglu²⁹, Hermann Girschick³⁰, Ronald Laxer³¹ and Seza Ozen³², ¹University of Washington, Seattle, WA, ²Department of Medicine, Middlemore Hospital, Auckland, Auckland, New Zealand, ³Department of Pediatric Rheumatology, Riley Children's Hospital, Carmel, IN, ⁴University of Washington, Bothell, WA, ⁵UNC Chapel Hill, Chapel Hill, NC, ⁶Children's Hospital of the King's Daughters/EVMS, Norfolk, VA, ⁷PRINTO, Istituto Giannina Gaslini, Genova, Italy, ⁸Hospital for Special Surgery, New York City, NY, ⁹IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ¹⁰Children's Hospital Colorado, Aurora, CO, ¹¹Children's Mercy Kansas City, Kansas City, MO, ¹²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹³Dr. von Hauner Children's Hospital, Munich, Germany, ¹⁴Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Ankara, Turkey, ¹⁵Stanford University School of Medicine, Palo Alto, CA, ¹⁶University of Florence, Firenze, Italy, ¹⁷Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, England, United Kingdom, ¹⁸CRMO Patient Partner, New York, NY, ¹⁹Stony Brook Children's Hospital, Stony Brook, NY, ²⁰Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, ²¹CRMO Patient Partner, Fort Collins, CO, ²²Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany, ²³Anna Meyer Children's Hospital, Firenze, Italy, ²⁴University of Utah and Primary Children's Hospital, Salt Lake City, UT, ²⁵Georgetown University, DERWOOD, MD, ²⁶Tufts Medical Center, Boston, MA, ²⁷University of Iowa Carver College of Medicine, Iowa City, IA, ²⁸University of Liverpool, Liverpool, United Kingdom, ²⁹Boston Children's Hospital, Boston, MA, ³⁰Vivantes Children’s Hospital, Wuerzburg, Germany, ³¹The Hospital for Sick Children, Toronto, ON, Canada, ³²Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey

Meeting: ACR Convergence 2020

Keywords: classification criteria, Imaging, registry

Session Information

Date: Sunday, November 8, 2020

Title: Pediatric Rheumatology – Clinical Poster II: Systemic JIA, Autoinflammatory, & Scleroderma

Session Type: Poster Session C

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

Background/Purpose: Chronic nonbacterial osteomyelitis (CNO)/chronic recurrent multifocal osteomyelitis (CRMO) predominantly affects children and young adults. Classification criteria are not available and diagnostic criteria that have been suggested have not been validated. We previously identified candidate items for the development of classification criteria.

Methods: We aimed to refine candidate items for pediatric classification criteria for CNO by comparing clinical, laboratory and imaging features of CNO against mimicking conditions. International multicentre collection of clinical and investigational features of cases with CNO or mimicker diseases with at least 12 months follow-up was conducted through a REDCap online database. Prevalence ratios of each collected item between CNO and mimickers were calculated. A p value of <.05 was considered significant.

Results: 450 cases were collected from 20 centers in 7 countries and 4 continents. Cases were filtered based on indicated confidence levels of diagnosis for CNO or mimickers using a cut-off of +/- 2 (moderately confident). 264 (59%) CNO cases and 145 (32%) mimicker controls were used for analysis. 41 (9%) cases were excluded. Key findings are summarized in Table 1.

When compared to mimicker diagnoses, CNO patients were predominantly female, more frequently exhibited intermittent versus continued pain (especially of neck, back and upper torso), but less commonly had fever. Clavicular swelling was more common in CNO, while active arthritis was less common as compared to controls. CNO patients more frequently had whole body imaging (usually whole-body MRI). Symmetric patterns of bone lesions were more common in CNO. CNO frequently involved the thoracic spine, clavicle, sternum/manubrium, pelvic bones, bilateral femur, bilateral tibia, unilateral fibula, and foot bones. Imaging features that are concerning for infection or malignancy (including cortical bone disruption, disorganized bone formation, mass structure, marrow infiltrate, abscess or geographic appearance) were less common in CNO. Lastly, complete and sustained response to antibiotic treatment was less frequent in CNO patients.

Conclusion: Using a case-based approach, key features of CNO were identified to support the development of classification criteria. Next steps will include expert panel discussions and a 1000Minds exercise.

Disclosure: Y. Zhao, Novartis, 1; R. Naden, ACR/EULAR, 1, 2; M. Oliver, None; Z. Wang, None; E. Wu, None; C. Aguiar, None; J. Akikusa, None; O. Basaran, None; K. Cain, None; M. Capponi, None; N. Donaldson, None; E. Fox, None; A. Insalaco, None; A. Jansson, None; U. Kaya Akca, None; T. Lee, None; E. Marrani, None; K. Mahmood, None; E. Murray, None; F. Nuruzzaman, None; K. Onel, None; M. Pardeo, None; L. Potts, None; N. Rogers, None; A. Schnabel, None; G. Simonini, Novartis, 5, 8, AbbVie, 5, 8; J. Soep, None; S. Stern, None; A. Theos, None; Y. Zhang, None; P. Ferguson, NIH/NIAMS R01AR059703, 1, Novartis, 1; C. Hedrich, None; F. Dedeoglu, Novartis, 1; H. Girschick, None; R. Laxer, Eli Lilly Canada, 1, Novartis, 1, Sanofi, 1; S. Ozen, SOBI, 1, Novartis, 1.

To cite this abstract in AMA style:

Zhao Y, Naden R, Oliver M, Wang Z, Wu E, Aguiar C, Akikusa J, Basaran O, Cain K, Capponi M, Donaldson N, Fox E, Insalaco A, Jansson A, Kaya Akca U, Lee T, Marrani E, Mahmood K, Murray E, Nuruzzaman F, Onel K, Pardeo M, Potts L, Rogers N, Schnabel A, Simonini G, Soep J, Stern S, Theos A, Zhang Y, Ferguson P, Hedrich C, Dedeoglu F, Girschick H, Laxer R, Ozen S. Comparison of Clinicopathologic and Imaging Features Between Chronic Nonbacterial Osteomyelitis and Its Mimickers: A Multi-national 450 Case-Control Study [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/comparison-of-clinicopathologic-and-imaging-features-between-chronic-nonbacterial-osteomyelitis-and-its-mimickers-a-multi-national-450-case-control-study/. Accessed .

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