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EULAR/American College of Rheumatology Classification Criteria for Pediatric Chronic Nonbacterial Osteomyelitis
Yongdong Zhao1, Melissa S Oliver2, Anja Schnabel3
1Pediatric Rheumatology, Department of Pediatrics, University of Washington School of Medicine, Seattle.
Insights
New classification criteria for pediatric chronic nonbacterial osteomyelitis (CNO) were developed and validated. These criteria offer high specificity and good sensitivity for diagnosing CNO in children.
Area of Science:
- Rheumatology
- Pediatrics
- Immunology
Background:
- Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease in children.
- Accurate classification is crucial for diagnosis and management.
- Existing criteria lacked international consensus and validation.
Purpose of the Study:
- To develop and validate international classification criteria for pediatric CNO.
- To establish a standardized diagnostic framework for CNO.
- To facilitate future research and improve patient outcomes.
Main Methods:
- International collaboration involving pediatric rheumatologists.
- Multi-phase approach: surveys, Delphi, nominal group technique, multicriteria decision analysis.
- Validation in two large patient cohorts (441 and 514 patients).
Main Results:
- New EULAR/ACR criteria include radiographic/MRI findings, bone pain, and exclusion criteria.
- Weighted additive criteria across clinical and laboratory domains.
- A total score ≥55 required for CNO classification.
- Achieved 82% sensitivity and 98% specificity in the validation cohort.
Conclusions:
- The developed EULAR/ACR criteria represent current understanding of pediatric CNO.
- These criteria are highly specific and sensitive, aiding accurate diagnosis.
- They provide a robust foundation for future CNO research and clinical practice.
Objective:
To develop and validate classification criteria for pediatric chronic nonbacterial osteomyelitis (CNO) jointly supported by EULAR and the American College of Rheumatology (ACR).
Methods:
This international initiative had 4 phases: (1) candidate items were proposed in a survey of pediatric rheumatologists, (2) criteria definition and reduction by Delphi and nominal group technique exercises, (3) criteria weighting using multicriteria decision analysis, and (4) refinement of weights and threshold score in a development cohort of 441 patients and validation in another cohort of 514 patients.
Results:
The new EULAR/ACR classification criteria for CNO require typical radiographic or magnetic resonance imaging findings and bone pain as an obligatory entry criterion and exclusion criteria of malignancy, infection, vitamin C deficiency, and hypophosphatasia, followed by additive weighted criteria in 5 clinical (site of bone lesions, pattern of bone lesions, age at onset, coexisting conditions, fever) and 4 pathology/laboratory domains (bone biopsy findings if done, anemia, C-reactive protein level, and erythrocyte sedimentation rate). A total score ≥55 is required for classification as CNO. The new criteria had a sensitivity of 82% and specificity of 98% in the validation cohort.
Conclusion:
These new classification criteria for pediatric CNO developed with international input reflect current views about CNO, have high specificity and good sensitivity, and provide a key foundation for future CNO research.
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