Abstract
ObjectiveTo assess the first-year features of patients with chronic nonbacterial osteomyelitis (CNO).MethodsPatients with a diagnosis of CNO, disease duration of under 13 months, and first registration in the German National Pediatric Rheumatologic Database (NPRD) between 2009 and 2018 were included in this cross-sectional analysis.ResultsOf 774 documented patients, 62.8% were female, and all patients had a median age of 11 years. The most affected clinical sites were the tibia (29.7%), pelvis (28.0%), and femur (27.8%). HLA-B27 was positive in 48 of 314 analyzed patients (15.3%). In 406 patients, an X-ray was performed at the first visit; X-ray results showed osteosclerosis/−lysis in 34.0% and hyperostosis in 14.5% of the patients. MRI scans (focal and whole-body scans) were performed in 648 patients, and 81.5% showed a positive TIRM/STIR signal. A total of 84.7% of the patients were administered nonsteroidal anti-inflammatory drugs, 9.6% were administered oral glucocorticoids, 10.8% were administered disease-modifying anti-rheumatic drugs (DMARDs), and 6.1% were administered bisphosphonates. An evaluation of the patient’s questionnaire showed an overall well-being (NRS 0–10) of 2.0. The PedCNO disease “activity” score revealed a 70% improvement in variables in 43% of patients in the initial 1-year follow-up. Copresentation with diagnostic criteria of pediatric enthesitis-related arthritis was rare.ConclusionTo our knowledge, the NPRD cohort seemed to be the largest cohort of children and adolescents suffering from CNO worldwide. Most patients were treated effectively with NSAIDs, and only a small group of patients was administered additional medication. The patient-defined measures of disease activity had a moderate impact on patients’ daily lives.Trial registrationNot applicable.
Highlights
Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory disease of the bone of unknown etiology
Over 10 years until 2018, we evaluated different features of CNO, including the following items: demographic, clinical and laboratory parameters (ESR, CrP, Human leukocyte antigen (HLA)-B27), biopsy, duration and activity of the disease, comanifestations, and radiological diagnostics
Course of disease—reach of inactive disease, treatment modalities, the Pediatric chronic nonbacterial osteomyelitis follow-up treatment score (PedCNO) score [18], patient-reported overall well-being, and physicianreported disease severity were analyzed
Summary
Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory disease of the bone of unknown etiology. The most severe and/or recurrent form of CNO is referred to as chronic recurrent multifocal osteomyelitis. CNO affects the metaphyses of the long bones, but inflammation can be found in the patient’s whole skeleton with the exception of the neurocranium [2]. CNO is a multisystemic disease, and organs other than the bone and joints, predominately the skin with psoriasiform or pustular eruptions and the intestines with chronic inflammatory bowel diseases, can be affected [2, 4]. Confirming the diagnosis of CNO can be challenging, as the list of differential diagnoses is long and no specific laboratory markers exist far. International efforts to improve classification, diagnosis, and treatment are on the way and may support clinical diagnosis and care in the future [11]
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