Associated diseases and their effects on disease course in patients with chronic non-bacterial osteomyelitis:
Betül Öksüz Aydın1, Fatma Aydın2, Özen Taş1
1Division of Pediatric Rheumatology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective:
Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods:
The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed. Patients were divided into two groups according to the presence or absence of comorbidities. The clinical, laboratory, and radiological characteristics and treatments of the groups were compared.
Results:
The study included 40 patients (65% male) diagnosed with CNO. The median (IQR) age at symptom onset was 10 (6.4) and at diagnosis was 11.5 (5.9) years. Fourteen (35%) patients had comorbidities. The comorbidities were FMF (n = 9), IBD (n = 3), uveitis (n = 3), psoriasis (n = 1) and acne conglabatae (n = 2). The group with comorbidities had higher number of bones involved (3 or more bones) (78.6% versus 42.3%) (p = 0.028), and 78.6% of patients with comorbidities received biologic treatment, while only 23.1% of patients without comorbidities were treated with biologics (p = 0.001).
Conclusion:
Familial Mediterranean fever, uveitis, IBD, psoriasis and acne conglabata were found to be the clinical conditions associated with CNO. Patients with CNO who had comorbidities appeared to have a more severe phenotype of the disease accompanied with more bone involvement and requiring more biologic treatment. Key Points • Chronic non-bacterial osteomyelitis (CNO), an auto-inflammatory bone disease, can be seen in association with other inflammatory conditions such as familial Mediterranean fever (FMF), uveitis, inflammatory bowel disease (IBD), psoriasis, and acne conglobata. • If CNO is associated with another inflammatory disease, the number of bones involved may be higher and patients may need more intensive treatments, such as biologics. • CNO may coexist with one or more inflammatory diseases, which may exacerbate the disease phenotype.
Insights
Chronic non-bacterial osteomyelitis (CNO) in patients with comorbidities presents with more bone involvement and requires more intensive biologic treatment. These associated inflammatory conditions, including familial Mediterranean fever, can worsen the CNO disease phenotype.
Area of Science:
- Pediatric Rheumatology
- Autoinflammatory Diseases
- Osteomyelitis
Background:
- Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease.
- CNO is often associated with other chronic inflammatory conditions like familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), inflammatory bowel disease (IBD), and pyoderma gangrenosum.
Purpose of the Study:
- To describe the clinical and follow-up characteristics of pediatric patients diagnosed with CNO.
- To compare the clinical features and treatment outcomes between CNO patients with and without comorbidities.
Main Methods:
- Retrospective review of clinical records of CNO patients from a pediatric rheumatology clinic (2018-2023).
- Patients were categorized into two groups: those with comorbidities and those without.
- Comparison of clinical, laboratory, radiological characteristics, and treatment modalities between the two groups.
Main Results:
- Forty patients with CNO were included; 35% had comorbidities, most commonly FMF (n=9), IBD (n=3), and uveitis (n=3).
- Patients with comorbidities showed a significantly higher incidence of multi-bone involvement (≥3 bones) (78.6% vs. 42.3%, p=0.028).
- A greater proportion of patients with comorbidities required biologic treatment (78.6% vs. 23.1%, p=0.001).
Conclusions:
- Familial Mediterranean fever, uveitis, IBD, psoriasis, and acne conglobata are associated with CNO.
- CNO patients with comorbidities exhibit a more severe disease phenotype, characterized by increased bone involvement and a higher likelihood of needing biologic therapies.
- The coexistence of CNO with other inflammatory diseases can exacerbate its clinical presentation.
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