Chronic non-bacterial osteomyelitis M86.3

Last updated on: 29.10.2020

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Synonym(s)

Chronic Nonbacterial Osteomyelitis; Chronic recurrent multifocal osteomyelitis; CNO; CRMO; OMIM: 259680; Recurrent multifocal osteomyelitis

History
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Giedion et al. 1972

Definition
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Chronic, non-bacterial osteomyelitis, also known as chronic recurrent multifocal osteomyelitis - CRMO - is a chronic, autoinflammatory systemic disease affecting bones, eyes, gastrointestinal tract and, to varying degrees (up to 30%), the skin.

Occurrence/Epidemiology
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According to various studies, the prevalence of CNO/CRMO is approximately between 1: 160,000 and 1:2,000,000; incidence between 1:250,000 and 1:1,000,000; w>m;

Etiopathogenesis
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A dysbalance of cytokine expression (disturbance of the IL-1 signaling pathway) by innate immune cells has been demonstrated (Cox AJ et al. 2017; Hofmann SR et al. 2017).

Manifestation
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childhood, adolescence

Clinical features
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The clinical picture is characterized by multiple, painfully swollen bone foci, especially in the metaphyses of the long bones, in the pelvis, in the shoulder girdle and in the spine. The skin lesions present as psoriasis or palmoplantar pustulosis .

Imaging
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MRI: Detection of +bone edema followed by osteolytic or sclerotic, later hyperostotic bone lesions (Zhao Y et al. 2018). There are also inflammatory reactions of the periosteum and soft tissues.

Laboratory
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Non-specific (Zhao Y et al. 2018).

Histology
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Biopsies of bone lesions: microbiologically sterile; histologically there are non-specific inflammatory changes.

Diagnosis
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The diagnosis is based on an overview of clinical (bone pain, psoriatic or psoriasiform skin symptoms), radiological and histological findings.

Differential diagnosis
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  • Acute or subacute bacterial osteomyelitis
  • Juvenile idiopathic arthritis
  • Hypophosphatasia
  • Langerhans cell histiocytosis
  • Malignancies such as osteosarcoma, Ewing's sarcoma, neuroblastoma, rhabdomyosarcoma, leukemias and lymphomas.
  • Syndromes with CNO/CRMO as component like:
    • Majeed Syndrome
    • PAPA Syndrome
    • DIRA syndrome (sterile multifocal osteomyelitis with periostitis and pustulosis)

Therapy
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Progression/forecast
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Recurrent course. Complete healing of the disease process is possible.

Possible are bone deformities with long-term impairment (vertebra plana, hyperostosis, pain syndrome).

The occurrence of a spondyloarthropathy in the long-term course has also been described.

Literature
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  1. Cox AJ et al (2017) Chronic Recurrent Multifocal Osteomyelitis and Related Diseases-Update on Pathogenesis. Curr Rheumatol Rep 19:18.
  2. Gicchino MF et al. (2018) Chronic recurrent multifocal osteomyelitis: a case report.Ital J Pediatr 44:26.
  3. Hofmann SR et al (2017) Chronic Recurrent Multifocal Osteomyelitis (CRMO): Presentation, Pathogenesis, and Treatment. Curr Osteoporos Rep 15:542-554.
  4. Roderick MR et al. (2016) Chronic recurrent multifocal osteomyelitis (CRMO) - advancing the diagnosis. Pediatr Rheumatol Online J 14:47.
  5. Roderick MR et al. (2018) Chronic recurrent multifocal osteomyelitis in children and adults: current understanding and areas for development. Rheumatology (Oxford) 57:41-48.
  6. Zhao Y et al. (2018) Chronic Nonbacterial Osteomyelitis and Chronic Recurrent Multifocal Osteomyelitis in Children. Pediatr Clin North Am 65:783-800.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020