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Chronic Non-Bacterial Osteomyelitis

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Abstract

Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disorder originally reported in the early 1970s. It typically presents in late childhood to early adolescence with bone pain and swelling. The most commonly affected areas include the metaphyses of long bones, the clavicle and the vertebral bodies. There are no diagnostic criteria making CNO a diagnosis of exclusion. While a variety of treatments have been reported to be effective, to date there are no clinical trials upon which a firm evidence-based decision of which treatment to use can be made. Recent work in mice and humans has led to new understanding of both genetic associations and the immunologic mechanisms that appear to be involved in the pathogenesis of CNO.

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Abbreviations

ASC:

Apoptosis-associated speck-like protein containg a CARD

CARRA:

Childhood Arthritis & Rheumatology Research Alliance

Casp1:

Caspase-1

CCL:

Chemokine (C-C motif) ligand

CMO:

Chronic multifocal osteomyelitis

CNO:

Chronic nonbacterial osteomyelitis

CRMO:

Chronic recurrent multifocal osteomyelitis

CRP:

C-reactive protein

DAMP:

Danger-associated molecular pattern

DC:

Dendritic cell

DIRA:

Deficiency of the interleukin-1 receptor antagonist

DMARD:

Disease modifying anti-rheumatic drug

ERK:

Extracellular signal-regulated kinase

ESR:

Erythrocyte sedimentation rate

FBLIM:

Filamin-binding LIM protein

FBP:

Formin binding protein

GM-CSF:

Granulocyte and monocyte colony stimulating factor

IL:

Interleukin

IL-RN:

Interleukin-1 receptor antagonist

LPIN2:

Lipin-2, a phosphatidate phosphatase

MAP kinase:

Mitogen-activated protein kinase

MCP-1:

Monocyte chemotactic protein 1 (also CCL2)

MIG:

Monokine induced by gamma interferon (also CXCL9)

MIP:

Macrophage inflammatory protein

MRI:

Magnetic resonance imaging

MTX:

Methotrexate

NF-κB:

Nuclear factor-κB

NK cells:

Natural killer cells

NLRP:

NACHT, LRR and PYD domains-containing protein

NSAIDs:

Nonsteroidal anti-inflammtory drugs

PAMP:

Pathogen-associated molecular pattern

PAPA:

Pyoderma gangrenosum and acne syndrome

PBMC:

Peripheral blood mononuclear cells

PRRs:

Pattern recognition receptors

Pstpip:

Proline-serine-threonine phosphatase-interacting protein

RANK:

Receptor activator of nuclear factor-κB

RANKL:

Receptor activator of nuclear factor-κB ligand

RANTES:

Regulated on activation, normal T cell expressed and secreted protein (also CCL5)

SAPHO:

Synovitis acne pustulosis hyperostosis osteitis syndrome

STAT:

Signal transducer and activator of transcription

sJIA:

Systemic juvenile idiopathic arthritis

TIRM:

Turbo inversion recovery measurement

TNF:

Tumor necrosis factor

VAS:

Visual analogue scale

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Correspondence to Christian M. Hedrich .

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Hedrich, C.M., Girschick, H.J. (2019). Chronic Non-Bacterial Osteomyelitis. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_31

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