Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies

doi:10.3899/jrheum.160256

Review

. 2016 Nov;43(11):1956-1964.

doi: 10.3899/jrheum.160256. Epub 2016 Sep 1.

Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies

Sigrun R Hofmann^{1

2}, Anja Schnabel^{1

2}, Angela Rösen-Wolff^{1

2}, Henner Morbach^{1

2}, Hermann J Girschick^{1

2}, Christian M Hedrich^{3

4}

Affiliations

¹ From the Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden; Department of Pediatrics, University of Würzburg, Würzburg; Children's Hospital, Vivantes Klinikum-Friedrichshain, Berlin, Germany.
² S.R. Hofmann, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Schnabel, MD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Rösen-Wolff, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; H. Morbach, MD, PhD, Department of Pediatrics, University of Würzburg; H.J. Girschick, MD, PhD, Children's Hospital, Vivantes Klinikum-Friedrichshain; C.M. Hedrich, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden.
³ From the Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden; Department of Pediatrics, University of Würzburg, Würzburg; Children's Hospital, Vivantes Klinikum-Friedrichshain, Berlin, Germany. Christian.hedrich@uniklinikum-dresden.de.
⁴ S.R. Hofmann, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Schnabel, MD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Rösen-Wolff, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; H. Morbach, MD, PhD, Department of Pediatrics, University of Würzburg; H.J. Girschick, MD, PhD, Children's Hospital, Vivantes Klinikum-Friedrichshain; C.M. Hedrich, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden. Christian.hedrich@uniklinikum-dresden.de.

PMID: 27585682
DOI: 10.3899/jrheum.160256

Review

Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies

Sigrun R Hofmann et al. J Rheumatol. 2016 Nov.

. 2016 Nov;43(11):1956-1964.

doi: 10.3899/jrheum.160256. Epub 2016 Sep 1.

Authors

Sigrun R Hofmann^{1

2}, Anja Schnabel^{1

2}, Angela Rösen-Wolff^{1

2}, Henner Morbach^{1

2}, Hermann J Girschick^{1

2}, Christian M Hedrich^{3

4}

Affiliations

¹ From the Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden; Department of Pediatrics, University of Würzburg, Würzburg; Children's Hospital, Vivantes Klinikum-Friedrichshain, Berlin, Germany.
² S.R. Hofmann, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Schnabel, MD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Rösen-Wolff, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; H. Morbach, MD, PhD, Department of Pediatrics, University of Würzburg; H.J. Girschick, MD, PhD, Children's Hospital, Vivantes Klinikum-Friedrichshain; C.M. Hedrich, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden.
³ From the Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden; Department of Pediatrics, University of Würzburg, Würzburg; Children's Hospital, Vivantes Klinikum-Friedrichshain, Berlin, Germany. Christian.hedrich@uniklinikum-dresden.de.
⁴ S.R. Hofmann, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Schnabel, MD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; A. Rösen-Wolff, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden; H. Morbach, MD, PhD, Department of Pediatrics, University of Würzburg; H.J. Girschick, MD, PhD, Children's Hospital, Vivantes Klinikum-Friedrichshain; C.M. Hedrich, MD, PhD, Pediatric Rheumatology and Immunology, Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden. Christian.hedrich@uniklinikum-dresden.de.

PMID: 27585682
DOI: 10.3899/jrheum.160256

Abstract

Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disorder, covering a clinical spectrum with asymptomatic inflammation of single bones at the one end, and chronic recurrent multifocal osteomyelitis (CRMO) at the other end. The exact molecular pathophysiology of CNO remains largely unknown. Provided familial clusters and the association with inflammatory disorders of the skin and intestine suggest a genetic predisposition. Recently, profound dysregulation of cytokine responses was demonstrated in CRMO. Failure to produce antiinflammatory cytokines interleukin (IL)-10 and IL-19 contributes to activation of inflammasomes and subsequent IL-1β release. In IL-10-deficient and in CNO-prone chronic multifocal osteomyelitis mice, IL-1β was linked to bone inflammation. Further, alterations to the gut microbiome were suggested in contributing to IL-1β release from innate immune cells in mice, offering an interesting target in the search for molecular mechanisms in CNO. Here, we summarize clinical presentation and treatment options in CNO/CRMO, current pathophysiological concepts, available mouse models, and promising future scientific directions.

Keywords: CHRONIC NONBACTERIAL OSTEOMYELITIS; CHRONIC RECURRENT MULTIFOCAL OSTEOMYELITIS; CYTOKINE; INFLAMMATION; TREATMENT.

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Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies

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Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies

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