PT  - JOURNAL ARTICLE
AU  - Sigrun R. Hofmann
AU  - Anja Schnabel
AU  - Angela Rösen-Wolff
AU  - Henner Morbach
AU  - Hermann J. Girschick
AU  - Christian M. Hedrich
TI  - Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies
AID  - 10.3899/jrheum.160256
DP  - 2016 Nov 01
TA  - The Journal of Rheumatology
PG  - 1956--1964
VI  - 43
IP  - 11
4099  - http://www.jrheum.org/content/43/11/1956.short
4100  - http://www.jrheum.org/content/43/11/1956.full
SO  - J Rheumatol2016 Nov 01; 43
AB  - 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.