RT Journal Article
SR Electronic
T1 Chronic Nonbacterial Osteomyelitis: Pathophysiological Concepts and Current Treatment Strategies
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1956
OP 1964
DO 10.3899/jrheum.160256
VO 43
IS 11
A1 Sigrun R. Hofmann
A1 Anja Schnabel
A1 Angela Rösen-Wolff
A1 Henner Morbach
A1 Hermann J. Girschick
A1 Christian M. Hedrich
YR 2016
UL http://www.jrheum.org/content/43/11/1956.abstract
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.