TY  - JOUR
A1  - Hedrich, Christian M.
A1  - Hofmann, Sigrun R.
A1  - Pablik, Jessica
A1  - Morbach, Henner
A1  - Girschick, Hermann J.
T1  - Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO)
JF  - Pediatric Rheumatology
N2  - Sterile bone inflammation is the hallmark of autoinflammatory bone disorders, including chronic nonbacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO). Autoinflammatory osteopathies are the result of a dysregulated innate immune system, resulting in immune cell infiltration of the bone and subsequent osteoclast differentiation and activation. Interestingly, autoinflammatory bone disorders are associated with inflammation of the skin and/or the intestine. In several monogenic autoinflammatory bone disorders mutations in disease-causing genes have been reported. However, regardless of recent developments, the molecular pathogenesis of CNO/CRMO remains unclear.

Here, we discuss the clinical presentation and molecular pathophysiology of human autoinflammatory osteopathies and animal models with special focus on CNO/CRMO. Treatment options in monogenic autoinflammatory bone disorders and CRMO will be illustrated.
KW  - TNF-α
KW  - PAPA
KW  - DIRA
KW  - Majeed
KW  - CNO
KW  - CRMO
KW  - bone
KW  - inflammation
KW  - IL-10
KW  - treatment
KW  - TLR4
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-132456
VL  - 11
IS  - 47
ER  - 
TY  - JOUR
A1  - Hedrich, Christian M.
A1  - Hofmann, Sigrun R.
A1  - Pablik, Jessica
A1  - Morbach, Henner
A1  - Girschick, Hermann J.
T1  - Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO)
JF  - Pediatric Rheumatology
N2  - Sterile bone inflammation is the hallmark of autoinflammatory bone disorders, including chronic nonbacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO). Autoinflammatory osteopathies are the result of a dysregulated innate immune system, resulting in immune cell infiltration of the bone and subsequent osteoclast differentiation and activation. Interestingly, autoinflammatory bone disorders are associated with inflammation of the skin and/or the intestine. In several monogenic autoinflammatory bone disorders mutations in disease-causing genes have been reported. However, regardless of recent developments, the molecular pathogenesis of CNO/CRMO remains unclear. Here, we discuss the clinical presentation and molecular pathophysiology of human autoinflammatory osteopathies and animal models with special focus on CNO/CRMO. Treatment options in monogenic autoinflammatory bone disorders and CRMO will be illustrated.
KW  - bisphosphonate treatment
KW  - IL-10 expression
KW  - TNF-α
KW  - IL-10
KW  - inflammation
KW  - bone
KW  - CRMO
KW  - CNO
KW  - DIRA
KW  - PAPA
KW  - Majeed-Syndrome
KW  - disease
KW  - deficiency
KW  - pediatric patients
KW  - treatment
KW  - TLR4
KW  - PAPA syndrome
KW  - hypertrophic osteodystrophy
KW  - chronic nonbacterial osteomyelitis
KW  - congenital dyserythropoietic anemia
Y1  - 2013
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-125694
SN  - 1546-0096
VL  - 11
IS  - 47
ER  -