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  - 
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  - Morbach, Henner
A1  - Reiser, Christiane
A1  - Girschick, Hermann J.
T1  - New Insights into Adult and Paediatric Chronic Non-bacterial Osteomyelitis CNO
JF  - Current Rheumatology Reports
N2  - Purpose of Review
To describe in detail the clinical synopsis and pathophysiology of chronic non-bacterial osteomyelitis and SAPHO syndrome.

Recent Findings
Chronic non-bacterial osteomyelitis (CNO) has been identified as a disease entity for almost 50 years. This inflammatory bone disorder is characterized by osteolytic as well as hyperostotic/osteosclerotic lesions. It is chronic in nature, but it can present with episodic flairs and phases of remission, which have led to the denomination “chronic recurrent osteomyelitis”, with its severe multifocal form “chronic recurrent multifocal osteomyelitis” (CRMO). For almost three decades, an infectious aetiology had been considered, since especially Propionibacterium acnes had been isolated from bone lesions of individual patients. However, this concept has been challenged since long-term antibiotic therapy did not alter the course of disease and modern microbiological techniques (including PCR) failed to confirm bone infection as an underlying cause. Over recent years, a profound dysregulation of cytokine expression profiles has been demonstrated in innate immune cells of CNO patients. A hallmark of monocytes from CNO patients is the failure to produce immune regulatory cytokines interleukin-10 (IL-10) and IL-19, which have been linked with genetic and epigenetic alterations. Subsequently, a significant upregulation of pro-inflammatory, NLRP3 inflammasome-dependent cytokines (IL-1β and TNF-α), has been demonstrated.

Summary
The current knowledge on CNO, the underlying molecular pathophysiology, and modern imaging strategies are summarized; differential diagnoses, treatment options, outcome measures, as well as quality of life studies are discussed.
KW  - chronic non-bacterial osteomyelitis
KW  - chronic recurrent multifocal osteomyelitis
KW  - bone autoinflammation
KW  - lymphoplasmacellular osteomyelitis
Y1  - 2020
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-232636
SN  - 1523-3774
VL  - 22
ER  -