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Morbach, H; Hedrich, CM; Beer, M; Girschick, HJ.
Autoinflammatory bone disorders.
Clin Immunol. 2013; 147(3):185-196 Doi: 10.1016/j.clim.2012.12.012
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Abstract:
Autoinflammatory bone disorders are characterized by chronic non-infectious osteomyelitis and inflammation-induced bone resorption and result from aberrant activation of the innate immune system. Sporadic chronic non-bacterial osteomyelitis (CNO) is the most common disease subtype. The clinical picture is highly variable and the exact underlying pathophysiology remains to be determined. Recently, novel insights in the pathophysiology of sterile bone inflammation have been gathered by analyzing patients with rare, monogenic inflammatory diseases. In this overview CNO and Majeed syndrome, cherubism, hypophosphatasia and primary hypertrophic osteoarthropathy will be discussed. For the latter four disorders, a genetic cause affecting bone metabolism and leading to chronic bone inflammation has been described. The exact pathophysiology of CNO remains to be determined. Insights from monogenic autoinflammatory bone diseases and the identification of distinct inflammatory pathways may help to understand the pathogenesis of bone inflammation and inflammation-induced bone resorption in more common diseases.
Find related publications in this database (using NLM MeSH Indexing)
Anemia, Dyserythropoietic, Congenital - genetics Anemia, Dyserythropoietic, Congenital - immunology
Animals -
Bone Diseases - genetics Bone Diseases - immunology
Bone Resorption - genetics Bone Resorption - immunology
Cherubism - genetics Cherubism - immunology
Chronic Disease -
Genetic Predisposition to Disease -
Hereditary Autoinflammatory Diseases - genetics Hereditary Autoinflammatory Diseases - immunology
Humans -
Hypophosphatasia - genetics Hypophosphatasia - immunology
Inflammation - genetics Inflammation - immunology
Mice -
Osteoarthropathy, Primary Hypertrophic - genetics Osteoarthropathy, Primary Hypertrophic - immunology
Osteomyelitis - genetics Osteomyelitis - immunology
Rare Diseases - genetics

Find related publications in this database (Keywords)
Chronic non-bacterial osteomyelitis
Chronic recurrent multifocal osteomyelitis
Hypophosphatasia
Cherubism
Primary hypertrophic osteoarthropathy
Autoinflammation
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