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Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO)

Please always quote using this URN: urn:nbn:de:bvb:20-opus-125694
  • 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 autoinflammatorySterile 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.show moreshow less

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Metadaten
Author: Christian M. Hedrich, Sigrun R. Hofmann, Jessica Pablik, Henner Morbach, Hermann J. Girschick
URN:urn:nbn:de:bvb:20-opus-125694
Document Type:Journal article
Faculties:Medizinische Fakultät / Kinderklinik und Poliklinik
Language:English
Parent Title (English):Pediatric Rheumatology
ISSN:1546-0096
Year of Completion:2013
Volume:11
Issue:47
Source:Pediatric Rheumatology 2013, 11:47. doi:10.1186/1546-0096-11-47
DOI:https://doi.org/10.1186/1546-0096-11-47
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 618 Gynäkologie, Geburtsmedizin, Pädiatrie, Geriatrie
Tag:CNO; CRMO; DIRA; IL-10; IL-10 expression; Majeed-Syndrome; PAPA; PAPA syndrome; TLR4; TNF-α; bisphosphonate treatment; bone; chronic nonbacterial osteomyelitis; congenital dyserythropoietic anemia; deficiency; disease; hypertrophic osteodystrophy; inflammation; pediatric patients; treatment
Release Date:2016/03/02
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung