Sterile osteomyelitis: a cardinal sign of autoinflammation

Autoinflammatory bone disorders (ABDs) are characterized by sterile bone inflammation stemming from dysregulated innate immune responses. This review focuses on the occurrence of sterile osteo­myelitis in ABDs and related diseases, notably chronic nonbacterial osteomyelitis (CNO) and its sporadic an...

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Main Authors: Tiago Borges, João Santos, Sérgio Silva
Format: Article
Language:English
Published: Termedia Publishing House 2024-12-01
Series:Rheumatology
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Online Access:https://reu.termedia.pl/Sterile-osteomyelitis-a-cardinal-sign-of-autoinflammation,196595,0,2.html
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author Tiago Borges
João Santos
Sérgio Silva
author_facet Tiago Borges
João Santos
Sérgio Silva
author_sort Tiago Borges
collection DOAJ
description Autoinflammatory bone disorders (ABDs) are characterized by sterile bone inflammation stemming from dysregulated innate immune responses. This review focuses on the occurrence of sterile osteo­myelitis in ABDs and related diseases, notably chronic nonbacterial osteomyelitis (CNO) and its sporadic and monogenic forms, such as deficiency of the interleukin-1 (IL-1) receptor antagonist, Majeed syndrome, CNO related to FBLIM1 mutation, and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA syndrome). Additionally, other autoinflammatory disorders (AIDs) are discussed, including classical periodic fever syndromes (e.g., familial Mediterranean fever, cryopyrin- associated periodic syndromes), monogenic rare AIDs (such as hyperostosis-hyperphosphatemia syndrome, H syndrome, interferonopathies, and Singleton-Merten’s syndrome), polygenic AIDs with bone involvement (e.g., Schnitzler’s syndrome, systemic juvenile idiopathic arthritis, adult-onset Still’s disease, and calcium pyrophosphate deposition disease), and bone dysplastic syndromes. Sterile osteomyelitis emerges as a cardinal sign of autoinflammation, aiding clinicians in both dia­gnosis and management of ABDs. Treatment typically involves tumor necrosis factor inhibitors or IL-1 antagonists.
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series Rheumatology
spelling doaj-art-cf605704aecd4825965855a0e6730c8c2025-01-27T11:21:27ZengTermedia Publishing HouseRheumatology0034-62332084-98342024-12-0162647548810.5114/reum/196595196595Sterile osteomyelitis: a cardinal sign of autoinflammationTiago Borges0https://orcid.org/0000-0002-8271-9865João Santos1Sérgio Silva2https://orcid.org/0000-0002-7073-1867Department of Internal Medicine, Trofa Saúde Hospital Privado em Gaia, Vila Nova de Gaia, PortugalUSF BarcelSaúde, Barcelos, PortugalDepartment of Internal Medicine, Trofa Saúde Hospital Privado em Gaia, Vila Nova de Gaia, PortugalAutoinflammatory bone disorders (ABDs) are characterized by sterile bone inflammation stemming from dysregulated innate immune responses. This review focuses on the occurrence of sterile osteo­myelitis in ABDs and related diseases, notably chronic nonbacterial osteomyelitis (CNO) and its sporadic and monogenic forms, such as deficiency of the interleukin-1 (IL-1) receptor antagonist, Majeed syndrome, CNO related to FBLIM1 mutation, and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA syndrome). Additionally, other autoinflammatory disorders (AIDs) are discussed, including classical periodic fever syndromes (e.g., familial Mediterranean fever, cryopyrin- associated periodic syndromes), monogenic rare AIDs (such as hyperostosis-hyperphosphatemia syndrome, H syndrome, interferonopathies, and Singleton-Merten’s syndrome), polygenic AIDs with bone involvement (e.g., Schnitzler’s syndrome, systemic juvenile idiopathic arthritis, adult-onset Still’s disease, and calcium pyrophosphate deposition disease), and bone dysplastic syndromes. Sterile osteomyelitis emerges as a cardinal sign of autoinflammation, aiding clinicians in both dia­gnosis and management of ABDs. Treatment typically involves tumor necrosis factor inhibitors or IL-1 antagonists.https://reu.termedia.pl/Sterile-osteomyelitis-a-cardinal-sign-of-autoinflammation,196595,0,2.htmlinnate immunityosteomyelitischronic recurrent multifocal osteomyelitis
spellingShingle Tiago Borges
João Santos
Sérgio Silva
Sterile osteomyelitis: a cardinal sign of autoinflammation
Rheumatology
innate immunity
osteomyelitis
chronic recurrent multifocal osteomyelitis
title Sterile osteomyelitis: a cardinal sign of autoinflammation
title_full Sterile osteomyelitis: a cardinal sign of autoinflammation
title_fullStr Sterile osteomyelitis: a cardinal sign of autoinflammation
title_full_unstemmed Sterile osteomyelitis: a cardinal sign of autoinflammation
title_short Sterile osteomyelitis: a cardinal sign of autoinflammation
title_sort sterile osteomyelitis a cardinal sign of autoinflammation
topic innate immunity
osteomyelitis
chronic recurrent multifocal osteomyelitis
url https://reu.termedia.pl/Sterile-osteomyelitis-a-cardinal-sign-of-autoinflammation,196595,0,2.html
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