scholarly journals Imaging of Joints and Bones in Autoinflammation

2020 ◽  
Vol 9 (12) ◽  
pp. 4074
Author(s):  
Katharina Ziegeler ◽  
Iris Eshed ◽  
Torsten Diekhoff ◽  
Kay Geert Hermann
Keyword(s):  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Diagnostic Information ◽  
Innate Immune ◽  
Future Research ◽  
Autoinflammatory Disorders ◽  
Comprehensive Overview ◽  
Crystal Deposition ◽  
Imaging Characteristics ◽  
Multifocal Osteomyelitis ◽  
Mediterranean Fever

Autoinflammatory disorders are commonly characterized by seemingly unprovoked systemic inflammation mainly driven by cells and cytokines of the innate immune system. In many disorders on this spectrum, joint and bone involvement may be observed and imaging of these manifestations can provide essential diagnostic information. This review aimed to provide a comprehensive overview of the imaging characteristics for major diseases and disease groups on the autoinflammatory spectrum, including familial Mediterranean fever (FMF), Behçet disease (BD), crystal deposition diseases (including gout), adult-onset Still’s disease (AoSD), and syndromatic synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO)/chronic recurrent multifocal osteomyelitis (CRMO). Herein, we discuss common and distinguishing imaging characteristics, phenotypical overlaps with related diseases, and promising fields of future research.

scholarly journals The classification, genetic diagnosis and modelling of monogenic autoinflammatory disorders

2018 ◽  
Vol 132 (17) ◽  
pp. 1901-1924 ◽  
Author(s):  
Fiona Moghaddas ◽  
Seth L. Masters
Keyword(s):  
Phenotypic Variability ◽  
Genetic Diagnosis ◽  
Innate Immune ◽  
Future Research ◽  
Autoinflammatory Disorders ◽  
Genetic Sequencing ◽  
Immune Pathway ◽  
Next Generation Sequencing Ngs

Monogenic autoinflammatory disorders are an increasingly heterogeneous group of conditions characterised by innate immune dysregulation. Improved genetic sequencing in recent years has led not only to the discovery of a plethora of conditions considered to be ‘autoinflammatory’, but also the broadening of the clinical and immunological phenotypic spectra seen in these disorders. This review outlines the classification strategies that have been employed for monogenic autoinflammatory disorders to date, including the primary innate immune pathway or the dominant cytokine implicated in disease pathogenesis, and highlights some of the advantages of these models. Furthermore, the use of the term ‘autoinflammatory’ is discussed in relation to disorders that cross the innate and adaptive immune divide. The utilisation of next-generation sequencing (NGS) in this population is examined, as are potential in vivo and in vitro methods of modelling to determine pathogenicity of novel genetic findings. Finally, areas where our understanding can be improved are highlighted, such as phenotypic variability and genotype–phenotype correlations, with the aim of identifying areas of future research.

Chronic recurrent multifocal osteomyelitis in children: a new, MRI-based method of quantifying inflammation in the bone

2015 ◽  
Author(s):  
Dionysios Alexandrou ◽  
Benjamin Jacobs ◽  
O'Donnell Paul ◽  
Rikin Hargunani ◽  
Ananya Guha ◽  
...  
Keyword(s):  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Multifocal Osteomyelitis

Chronic recurrent multifocal osteomyelitis in children

1988 ◽  
Vol 29 (5) ◽  
pp. 565-570 ◽  
Author(s):  
W. Mortensson ◽  
G. Edeburn ◽  
M. Fries ◽  
R. Nilsson
Keyword(s):  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Multifocal Osteomyelitis

scholarly journals Patient-reported effectiveness and safety of Pamidronate in NSAIDs-refractory chronic recurrent multifocal osteomyelitis in children

2021 ◽  
Author(s):  
Bartłomiej Juszczak ◽  
Jerzy Sułko
Keyword(s):  
Adverse Drug Reactions ◽  
Social Life ◽  
Chronic Recurrent Multifocal Osteomyelitis ◽  
Daily Activities ◽  
Drug Reactions ◽  
Patient Reported ◽  
Multifocal Osteomyelitis ◽  
Drug Dependent ◽  
Treatment Onset ◽  
The Impact

AbstractTo evaluate patient-reported effectiveness, safety and social influence of Pamidronate in the therapy of NSAIDs-refractory Chronic Recurrent Multifocal Osteomyelitis in children. Authors reviewed self-created questionnaires, which asked patients for symptoms alleviation, adverse drug reactions frequency and degree of severity and daily activities self-reliance. Only surveys with complete answers, which were returned to authors by an e-mail from juvenile patients treated for NSAIDs-refractory Chronic Recurrent Multifocal Osteomyelitis at the University Children’s Hospital of Cracow were analyzed. Between 2010 and 2019, 61 children were diagnosed with NSAIDs-refractory Chronic Recurrent Multifocal Osteomyelitis at our department. Out of 61 requests sent, 42 complete replies (33 females, 9 males) were gathered and analyzed. All patients included in this research were administered with at least one set of Pamidronate intravenously in the dose of 1 mg/kg/day for 3 consecutive days. Our analysis shows remarkable in terms of patient’s impressions decrease of pain intensity after 2.5 series of Pamidronate on average, and total pain resolution after 5.9 series on average. Overall number of adverse drug reaction events reported by responders was 105. One patient developed drug-dependent renal insufficiency in the course of therapy. Outcome assessment indicates that nearly 50% of the studied population was more eager to participate in social life just after the first infusion of the drug. 95% of the surveyed unanimously agreed to recommend Pamidronate therapy to cure NSAIDs-refractory CRMO. 39 out of 42 (93%) patients considered Pamidronate effective at the end of the treatment. Onset of Pamidronate’s action is gradual and differs in terms of symptoms alleviation between sexes. The therapy can induce considerable number of adverse drug reactions (2.5 per patient). Only 3 out of 42 (7%) patients were free from any ADRs. To demonstrate the impact of the use of Pamidronate on daily activities more precisely, further research with quantification of the quality of life is warranted.

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