Juvenile Sjögren’s Syndrome

Thatayatikom, Akaluck; Thatayatikom, Sthorn; Cha, Seunghee

doi:10.1007/978-3-030-90977-2_7

Cited by 4 publications

(5 citation statements)

References 75 publications

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“…The retrospective approach in this study did not allow implementation of a uniform treatment protocol for all patients and a comprehensive evaluation of the treatment response. Importantly, our findings are in line with other studies indicating that CRMO is a heterogeneous disease 34,35 and that “CMRO” might be considered as an umbrella term for phenotypically similar disorders with potentially diverse etiopathogenetic backgrounds and sterile osteomyelitis as a dominant feature.…”

Section: Discussionsupporting

confidence: 92%

Two phenotypes of Chronic Recurrent Multifocal Osteomyelitis with different patterns of bone involvement

Cebecauerova

Malcová

Koukolská

et al. 2022

Preprint

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IntroductionChronic Recurrent Multifocal Osteomyelitis (CRMO) is an autoinflammatory bone disorder with predominantly paediatric onset. Children present with multifocal osteolytic lesions accompanied by bone pain and soft tissue swelling. Patients often exhibit extraosseous co-morbidities such as psoriasis, inflammatory bowel disease, and arthritis.ObjectivesComparison of children with two different phenotypes of CRMO defined by presence or absence of extraosseous co-morbidities.MethodsChildren diagnosed with CRMO at the Motol University Hospital between 2010-2020 were retrospectively reviewed, and according to the absence or presence of extraosseous manifestations divided into two cohorts – osteolytic CRMO and complex CRMO. The two groups were compared in terms of demographic data, age at disease onset, number and site of bone lesions, laboratory biomarker values, and need of escalation to a second-line therapyResultsThirty-seven children (30 female, 7 male) with confirmed CRMO were included in the analysis. The mean age at disease onset was ten years. All but 3 patients presented with multifocal disease. Twenty-three children (62%) had at least one extraosseous manifestation (13 sacroiliitis, 8 inflammatory bowel disease, 6 skin disease [acne, pustulosis, or psoriasis], 7 arthritis). Complex CRMO was associated with a significantly higher ESR rate (p=0.0064) and CRP level (p=0.018). The groups did not differ in number of foci or in age at disease onset. Bone lesion distribution differed between the two groups with significantly more frequent involvement of clavicle (p=0.011) and pelvis (p=0.038) in patients with complex CRMO. Children with complex CRMO more often needed escalation of therapy to DMARDs and biologic agents.ConclusionOur data suggest that CRMO affecting solely the skeleton has milder course compared to complex CRMO with extraskeletal features. Further studies are needed to explore the clinical as well as the patient reported outcomes and promote individually tailored therapeutic strategies in both CRMO phenotypes.

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Section: Discussionsupporting

confidence: 92%

Two phenotypes of Chronic Recurrent Multifocal Osteomyelitis with different patterns of bone involvement

Cebecauerova

Malcová

Koukolská

et al. 2022

Preprint

View full text Add to dashboard Cite

show abstract

“…The retrospective approach in this study did not allow implementation of a uniform treatment protocol for all patients and a comprehensive evaluation of the treatment response between the two groups of CRMO. Importantly, our findings are in line with other studies indicating that CRMO is a heterogeneous disease [ 36 , 37 ] and might be considered as an umbrella term for phenotypically similar disorders with potentially diverse etiopathogenetic backgrounds and sterile osteomyelitis as a dominant feature.…”

Section: Discussionsupporting

confidence: 91%

Two phenotypes of chronic recurrent multifocal osteomyelitis with different patterns of bone involvement

et al. 2022

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Introduction Chronic Recurrent Multifocal Osteomyelitis (CRMO) is an autoinflammatory bone disorder with predominantly paediatric onset. Children present with multifocal osteolytic lesions accompanied by bone pain and soft tissue swelling. Patients often exhibit extraosseous co-morbidities such as psoriasis, inflammatory bowel disease, and arthritis. Objectives Comparison of children with two different phenotypes of CRMO defined by presence or absence of extraosseous co-morbidities. Methods Children diagnosed with CRMO at the Motol University Hospital between 2010 and 2020 were retrospectively reviewed, and according to the absence or presence of extraosseous manifestations divided into two cohorts – bone limited CRMO and complex CRMO. The two groups were compared in terms of demographic data, age at disease onset, number and site of bone lesions, laboratory biomarker values, and need of escalation to a second-line therapy. Results Thirty-seven children (30 female, 7 male) with confirmed CRMO were included in the analysis. The mean age at disease onset was 10 years. All but 3 patients presented with multifocal disease. Twenty-three children (62%) had at least one extraosseous manifestation (13 sacroiliitis, 8 inflammatory bowel disease, 6 skin disease [acne, pustulosis, or psoriasis], 7 arthritis). Complex CRMO was associated with a significantly higher ESR rate (p = 0.0064) and CRP level (p = 0.018). The groups did not differ in number of foci or in age at disease onset. Bone lesion distribution differed between the two groups with significantly more frequent involvement of clavicle (p = 0.011) and pelvis (p = 0.038) in patients with complex CRMO. Children with complex CRMO more often needed escalation of therapy to DMARDs and biologic agents. Conclusion Our data suggest that CRMO affecting solely the skeleton has milder course compared to complex CRMO with extraskeletal features. Further studies are needed to explore the clinical as well as the patient reported outcomes and promote individually tailored therapeutic strategies in both CRMO phenotypes.

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“…Juvenile idiopathic arthritis (JIA) represents the most common rheumatic disease in the pediatric population, defined as persistent joint inflammation with a duration of at least 6 weeks, onset before the age of sixteen and of unknown etiology [ 1 ]. Certain groups of pediatric patients, including JIA patients, are considered to have a greater risk of developing CVD and their complications than the general population due to the chronic nature of the disease [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Assessment of Cardiovascular Risk Factors in Patients with Juvenile Idiopathic Arthritis

et al. 2023

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Introduction: The aim of this study was to assess the exposure to cardiovascular disease (CVD) risk factors in patients with juvenile idiopathic arthritis (JIA). Intima–media complex thickness (IMT), selected metabolic parameters and health behaviors were assessed in the course of the study. Methods: The study included study group, which consisted of 45 patients with JIA and 37 healthy age- and sex-matched children in the control group. Analyses in both groups included anthropometric parameters, laboratory tests, IMT and a questionnaire on exposure to modifiable CVD risk factors. Results: The study confirmed that CVD risk factors were present in both groups of patients. Significantly more children with JIA had abnormal BMI (p = 0.006) compared to the control group. Children in the study group were more likely to consume fruit regularly (p = 0.021) and less likely to consume fast food (p = 0.011) and sweetened beverages (p = 0.042) than children in the control group. Only 1 patient with JIA met criteria for ideal cardiovascular health. Dietary habits were not associated with IMT values, BMI, presence of joint pain or biochemical parameters in the study group. Conclusions: Patients with JIA are exposed to cardiovascular risk factors equally to their healthy peers. Ideal cardiovascular health should be pursued in the pediatric population with particular attention paid to patients with chronic diseases (i.e., JIA). The application of carotid artery IMT measurement in the assessment of CVD risk requires studies on a larger group of patients.

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Juvenile Sjögren’s Syndrome

Cited by 4 publications

References 75 publications

Two phenotypes of Chronic Recurrent Multifocal Osteomyelitis with different patterns of bone involvement

Two phenotypes of Chronic Recurrent Multifocal Osteomyelitis with different patterns of bone involvement

Two phenotypes of chronic recurrent multifocal osteomyelitis with different patterns of bone involvement

Assessment of Cardiovascular Risk Factors in Patients with Juvenile Idiopathic Arthritis

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