Following publication of the original article 1, the authors reported that the names of two institutional authors - EUROFEVER and the Paediatric Rheumatology International Trials Organisation ...(PRINTO) - had been unintentionally omitted in the final online version of the manuscript. The corrected author list is shown in this Correction..
Despite the well-known role of obesity as risk factor for Juvenile Idiopathic Arthritis (JIA) severity, emerging but limited evidence suggested a similar role for underweight. We investigated the ...role of body mass index (BMI) across its full spectrum in a cohort of children with JIA.
We retrospectively studied 113 children with JIA classified according to the International League of Association for Rheumatology (ILAR) criteria attending our Rheumatology Clinic. The patients underwent a comprehensive evaluation including both clinical and biochemical assessments. According to BMI Z-score, the cohort was divided into five groups as underweight, normal weight, overweight (OW), obesity (OB), and severe OB. Disease activity was calculated by Juvenile Arthritis Disease Activity Score 10 (JADAS-10) joint reduced count and relapses were defined according to Wallace criteria.
The mean age of the cohort was 7.43 ± 4.03 years. The prevalence of underweight, normal weight, OW, OB, and severe OB was 7.2%, 54.1%, 10.8%, 17.1%, and 10.8%, respectively. Significant higher ferritin levels and erythrocyte sedimentation rate values were found in patients with severe OB and underweight compared to subjects belonging to normal weight, OW, and OB groups. A greater JADAS-10 score was observed in underweight patients and in those with severe OB than other groups. The relapse rate was higher in patients with severe OB and underweight compared to other groups.
Conclusions
: Both underweight and OB might negatively affect JIA course. Weight control is fundamental in children with JIA to avoid a more unfavourable course of the disease.
What is Known:
• Obesity represents a well-known risk factor for JIA severity.
• The role of underweight in children with JIA is still poorly explored.
What is New:
• As observed in children with obesity, underweight young patients with JIA seem to experience a more severe JIA course.
• Healthy lifestyle promotion in children with JIA is a crucial step in the management of the disease.
To describe the clinical characteristics, treatment response and genetic findings in a large cohort of patients with undefined systemic autoinflammatory diseases (SAIDs).
Clinical and genetic data ...from patients with undefined SAIDs were extracted from the Eurofever registry, an international web-based registry that retrospectively collects clinical information on patients with autoinflammatory diseases.
This study included 187 patients. Seven patients had a chronic disease course, 180 patients had a recurrent disease course. The median age at disease onset was 4.3 years. Patients had a median of 12 episodes per year, with a median duration of 4 days. Most commonly reported symptoms were arthralgia (n=113), myalgia (n=86), abdominal pain (n=89), fatigue (n=111), malaise (n=104) and mucocutaneous manifestations (n=128). In 24 patients, relatives were affected as well. In 15 patients, genetic variants were found in autoinflammatory genes. Patients with genetic variants more often had affected relatives compared with patients without genetic variants (p=0.005). Most patients responded well to non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, colchicine and anakinra. Complete remission was rarely achieved with NSAIDs alone. Notable patterns were found in patients with distinctive symptoms. Patients with pericarditis (n=11) were older at disease onset (33.8 years) and had fewer episodes per year (3.0/year) compared with other patients. Patients with an intellectual impairment (n=8) were younger at disease onset (2.2 years) and often had relatives affected (28.6%).
This study describes the clinical characteristics of a large cohort of patients with undefined SAIDs. Among these, patients with pericarditis and intellectual impairment appear to comprise distinct subsets.
In addition to disease-specific complications, juvenile idiopathic arthritis (JIA) has been linked to metabolic impairments in adults. Recent data supported the usefulness of uric acid (UA) as risk ...factor for cardiometabolic derangements. Given the lack of pediatric evidence in this field, we aimed to explore this association in a cohort of children diagnosed with JIA. We retrospectively evaluated 113 children diagnosed with JIA classified according to the International League of Association for Rheumatology (ILAR) criteria attending our Rheumatology Clinic. Both clinical and biochemical assessments were performed. Participants were stratified in four groups according to quartiles of serum UA. Disease activity was calculated by the Juvenile Arthritis Disease Activity Score 10 (JADAS-10) joint reduced count, and cut-offs for disease states were applied. Patients belonging to the highest UA quartile showed higher serum triglycerides, total cholesterol, creatinine, and glucose levels (
p
= 0.01,
p
= 0.025,
p
= 0.04, and
p
= 0.005, respectively) and lower HDL cholesterol values (
p
< 0.0001) than subjects belonging to the lowest quartiles. Ferritin, erythrocyte sedimentation rate levels, and age at disease onset did not significantly differ across UA quartiles (all
p
> 0.05). As activity disease index, JADAS-10 score significantly increased across UA quartiles (
p
= 0.009).
Conclusion
: Children with JIA presented with a worse cardiometabolic profile and a greater disease severity across UA quartiles. Our findings suggest that in clinical practice, UA might represent a useful marker of cardiometabolic risk and disease severity in children with JIA.
What is Known:
•
JIA has been linked to metabolic derangements in adulthood.
•
UA has been recognized as a marker of cardiometabolic risk both in adults and children.
What is New:
•
Children with JIA belonging to the highest UA quartile showed a worse cardiometabolic profile and a greater disease severity.
•
UA might represent a helpful marker not only of cardiometabolic risk but also of disease severity in children with JIA.
The aim of the study was to evaluate the cephalometric differences and condylar asymmetry between patients with juvenile idiopathic arthritis (JIA) and normal control group. Sixty-two JIA patients ...with a latero-lateral cephalogram and orthopantomography, seeking for orthodontic therapy, and 62 normal matched subjects were comprised in the study. Cephalometric analysis was used for the evaluation of facial morphology while the method of Habets et al. (J Oral Rehabil 15(5): 465–471,
1988
) was used to compare the condyles in orthopantomography. The significance of between-group differences was assessed using the Mann–Whitney test, as appropriate. The results showed a prevalence of the upper maxilla with hypomandibulia (class II), hyperdivergency with short vertical ramus posterior and posterior rotation of the mandible in JIA children (SNB, ANB, NSL/ML, Fh/ML, NL/ML, ArGo, ML
P
< 0.0001, ML/Oc
P
< 0.004, ArGo/GoGn
P
= 0.02, no difference for SNA). The condyles of the JIA group resulted highly asymmetric (
P
< 0.0001). The growth pattern of JIA patients resulted clearly different from normal subjects. This serious impairment of the cranial growth may be considered as an indicator of the need for early and continuous orthognatodonthic therapy during the entire period of development for all JIA patients, independently from temporomandibular joint signs or symptoms. To this end, it is important that rheumatologists and orthognathodontists set up a multidisciplinary treatment planned to control the side effects of a deranged growing pattern, to strictly avoid any orthodontic therapies that may worsen function and growth, and to promote treatments improving the physiology and biology of the cranial development.
To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis ...(c-TA).
Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis <or=18 years. Step 2: blinded classification by consensus panel of a representative sample of 280 cases. Step 3: statistical (sensitivity, specificity, area under the curve and kappa-agreement) and nominal group technique consensus evaluations.
827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classified as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classification of c-PAN required a systemic inflammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of five criteria: (1) skin involvement; (2) myalgia/muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classification of c-WG required three of six criteria: (1) histopathological evidence of granulomatous inflammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classification of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of five criteria: (1) pulse deficit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) elevated acute phase reactant.
European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society propose validated classification criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specificity.
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