Juvenile-onset fibromyalgia (JFM) is a poorly understood chronic pain condition most commonly affecting adolescent girls. The condition is characterized by widespread musculoskeletal pain and other ...associated symptoms, including fatigue, nonrestorative sleep, headaches, irritable bowel symptoms, dysautonomia and mood disorders such as anxiety and/or depression. In the past few years, there has been a greater focus on understanding JFM in adolescents. Research studies have provided insight into the clinical characteristics of this condition and its effect on both short-term and long-term psychosocial and physical functioning. The importance of early and effective intervention is being recognized, as research has shown that symptoms of JFM tend to persist and do not resolve over time as was previously believed. Efforts to improve treatments for JFM are underway, and new evidence strongly points to the potential benefits of cognitive-behavioural therapy on improving mood and daily functioning. Research into pharmacotherapy and other nonpharmacological options is in progress. Advancements in the understanding of adult fibromyalgia have paved the way for future studies on diagnosis, assessment and management of JFM. This Review focuses on our current knowledge of the condition, provides an update of the latest research advances, and highlights areas for further study.
Objectives To evaluate the utility of the 2010 American College of Rheumatology (ACR) adult fibromyalgia criteria for use in adolescents with juvenile fibromyalgia (JFM). Study design Participants ...included 47 adolescent girls diagnosed with JFM (mean age = 15.3 years) and 48 age- and sex-matched adolescents (mean age = 15.0 years) with localized chronic pain (eg, headaches or abdominal pain). A trained examiner administered the Widespread Pain Index and Symptom Severity measures and also completed a manual tender point exam. Clinicians completed a form indicating the presence of active JFM per Yunus and Masi (1985) criteria, the only available and most commonly used measure for JFM. Criterion validity analysis was performed as well as t tests comparing symptoms between JFM and controls. Results With the Yunus and Masi criteria used as the gold standard, the 2010 ACR fibromyalgia criteria showed a sensitivity of 89.4% and specificity of 87.5%. Conclusion The 2010 ACR measure appears to be a valuable tool for the identification of JFM. However, a slight modification to the 2010 ACR measure and inclusion of a clinical exam is recommended.
Cutaneous vasculitis in children is rare. Causes of cutaneous vasculitis are varied and are typically differentiated by the affected vessel size. A skin biopsy remains the gold standard for diagnosis ...but other causes for vasculitis, including systemic conditions, should be considered. This article discusses the childhood conditions commonly presenting with cutaneous vasculitis (leukocytoclastic vasculitis, cutaneous polyarteritis nodosa), biopsy recommendations and findings, and management and potential differential diagnoses, and includes a brief summary of other diseases that may include cutaneous symptoms as a constellation of other systemic findings.
•Uncontrolled, chronic anterior uveitis leads to ocular morbidity in children.•Failed treatment with methotrexate and adalimumab and/or infliximab is not uncommon.•Alternative biologic agents include ...golimumab, tocilizumab, and abatacept.•Nine patients with resistant disease were successfully treated with alternative biologic agents.
A significant number of children with noninfectious, chronic anterior uveitis (CAU) fail to respond to conventional therapy; however, successful alternative biologic treatments (ABT) have not been well described. This study aims to review the clinical and treatment characteristics of children with CAU who require ABT.
Retrospective, nonrandomized clinical study.
Setting: Tertiary center. Study Population: Children with noninfectious CAU. Observation Procedures: Clinical characteristics, uveitis course, complications, and treatment were compared among patients treated with methotrexate (MTX) monotherapy, conventional TNFα inhibitors (cTNFi), and ABT for >3 months. Main Outcome Measure: Success of ABT (abatacept, tocilizumab, and/or golimumab) in children failing conventional treatment.
Of the 52 children with CAU, 75% had juvenile idiopathic arthritis. CAU was controlled in 15 children receiving MTX monotherapy, 28 receiving cTNFi, and 9 receiving ABT (n = 1, abatacept; n = 3, tocilizumab; n = 5, golimumab). Patients in the ABT group had a greater number of total ocular complications per person before ABT than those in the control groups (3.4 vs 0.7 MTX, P < .001, and 1.5 cTNFi, P < .001, respectively). In all 9 children on ABT, treatment led to control of CAU and topical glucocorticoids tapered to ≤2 drops/d with no new ocular complications.
In this study, alternative biologics (abatacept, golimumab, and tocilizumab) were useful for treating CAU in children who fail MTX and cTNFi therapy. Patients who were controlled on ABT had more disease activity, ocular complications, and anti-cTNFi neutralizing antibodies (before ABT) than those managed with conventional therapy. Larger studies are required to confirm these findings.
We report the clinical, radiological, laboratory, and neuropathological findings in support of the first diagnosis of lethal, small-vessel cerebral vasculitis triggered by severe acute respiratory ...syndrome coronavirus 2 (SARS-CoV-2) in a pediatric patient.
A previously healthy, eight-year-old Hispanic girl presented with subacute left-sided weakness two weeks after a mild febrile illness. SARS-CoV-2 nasopharyngeal swab was positive. Magnetic resonance imaging revealed an enhancing right frontal lobe lesion with significant vasogenic edema. Two brain biopsies of the lesion showed perivascular and intraluminal lymphohistiocytic inflammatory infiltrate consistent with vasculitis. Despite extensive treatment with immunomodulatory therapies targeting primary angiitis of the central nervous system, she experienced neurological decline and died 93 days after presentation. SARS-CoV-2 testing revealed positive serum IgG and positive cerebrospinal fluid IgM. Comprehensive infectious, rheumatologic, hematologic/oncologic, and genetic evaluation did not identify an alternative etiology. Postmortem brain autopsy remained consistent with vasculitis.
This is the first pediatric presentation to suggest that SARS-CoV-2 can lead to a fatal, postinfectious, inflammatory small-vessel cerebral vasculitis. Our patient uniquely included supportive cerebrospinal fluid and postmortem tissue analysis. While most children recover from the neurological complications of SARS-CoV-2, we emphasize the potential mortality in a child with no risk factors for severe disease.
Ultrasonography in Pediatric Rheumatology Vega-Fernandez, Patricia; Ting, Tracy V; Pratt, Laura ...
Rheumatic diseases clinics of North America,
02/2022, Volume:
48, Issue:
1
Journal Article
Peer reviewed
This review highlights the increasing evidence from the last few years supporting the use of musculoskeletal ultrasonography (MSUS) in the evaluation and management of patients with pediatric ...rheumatic diseases, particularly focusing on juvenile idiopathic arthritis. Recently developed definitions for the sonographic appearance of healthy and pathologic joints in children are discussed. Further topics explored include how MSUS enhances the diagnosis of inflammatory joint disease (synovitis, enthesitis, tenosynovitis), including the detection of subclinical synovitis. There is a brief summary on the use of ultrasonography in the evaluations of myositis, Sjögren syndrome, and scleroderma.
Objective
Systemic juvenile idiopathic arthritis (JIA) associated with lung disease (JIA‐LD) is a potentially life threating complication in children with systemic JIA. Although high‐resolution ...computed tomography (HRCT) is considered the gold standard imaging modality for evaluating interstitial lung disease (ILD), lung ultrasound (US) has shown utility for ILD screening in adults with connective tissue diseases at lower cost and without using ionizing radiation. The goals of this pilot study were to describe lung US features in children with known systemic JIA‐LD and to assess the feasibility of lung US in this population.
Methods
Children age <18 years with systemic JIA‐LD and healthy controls were enrolled. Lung US acquisition was performed at 14 lung positions. Demographic, clinical, and HRCT data were collected and reviewed. Feasibility was assessed through patient surveys. Lung US findings were qualitatively and semiquantitatively assessed and compared to HRCT findings.
Results
Lung US was performed in 9 children with systemic JIA‐LD and 6 healthy controls and took 12 minutes on average to perform. Lung US findings in systemic JIA‐LD included focal to diffuse pleural irregularity, granularity, and thickening, with associated scattered or coalesced B‐lines, and subpleural consolidations. Lung US findings appeared to correspond to HRCT findings.
Conclusion
Lung US in systemic JIA‐LD reveals highly conspicuous abnormalities in the pleura and subpleura that appear to correlate with peripheral lung findings on HRCT. Lung US is a feasible imaging tool in children even from an early age. This study suggests a potential role of lung US in systemic JIA‐LD screening, diagnosis, and/or prognostication.
Objective
We undertook this study to validate the Pediatric Arthritis Ultrasound Scoring System for the knee joint (PAUSS‐knee) in children with juvenile idiopathic arthritis (JIA).
Methods
Children ...with JIA were enrolled to prospectively receive a musculoskeletal ultrasound (MSUS) examination of the knee and a physical examination to determine presence/absence of clinical arthritis. MSUS images were scored using the PAUSS‐knee, a semiquantitative MSUS scoring system (0–3, normal to severe) for B‐mode and power Doppler mode. In addition to MSUS, a subset of participants also received magnetic resonance imaging (MRI) of the knee, which was scored according to the combined Juvenile Arthritis MRI Scoring (JAMRIS) system. Spearman's correlations (rs) were used to calculate associations between variables. Test characteristics of the PAUSS‐knee were calculated with MRI as the reference standard. Inflammatory biomarkers were assessed in synovial fluid from involved knees.
Results
Eighty children with JIA contributed 112 MSUSs and 25 MRIs of the knee. Of the knees, 41% (n = 46) had clinical evidence of arthritis. The B‐mode PAUSS‐knee score moderately correlated with clinically determined arthritis (rs = 0.54, P < 0.001) and strongly correlated with the JAMRIS score (rs = 0.75, P < 0.001). Compared with MRI, the area under the curve for the B‐mode PAUSS‐knee was 0.92. For a cutoff of >1, the B‐mode PAUSS‐knee had a sensitivity of 83% and specificity of 82%. Biomarker analysis indicates that interleukin‐2R levels correlate with PAUSS score.
Conclusion
Our data indicate that the PAUSS‐knee has excellent accuracy for the diagnosis of arthritis when compared with MRI. The PAUSS‐knee has the potential to effectively inform JIA medical decision‐making in real time.
Abstract
Objective
The clinical decision-making process in paediatric arthritis lacks an objective, reliable bedside imaging tool. The aim of this study was to develop a US scanning protocol and ...assess the reliability of B-mode and Doppler scoring systems for inflammatory lesions of the paediatric ankle.
Methods
As part of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) US group, 19 paediatric rheumatologists through a comprehensive literature review developed a set of standardized views and scoring systems to assess inflammatory lesions of the synovial recesses as well as tendons of the paediatric ankle. Three rounds of scoring of still images were followed by one practical exercise. Agreement among raters was assessed using two-way single score intraclass correlation coefficients (ICC).
Results
Of the 37 initially identified views to assess the presence of ankle synovitis and tenosynovitis, nine views were chosen for each B-mode and Doppler mode semi-quantitative evaluation. Several scoring exercises and iterative modifications resulted in a final highly reliable scoring system: anterior tibiotalar joint ICC: 0.93 (95% CI 0.92, 0.94), talonavicular joint ICC: 0.86 (95% CI 0.81, 0.90), subtalar joint ICC: 0.91 (95% CI 0.88, 0.93) and tendons ICC: 0.96 (95% CI 0.95, 0.97).
Conclusion
A comprehensive and reliable paediatric ankle US scanning protocol and scoring system for the assessment of synovitis and tenosynovitis were successfully developed. Further validation of this scoring system may allow its use as an outcome measure for both clinical and research applications.
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