Objective
The use of musculoskeletal ultrasound is increasing among pediatric rheumatologists. Reliable scoring systems are needed for the objective assessment of synovitis. The aims of this study ...were to create a standardized and reproducible image acquisition protocol for B‐mode and Doppler ultrasound of the pediatric knee, and to develop a standardized scoring system and determine its reliability for pediatric knee synovitis.
Methods
Six pediatric rheumatologists developed a set of standard views for knee assessment in children with juvenile arthritis. Subsequently, a comprehensive literature review, practical exercises, and a consensus process were performed. A scoring system for both B‐mode and Doppler was then developed and assessed for reliability. Interreader reliability or agreement among a total of 16 raters was determined using 2‐way single‐score intraclass correlation coefficient (ICC) analysis.
Results
Twenty‐one views to assess knee arthritis were initially identified. Following completion of practical exercises and subsequent consensus processes, 3 views in both B‐mode and Doppler were selected: suprapatellar longitudinal and medial/lateral parapatellar transverse views. Several rounds of scoring and modifications resulted in a final ICC of suprapatellar view B‐mode 0.89 (95% confidence interval 95% CI 0.86–0.92) and Doppler 0.55 (95% CI 0.41–0.69), medial parapatellar view B‐mode 0.76 (95% CI 0.68–0.83) and Doppler 0.75 (95% CI 0.66–0.83), and lateral parapatellar view B‐mode 0.82 (95% CI 0.75–0.88) and Doppler 0.76 (95% CI 0.66–0.84).
Conclusion
A novel B‐mode and Doppler image acquisition and scoring system for assessing synovitis in the pediatric knee was successfully developed through practical exercises and a consensus process. Study results demonstrate overall good‐to‐excellent reliability.
This prospective longitudinal study examined the long-term physical and psychosocial outcomes of adolescents with juvenile-onset fibromyalgia (JFM), compared with healthy control subjects, into early ...adulthood.
Adolescent patients with JFM initially seen at a pediatric rheumatology clinic (n = 94) and age- and gender-matched healthy control subjects (n = 33) completed online measures of demographic characteristics, pain, physical functioning, mood symptoms, and health care utilization at ∼6 years' follow-up (mean age: 21 years). A standard in-person tender-point examination was conducted.
Patients with JFM had significantly higher pain (P < .001), poorer physical function (P < .001), greater anxiety (P < .001) and depressive symptoms (P < .001), and more medical visits (P < .001)than control subjects. The majority (>80%) of JFM patients continued to experience fibromyalgia symptoms into early adulthood, and 51.1% of the JFM sample met American College of Rheumatology criteria for adult fibromyalgia at follow-up. Patients with JFM were more likely than control subjects to be married and less likely to obtain a college education.
Adolescent patients with JFM have a high likelihood of continued fibromyalgia symptoms into young adulthood. Those who met criteria for fibromyalgia in adulthood exhibited the highest levels of physical and emotional impairment. Emerging differences in educational attainment and marital status were also found in the JFM group. JFM is likely to be a long-term condition for many patients, and this study for the first time describes the wide-ranging impact of JFM on a variety of physical and psychosocial outcomes that seem to diverge from their same-age peers.
OBJECTIVES:Current therapies for juvenile fibromyalgia (JFM), such as cognitive-behavioral therapy (CBT), improve pain coping but are less effective for pain reduction or engagement in physical ...activity. The Fibromyalgia Integrative Training for Teens (FIT Teens) program combines CBT with specialized neuromuscular exercise training for adolescents with JFM. The current investigation examined the effects of FIT Teens versus CBT on secondary outcomes of strength and functional biomechanics, utilizing 3D Motion capture technology. This study aimed to explore improvements in strength and biomechanics in both a CBT-only group and the FIT Teens intervention.
MATERIALS AND METHODS:Forty adolescents with JFM (12 to 18 y) were randomized to an 8-week, group-based protocol of either FIT Teens or CBT only. Assessments occurred pretreatment and posttreatment. Hip and knee strength were assessed with dynamometry, dynamic postural stability was measured using the Star Excursion Balance Test, and movement biomechanics were assessed with 3D motion analyses during a drop vertical jump (DVJ) task.
RESULTS:The FIT Teens group exhibited improvements in hip abduction strength and greater external hip rotation during the DVJ task. Some differences between the FIT Teens and CBT groups were observed in peak hip internal moment in the transverse plane. Decreased hip adduction during the DVJ was also observed in the FIT Teens group.
DISCUSSION:Results suggest that the FIT Teens program shows promise in improving hip abduction strength and body biomechanics, indicating improvements in stability during functional movements. These improvements may facilitate ability to initiate and maintain regular physical activity in youth with widespread musculoskeletal pain.
Objective
Juvenile fibromyalgia (FM) is a prevalent chronic pain condition affecting children and adolescents worldwide during a critical period of brain development. To date, no published studies ...have addressed the pathophysiology of juvenile FM. This study was undertaken to characterize gray matter volume (GMV) alterations in juvenile FM patients for the first time, and to investigate their functional and clinical relevance.
Methods
Thirty‐four female adolescents with juvenile FM and 38 healthy adolescents underwent a structural magnetic resonance imaging examination and completed questionnaires assessing core juvenile FM symptoms. Using voxel‐based morphometry, we assessed between‐group GMV differences and associations between GMV and functional disability, fatigue, and pain interference in juvenile FM. We also studied whether validated brain patterns predicting pain, cognitive control, or negative emotion were amplified/attenuated in juvenile FM patients and whether structural alterations reported in adult FM were replicated in adolescents with juvenile FM.
Results
Compared to controls, juvenile FM patients showed GMV reductions in the anterior midcingulate cortex (aMCC) region (family‐wise error corrected P PFWE‐corr = 0.04; estimated with threshold‐free cluster enhancement TFCE; n = 72) associated with pain. Within the juvenile FM group, patients reporting higher functional disability had larger GMV in inferior frontal regions (PFWE‐corr = 0.006; TFCE estimated; n = 34) linked to affective, self‐referential, and language‐related processes. Last, GMV reductions in juvenile FM showed partial overlap with findings in adult FM, specifically for the anterior/posterior cingulate cortices (P = 0.02 and P = 0.03, respectively; n = 72).
Conclusion
Pain‐related aMCC reductions may be a structural hallmark of juvenile FM, whereas alterations in regions involved in emotional, self‐referential, and language‐related processes may predict disease impact on patients’ well‐being. The partial overlap between juvenile and adult FM findings strengthens the importance of early symptom identification and intervention to prevent the transition to adult forms of the disease.
Hypermobile Ehlers-Danlos syndrome (hEDS) is characterized by joint and skin laxity, and often accompanied by chronic pain, dysautonomia, increased distress and, functional limitations. The journey ...to accurate diagnosis is often prolonged due to unclear etiology of symptoms. This manuscript is a narrative review of the literature on illness uncertainty (IU) in hEDS, highlighting the unique facets of IU in this population, as compared to the broader chronic pain population (given symptom overlap between these two disease groups), that warrant additional investigation. Additionally, we considered the unique challenges associated with IU in the context of the developmental nuances of pediatric populations. Specifically, we aimed to (1) map the extant literature of the IU experience in chronic pain conditions broadly including the pediatric and adult research to identify key concepts related to IU and incorporate potential developmental considerations in IU; (2) delineate and describe the IU experience specifically in patients with hEDS, with the goal of identifying gaps in the literature based on aspects of presentation in hEDS that do and do not differ from the broader chronic pain population; and (3) elucidate the potential areas of adverse impact of IU in both general chronic pain populations, and those with hEDS specifically, to provide actionable areas for future research and clinical care of individuals with hEDS. Results of this review indicate that IU has been well-studied in chronic pain generally, but inadequately evaluated in hEDS specifically. Specific features of hEDS (complexity of the disorder, involvement of multiple bodily systems, contribution of organic pathology) may uniquely contribute to IU in this population. This review suggests that ambiguities surrounding the diagnosis of hEDS, symptom course, and treatment recommendations, along with misdiagnosis, perceived dismissal of symptoms, or attribution of symptoms to mental health concerns might increase risk for IU and related distress in patients.
Findings from the present review suggest that distinct features of hEDS yield a set of driving factors for IU that may be somewhat different than those faced by patients with chronic pain or other medical conditions. The development of a validated measure of IU to appropriately assess this construct in patients with hEDS is a research priority. In the clinical setting, providers should be attentive to the potentially aversive diagnostic and treatment experiences reported by patients and attempt to provide clear explanations based on the extant knowledge of hEDS, and implement best-practice recommendations for multidisciplinary treatment.
Ehlers‐Danlos syndrome (EDS) is a heterogeneous group of inherited disorders of connective tissue. EDS hypermobility type (EDS‐HT), characterized by joint hypermobility, is most common and ...increasingly recognized in pediatrics. Treatment involves protecting joints, preventing injuries, and managing symptoms/comorbidities. Pediatric EDS‐HT patients often see multiple medical providers; however, data on healthcare utilization (HCU) in this population are lacking. This retrospective, electronic chart review examines HCU data 1 year prior and subsequent to a new diagnosis of EDS‐HT using Villefranche criteria. Demographics, diagnoses, and HCU (office visits, therapies, hospital encounters/procedures, and tests) were obtained for N = 102 youth attending a Connective Tissue Disorder Clinic over a 21‐month timeframe. After EDS‐HT diagnosis, HCU patterns shifted to reflect greater involvement of therapy (physical, psychological, and occupational) and symptom management. More genetics, rheumatology, and orthopedics visits occurred prediagnosis, and more physical therapy, pain management, cardiology, and neurology visits occurred postdiagnosis. Testing and hospital encounter/procedure frequencies did not change. Overall, the pattern of HCU changed from diagnostic to treatment, in accordance with evidence‐based EDS‐HT care. Understanding HCU patterns of pediatric patients with EDS‐HT can elucidate patient interaction with the health care system, with the potential to inform and improve the standard of care.
In a cohort of 70 patients with childhood-onset systemic lupus erythematosus (cSLE): to determine the baseline adherence to medications and visits; to investigate the effects of cellular text ...messaging reminders (CTMR) on adherence to clinic visits; and to study the influence of CTMR on adherence to use of hydroxychloroquine (HCQ).
CTMR were sent to 70 patients prior to clinic visits for 14 months. A subgroup of patients were evaluated for medication adherence to HCQ: 19 patients receiving CTMR prior to each scheduled HCQ dose were compared to 22 patients randomized to standard of care education about HCQ. Visit adherence was measured using administrative databases. Pharmacy refill information, self-report of adherence, and HCQ blood levels were utilized to monitor medication adherence to HCQ. Sufficient adherence to visits or HCQ was defined as estimates > 80%. Disease activity was primarily monitored with the Systemic Lupus Erythematosus Disease Activity Index.
At baseline, 32% of patients were sufficiently adherent to HCQ, and 81% to clinic visits. Visit adherence improved significantly by > 80% among those who were nonadherent to clinic visits at the baseline CTMR (p = 0.01). CTMR did not influence adherence to HCQ over time.
Patients with cSLE were only modestly adherent to HCQ and clinic visits. CTMR may be effective for improving visit adherence among adolescents and young adults with cSLE, but it does not improve adherence to HCQ.
ObjectiveTreat-to-target (T2T) strategies are advocated to improve prognosis in childhood-onset SLE (cSLE). Proposed T2T states include SLEDAI score of <4 (SLEDAI-LD), limited corticosteroid use ...(low-CS), and lupus low disease activity state (LLDAS). We sought to compare T2T states for their association with cSLE prognosis under consideration of relevant disease characteristics such as pre-existing damage, race and lupus nephritis (LN).MethodsLongitudinal data from 165 patients enrolled in the Cincinnati Lupus Registry were included. LN presence was based on renal biopsy, and patients were followed up until 18 years of age.ResultsThe 165 patients (LN: 45, white: 95) entered the registry within a median of 0 (IQR: 0–1) year post diagnosis and were followed up for a median of 4 (IQR: 2–5) years during which 80%, 92% and 94% achieved LLDAS, low-CS and SLEDAI-LD. Patients with LN were significantly less likely to achieve any T2T state (all p<0.03) and required a significantly longer time to reach them (all p<0.0001). Over the study period, patients maintained low-CS, SLEDAI-LD or LLDAS for a median of 76% (IQR: 48%–100%), 86% (IQR: 55%–100%) or 39% (IQR: 13%–64%) of their follow-up. Significant predictors of failure to maintain LLDAS included LN (p≤0.0062), pre-existing damage (p≤0.0271) and non-white race (p≤0.0013). There were 22%, 20% and 13% of patients who reached SLEDAI-LD, CS-low and LLDAS and nonetheless acquired new damage. Patients with LN had a higher risk of new damage than patients without LN even if achieving low-CS (p=0.009) or LLDAS (p=0.04).ConclusionsPatients with LN and pre-existing damage are at higher risk of increased future damage acquisition, even if achieving a T2T state such as LLDAS. Among proposed common T2T states, the LLDAS is the hardest to achieve and maintain. The LLDAS may be considered the preferred T2T measure as it conveys the highest protection from acquiring additional disease damage.
Non-adherence is a prevalent and modifiable issue in juvenile idiopathic arthritis (JIA) that currently lacks provider-based intervention. Education surrounding disease status is one way in which ...families remain engaged in their care. Musculoskeletal ultrasound is one such form of demonstrative, real-time education that may impact the way patients and caregivers self-manage their disease. The aims of this study are to 1) assess the feasibility, acceptability and perceived usefulness of musculoskeletal ultrasound as a non-adherence intervention tool and 2) to examine changes in methotrexate adherence in adolescents with JIA following the ultrasound.
Eight adolescents with polyarticular or extended oligoarticular JIA and their caregivers completed this 12 week study. A within subject design was used to compare baseline and post-intervention adherence, quality of life and disease activity indices. Adherence measures included electronic measurement of methotrexate in addition to self-reported adherence questionnaires. The ultrasound intervention included a one-time, rheumatologist provided, educational examination of three or more currently or historically active joints.
The ultrasound intervention was found to be both feasible and acceptable. One hundred percent of eligible participants completed the ultrasound intervention. The ultrasound was well received by patients and caregivers, with most believing this to be a helpful tool. Baseline adherence was 75.3% among participants, with half of the participants being classified as non-adherent. Electronically measured and self-reported adherence measures did not show significant changes during the post-intervention period. Two participants improved, four participants maintained, and two participants decreased adherence. On ultrasound, 18/27 (66.7%) of the examined joints displayed abnormalities, with 63% being discrepant and additive to the rheumatologist's physical examination.
While our intervention did not show any changes in adherence, quality of life or disease activity indices in this proof-of-concept trial, the intervention does show promise in acceptability measures and merits future study in a more robust trial design. An additional study benefit was that the musculoskeletal ultrasound intervention was able to demonstrate subclinical disease, leading to clinically impactful therapeutic changes in several participants.
Objective
Juvenile fibromyalgia (JFM) is a complex chronic pain condition that remains poorly understood. The study aimed to expand the clinical characterization of JFM in a large representative ...sample of adolescents with JFM and identify psychological factors that predict pain interference.
Methods
Participants were 203 adolescents (ages 12–17 years) who completed baseline assessments for the multisite Fibromyalgia Integrative Training for Teens (FIT Teens) randomized control trial. Participants completed the Pain and Symptom Assessment Tool, which includes a Widespread Pain Index (WPI; 0–18 pain locations) and Symptom Severity checklist of associated somatic symptoms (SS; 0–12) based on the 2010 American College of Rheumatology criteria for fibromyalgia. Participants also completed self‐report measures of pain intensity, functional impairment, and psychological functioning.
Results
Participants endorsed a median of 11 painful body sites (WPI score) and had a median SS score of 9. Fatigue and nonrestorative sleep were prominent features and rated as moderate to severe by 85% of participants. Additionally, neurologic, autonomic, gastroenterologic, and psychological symptoms were frequently endorsed. The WPI score was significantly correlated with pain intensity and catastrophizing, while SS scores were associated with pain intensity and all domains of physical and psychological functioning. Depressive symptoms, fatigue, and pain catastrophizing predicted severity of pain impairment.
Conclusion
JFM is characterized by chronic widespread pain with fatigue, nonrestorative sleep, and other somatic symptoms. However, how diffusely pain is distributed appears less important to clinical outcomes and impairment than other somatic and psychological factors, highlighting the need for a broader approach to the assessment and treatment of JFM.
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