Non-Pharmacologic Intensive Interdisciplinary Pain Treatment in Pediatrics: Impact on Symptoms, Daily Functioning, and the Family Unit.

Objectives: To assess non-pharmacologic treatment outcomes pertaining to health-related quality of life (HRQoL) in youth with chronic idiopathic pain and their families. Methods: We conducted a retrospective cohort study of 115 youth with chronic idiopathic pain enrolled in a non-pharmacologic, hospital-based intensive interdisciplinary pain treatment (IIPT) program. HRQoL measures for the patient (Pediatric Quality of Life Inventory [PedsQL] short form) and family unit (PedsQL Family Impact) were collected on admission and discharge as part of routine clinical care. Changes in PedsQL scores were calculated using the Wilcoxon signed-rank test. Multivariable linear regression was used to explore factors associated with patient-level HRQoL. Results: Both individuals and the family unit reported that their HRQoL improved in all domains by program completion. Improvements in pain and allodynia were present for program participants at the time of completion as well as at the 3-month follow-up, suggesting durability of these effects. Conclusions: A non-pharmacologic IIPT program is a compelling treatment for pediatric and adolescent chronic idiopathic pain, for both patients and the family unit. Patients participating in this program had positive treatment outcomes with significantly improved subjective and objective measures of physical, emotional, social, and cognitive function.

The impact of the COVID-19 pandemic on pediatric rheumatology practice: an international, cross-sectional survey study.

OBJECTIVE: The COVID-19 pandemic has affected patient care in general. We aimed to analyze the impact of the pandemic on pediatric rheumatology practice. METHODS: An online survey including 22 questions was created by the representatives of the Emerging RheumatoloGists and rEsearchers (EMERGE) group of the Pediatric Rheumatology European Society (PReS) on SurveyMonkey. The descriptive analysis of the responses was performed on SurveyMonkey. RESULTS: Overall, 469 pediatric rheumatologists (F/M: 2.9) from 70 countries completed the survey. The practice of drug prescription is not affected by the pandemic, according to 65.3 % of the respondents, while 24.3 % and 16.5 % are prescribing biologic drugs and corticosteroids less often, respectively. Over 40 % of the respondents have seen an increased number of patients with vasculitis or chilblains during the pandemic. One-third of the respondents stated no adjustments in their clinical practice after 2.5 years of COVID-19 pandemic. The rest indicated implementing various changes, with an emphasis on incorporating telemedicine. Telemedicine constitutes ≥10 % of the clinical practice for one-third of the participants. Nonetheless, 35.5 % agree that there are still delays in patient care due to the pandemic. However, most (∼90 %) think our practice is returning to the pre-pandemic routine. CONCLUSION: The findings of our study indicate a significant alteration in pediatric rheumatology practice due to the pandemic. This includes increased caution when prescribing anti-rheumatic drugs, a transition towards telemedicine utilization, delays in routine care, and a rise in COVID-19-related inflammatory conditions. It is imperative to address these aspects in order to improve patient care in pediatric rheumatology.

"I'd like more options!": Interviews to explore young people and family decision-making needs for pain management in juvenile idiopathic arthritis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common pediatric rheumatic condition and is associated with symptoms such as joint pain that can negatively impact health-related quality of life. To effectively manage pain in JIA, young people, their families, and health care providers (HCPs) should be supported to discuss pain management options and make a shared decision. However, pain is often under-recognized, and pain management discussions are not optimal. No studies have explored decision-making needs for pain management in JIA using a shared decision making (SDM) model. We sought to explore families' decision-making needs with respect to pain management among young people with JIA, parents/caregivers, and HCPs. METHODS: We conducted semi-structured virtual or face-to-face individual interviews with young people with JIA 8-18 years of age, parents/caregivers and HCPs using a qualitative descriptive study design. We recruited participants online across Canada and the United States, from a hospital and from a quality improvement network. We used interview guides based on the Ottawa Decision Support Framework to assess decision-making needs. We audiotaped, transcribed verbatim and analyzed interviews using thematic analysis. RESULTS: A total of 12 young people (n = 6 children and n = 6 adolescents), 13 parents/caregivers and 11 HCPs participated in interviews. Pediatric HCPs were comprised of rheumatologists (n = 4), physical therapists (n = 3), rheumatology nurses (n = 2) and occupational therapists (n = 2). The following themes were identified: (1) need to assess pain in an accurate manner; (2) need to address pain in pediatric rheumatology consultations; (3) need for information on pain management options, especially nonpharmacological approaches; (4) importance of effectiveness, safety and ease of use of treatments; (5) need to discuss young people/families' values and preferences for pain management options; and the (6) need for decision support. Themes were similar for young people, parents/caregivers and HCPs, although their respective importance varied. CONCLUSIONS: Findings suggest a need for evidence-based information and communication about pain management options, which would be addressed by decision support interventions and HCP training in pain and SDM. Work is underway to develop such interventions and implement them into practice to improve pain management in JIA and in turn lead to better health outcomes.

Pediatric Complex Regional Pain Syndrome With and Without a History of Prior Physical Trauma at Onset.

OBJECTIVE: To determine whether differences exist between children with complex regional pain syndrome (CRPS) who identify an inciting physical traumatic event (group T) versus those without such history (group NT). METHODS: We performed a single-center, retrospective study of children diagnosed with CRPS, 18 years old or younger, presenting between April 2008 and March 2021 and enrolled in a patient registry. Abstracted data included clinical characteristics, pain symptoms, Functional Disability Inventory, psychological history, and Pain Catastrophizing scale for children. Charts were reviewed for outcome data. RESULTS: We identified 301 children with CRPS, 95 (64%) reported prior physical trauma. There was no difference between the groups regarding age, sex, duration, pain level, function, psychological symptoms, and scores on the Pain Catastrophizing Scale for Children. However, those in group T were more likely to have had a cast (43% vs 23%, P < 0.001). Those in group T were less likely to experience complete resolution of symptoms (64% vs 76%, P = 0.036). There were no other outcome differences between the groups. DISCUSSION: We found minimal differences in children with CRPS who report a prior history of physical trauma to those who do not. Physical trauma may not play as significant a role as immobility, such as casting. The groups mostly had similar psychological backgrounds and outcomes.

Improving Outcomes of Pediatric Lupus Care Delivery With Provider Goal-Setting Activities and Multidisciplinary Care Models.

OBJECTIVE: The present study was undertaken to evaluate high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model using an electronic health record (EHR)-enabled pediatric lupus registry. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE). METHODS: We implemented standardized EHR documentation tools to autopopulate a SLE registry. We compared pediatric Lupus Care Index (pLCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up 1) before versus during provider goal-setting activities and population management, and 2) in a multidisciplinary lupus nephritis versus rheumatology clinic. We estimated associations between pLCI and subsequent prednisone use adjusted for time, current medication, disease activity, clinical features, and social determinants of health. RESULTS: We analyzed 830 visits by 110 patients (median 7 visits per patient [interquartile range 4-10]) over 3.5 years. The provider-directed activity was associated with improved pLCI performance (adjusted ß 0.05 [95% confidence interval (95% CI) 0.01, 0.09]; mean 0.74 versus 0.69). Patients with nephritis in multidisciplinary clinic had higher pLCI scores (adjusted ß 0.06 [95% CI 0.02, 0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted relative risk [RR] 1.27 [95% CI 1.02, 1.57]). A pLCI score of ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95% CI 0.53, 0.93). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use. CONCLUSION: Greater attention to quality metrics is associated with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery.

The Feasibility and Acceptability of Resilience Coaching for Adolescent Chronic Musculoskeletal Pain: A Single-Arm Pilot Trial.

Promoting Resilience in Stress Management (PRISM) is a well-established resilience coaching program for youth with chronic illness. It is a one-on-one intervention targeting skills in stress management, goal-setting, cognitive reframing, and meaning-making. We aimed to (i) assess the feasibility and acceptability of PRISM and (ii) explore PRISM's impact on clinical outcomes among youth with chronic musculoskeletal pain (CMP). This was a single-arm pilot trial of PRISM for youth with CMP aged 12-17 years. Patients completed patient-reported outcome measures (PROs) pre- and post- intervention; patients and caregivers provided qualitative feedback. Twenty-seven patients were enrolled (63% enrollment rate); 82% percent were female. The patients' median age was 16 years (IQR: 13-16). The intervention completion rate was 81% (n = 22). The mean satisfaction for PRISM overall was 4.3 (SD 0.9), while the mean acceptability of the intervention measure (AIM) was 4.4 (SD 0.89). Participants reported improved resilience (2.2 [SD 5.1]), functional disability (-3.5 [IQR: -6.0, 1.0]), and psychological distress (-1.0 [-5.0, 2.0]) from baseline to immediately post-treatment; pain intensity, pain catastrophizing, and global health were similar at both time points. Feedback was positive and suggested that a group component may be helpful. PRISM is feasible and acceptable among youth with CMP. Exploratory analyses suggest improvements in clinically relevant outcomes, warranting further investigation.

Recognizing medical child abuse in children presenting with chronic pain.

Medical child abuse (MCA), previously referred to as Munchausen by proxy, can present as chronic pain. We report the presentation of five children seeking treatment for chronic pain who we identified as victims of MCA. The index case had essentially not eaten for the 6 years of her life due to alleged allergies to all foods, developed severe pain, used a wheelchair for ambulation beyond a few blocks, and was alleged to have dysautonomia requiring oxygen monitoring at night. Other cases posed as arthritis that resulted in foot amputation and total body pain, fibromyalgia with alleged mutation negative Stickler syndrome who had symptoms only in her mother's presence, severe incapacitating intermittent pains along with abdominal pain that resulted in appendectomy, cholecystectomy, and pancreatectomy, and alleged disabling hypermobile Ehlers-Danlos in a non-hypermobile child for which the mother sought a power wheelchair. The unusual pattern to the pain, the presence of multiple additional, atypical symptoms and diagnoses, and a generally well appearing child are characteristic. The perpetrator is typically over-invested in the symptoms, derives tangible and intangible secondary gain from the child's alleged illnesses, and is able to present the child in such a fashion to enlist the physician to aid in perpetuating the abuse. These children are highly over-medicalized and suffer significant morbidity. Multiple barriers exist to identifying and reporting these children to Child Protective Services, which need to be recognized and overcome in order to protect these vulnerable children.

Characterizing Neurocognitive Impairment in Juvenile Fibromyalgia Syndrome: Subjective and Objective Measures of Dyscognition.

Objectives: Our understanding of brain fog, or dyscognition, among youth with juvenile fibromyalgia syndrome is limited. We aimed to determine the prevalence of subjective (self-reported) and objective dyscognition, as well as factors associated with subjective dyscognition in juvenile fibromyalgia syndrome. Methods: A cross-sectional cohort study of patients (n = 31) 12-17 years old diagnosed with primary juvenile fibromyalgia syndrome and one of their parents from 2017 to 2019. Subjects completed a series of survey measures and patients completed a brief neurocognitive battery. Subjective dyscognition was determined based on scores on the Pediatric Quality of Life Inventory (PedsQL) Cognitive Functioning Scale and Behavior Rating Inventory of Executive Function (BRIEF-2) global executive composite (GEC). Objective dyscognition was defined as impairment of more than two standard deviations in any of the neurocognitive domains. We used Fisher's exact test or Wilcoxon rank-sum test, as appropriate, to compare clinical patients based on the presence of dyscognition. Multivariable logistic regression modeling was performed to determine factors associated with subjective dyscognition. Results: Of the 31 subjects, 65% reported subjective dyscognition and 39% had objective dyscognition, primarily in the domains of psychomotor speed (23%), executive function (23%), and attention (3%). Subjective dyscognition was not indicative of objective dyscognition. Subjective dyscognition was independently associated with functional disability (OR: 1.19 [95% CI: 1.02-1.40]) and anxiety (OR: 1.12 [95% CI: 1.02-1.24]). Discussion: Adolescents with fibromyalgia predominantly experience subjective dyscognition but more than 1/3 also experience objective dyscognition. Future research should explore the impact of interdisciplinary rehabilitation programs on the treatment of dyscognition in youth with JFMS.

Exploring the intersection of adverse childhood experiences, pediatric chronic pain, and rheumatic disease.

BACKGROUND: While the general relationship between ACEs and the development of chronic pain has become increasingly clear, how ACEs may shape a child's clinical presentation with regards to chronic pain has yet to be fully expounded. We aimed to determine the association between ACEs and clinical manifestations of pediatric chronic pain and explore the interaction of ACEs and pediatric rheumatic disease among youth with chronic pain on health-related outcomes. METHODS: We conducted a cross-sectional cohort study of patients aged ≤18 years with chronic pain seen in a pediatric rheumatology amplified pain clinic between August 2018 and July 2020. We stratified subjects into three groups: no ACEs, one ACE, and ≥ 2 ACEs. We assessed clinical signs and symptoms associated with the presence of ACEs using Chi-square or Wilcoxon-rank test. The association between ACEs as well as other variables of interest with functional impairment was tested using simple and multivariable linear regression. RESULTS: Of the 412 patients included, more than 75% of patients reported at least one ACE. Most frequent included history of mental illness in a first degree relative (56%) and parental divorce or separation (20%). Those with ≥2 ACEs had more somatic symptoms, worse functional disability, and a higher proportion of mental health conditions. There appeared to be a dose dependent interaction between ACEs and functional disability from co-morbid rheumatologic disease. In multivariable regression, higher verbal pain score, symptom severity score (SSS), and presence of autonomic changes were associated with estimated average increase in FDI score (ß = 1.05, 1.95 and 4.76 respectively; all p < 0.01). CONCLUSION: Children with chronic pain and/or rheumatologic diseases who are exposed to ACEs are at increased risk of greater symptomatology, functional disability, and somatization of symptoms. Our findings indicate an ongoing need for systemic evaluation of ACEs in children with chronic pain and/or rheumatic disease and incorporation of trauma-based care.

Evaluation of pediatric rheumatology telehealth satisfaction during the COVID-19 pandemic.

BACKGROUND: During the Coronavirus disease 2019 pandemic, ambulatory pediatric rheumatology healthcare rapidly transformed to a mainly telehealth model. However, pediatric patient and caregiver satisfaction with broadly deployed telehealth programs remains largely unknown. This study aimed to evaluate patient/caregiver satisfaction with telehealth and identify the factors associated with satisfaction in a generalizable sample of pediatric rheumatology patients. METHODS: Patients with an initial telehealth video visit with a rheumatology provider between April and June 2020 were eligible. All patients/caregivers were sent a post-visit survey to assess a modified version of the Telehealth Usability Questionnaire (TUQ) and demographic and clinical characteristics. TUQ total and sub-scale (usefulness, ease of use, effectiveness, satisfaction) scores were calculated and classified as "positive" based on responses of "agree" or "strongly agree" on a 5-point Likert scale. Results were analyzed using standard descriptive statistics and Wilcoxon signed rank testing. The association between demographic and clinical characteristics with TUQ scores was assessed using univariate linear regression. RESULTS: 597 patients/caregivers met inclusion criteria, and the survey response rate was 42% (n = 248). Juvenile idiopathic arthritis was the most common diagnosis (33.5%). The majority of patients were diagnosed greater than 6 months previously (72.6%) and were prescribed chronic medications (59.7%). The median total TUQ score was 4 (IQR: 4-5) with positive responses in 81% of items. Of the subscales, usefulness scores were lowest (median: 4, p < 0.001). Telehealth saves time traveling was the highest median item score (median = 5, IQR: 4-5). Within subscales, items that scored significantly lower included convenience, providing for needs, seeing rheumatologist as well as in person, and being an acceptable way to receive rheumatology services (all p < 0.001). There were no significant demographic or clinical features associated with TUQ scores. CONCLUSIONS: Our results suggest telehealth is a promising mode of healthcare delivery for pediatric rheumatic diseases but also identifies opportunities for improvement. Innovation and research are needed to design a telehealth system that delivers high quality and safe care that improves healthcare outcomes. Since telehealth is a rapidly emerging form of pediatric rheumatology care, improved engagement and training of patients, caregivers, and providers may help improve the patient experience in the future.

Validation of coding algorithms for the identification of herpes zoster among children.

PURPOSE: To examine the validity of International Classification of Diseases, 10th Revision, (ICD-10) code-based algorithms for herpes zoster (HZ) in the electronic medical record (EMR) of a large, integrated pediatric healthcare network and to examine baseline demographics and chronic comorbidities associated with HZ in a representative pediatric population. METHODS: We reviewed the electronic charts of all patients with a single ICD-10 for HZ (B02.xx) as their primary or secondary diagnosis in the EMR of the Children's Hospital of Philadelphia (CHOP) healthcare network from January 2010-March 2019. The positive predictive value (PPV) for a single code for HZ was calculated and alternative algorithms were examined to determine which method resulted in the highest PPV. RESULTS: The PPV for a single ICD-10 code was 91.7% (95% CI 80.8-95.4) for definitive and/or probable cases of HZ and 63.9% (95% CI 53.4%-75.5%) for definitive cases alone. Adding a prescription for an antiviral did not improve the PPV. However, adding a new code for rash entered within 1 week of the HZ code increased the PPV to 100% for definitive and/or probable cases but with substantial loss of sensitivity. A high proportion of children with HZ who required inpatient hospitalization had chronic disease (70%) and were on systemic immunomodulatory therapy (50%). CONCLUSIONS: HZ can be identified with a high PPV in electronic medical records of children using ICD-10 code alone. These findings lay the foundation for future pharmacoepidemiologic research to better understand risk factors for HZ infection.

From Theory to Patient Care: A Model for the Development, Adaptation, and Testing of Psychosocial Interventions for Patients With Serious Illness.

Psychosocial and supportive care interventions are a cornerstone of palliative care science, yet there is little published guidance regarding how to develop, test, adapt, and ultimately disseminate evidence-based interventions. Our objective was to describe the application of a single intervention-development model in multiple populations of patients with serious illness. Specifically, we use the "Promoting Resilience in Stress Management" (PRISM) intervention as an exemplar for how the Obesity Related Behavioral Intervention Trials (ORBIT) intervention-development model may be applied to: 1) create an initial palliative care intervention; 2) adapt an existing intervention for a new patient-population; 3) expand an existing intervention to include new content; and, 4) consider dissemination and implementation of a research-proven intervention. We began by identifying key psychological and social science theories and translating them a testable clinical hypothesis. Next, we conducted observational studies and randomized trials to design, refine, and standardize PRISM within unique patient-populations. We moved backwards in the ORBIT model when necessary to adapt or expand PRISM content and delivery-strategies to meet patient-reported needs. Finally, we began to explore PRISM's effectiveness using Dissemination and Implementation research methods. Key lessons include the need to ground intervention-development in evidence-based theory; involve patient, clinician, and other stakeholders at every phase of development; "meet patients where they are at" with flexible delivery strategies; invest in the time to find the right scientific premise and the right intervention content; and, perhaps most importantly, involve an interdisciplinary research team.

Disordered eating among adolescents with chronic pain: the experience of a pediatric rheumatology subspecialty pain clinic.

BACKGROUND: Disordered eating and chronic pain often co-occur in adolescents, but the relationship between these conditions is not well understood. We aimed to determine the prevalence of and to identify the clinical characteristics associated with the presence of disordered eating among adolescents with chronic musculoskeletal pain (CMP) presenting to a pediatric rheumatology subspecialty pain clinic. METHODS: This was a retrospective cohort study of pediatric patients presenting to a pediatric rheumatology subspecialty pain clinic for an initial consultation from March 2018 to March 2019. We complemented data from an existing patient registry with secondary chart review for patients identified with disordered eating. We compared patient characteristics based on the presence or absence of disordered eating among adolescents with CMP. Logistic regression modeling was used to determine factors associated with disordered eating. RESULTS: Of the 228 patients who were seen for an initial consultation in the pain clinic in 1 year, 51 (22.4%) had disordered eating. Only eight (15.7%) of the 51 patients identified with disordered eating had a previously documented formal eating disorder diagnosis. Through multivariate logistic regression modeling, we found that disordered eating was associated with older age, higher functional disability, presence of abdominal pain, presence of gastrointestinal comorbidities, and presence of anxiety (all p < 0.05). CONCLUSIONS: Adolescents with chronic pain, especially those who experience gastrointestinal issues, anxiety, and greater functional disability, should be evaluated for disordered eating by the treating clinician in order to ensure timely and appropriate treatment.

Suicidal risk and resilience in juvenile fibromyalgia syndrome: a cross-sectional cohort study.

BACKGROUND: To characterize suicidality among youth with juvenile fibromyalgia syndrome (JFMS) receiving treatment from pediatric rheumatologists at a tertiary care center in order to determine the prevalence of suicidality in JFMS and to explore risk factors for persistent suicidal ideation. METHODS: We performed a cross-sectional cohort study of children 12-17 years old with JFMS seen in a specialty pediatric rheumatology pain clinic from 7/2017-9/2019. All subjects completed patient-reported outcomes measures, complemented by retrospective chart review. Subjects who endorsed item 8 on the Children's Depression Inventory, 2nd Edition (CDI-2) were categorized as endorsing suicidal ideation. We assessed for differences between the suicidal and non-suicidal patients using Wilcoxon-rank sum test. Logistic regression modeling was performed to identify psychosocial factors associated with suicidality. RESULTS: Of the 31 subjects, more than one-quarter endorsed suicidality. Nearly 90% of teens with suicidal ideation were established in outpatient counseling. In bivariate analyses, suicidality was associated with lower resilience and greater depression and anxiety (all p < 0.05). Pain intensity trended towards a statistically significant positive association (OR: 1.16 [0.99-1.37]; p = 0.06). Lower resilience was independently associated with suicidality (OR: 0.90 [95% CI: 0.82-0.98]; p < 0.02). CONCLUSIONS: Suicidality was prevalent among youth with JFMS and persistent despite concurrent receipt of mental health services. Higher patient-level resilience was independently associated with a reduced odds of suicidality. Future work should examine the role of resilience training on reducing psychological distress and mitigating the risk of suicidality in JFMS.

The spectrum of pediatric amplified musculoskeletal pain syndrome.

BACKGROUND: Children presenting with musculoskeletal pain to pediatric rheumatology clinics are very heterogeneous and on a continuum from those with localized pain to total body pain. Many report intermittent, rather than constant, pain. We examined clinical and psychological characteristics of these children at presentation and specifically those who fulfilled the criteria for fibromyalgia. METHODS: We performed a retrospective, cross-sectional cohort study of children under ≤18 years old presenting to the pediatric rheumatology pain clinic between January 2015 and July 2019 and enrolled in a patient registry. We included children diagnosed with amplified pain, excluding those fulfilling criteria for complex regional pain syndrome. Abstracted data included clinical characteristics, pain symptoms, functional disability inventory (FDI), widespread pain index, and symptom severity scale. RESULTS: We analyzed 636 subjects, predominantly non-Hispanic Caucasian females. Using median split method, 54% had diffuse pain (≥ 5 body regions involved), but, of these, only 58% met criteria for fibromyalgia. Subjects with diffuse pain, compared to those with localized pain had a longer duration of pain (24 vs 12 months, p < 0.01), reported greater pain intensity (6/10 vs 5/10, p < 0.001), greater mental health burden, and poorer function (FDI 25 vs 19, p < 0.0001). Subjects with limited pain more often reported a history of trigger event (34% vs 24%, p < 0.01) but not autonomic changes (14% vs 14%, p = 0.94). The presence of adverse childhood experiences did not differ among those with limited versus diffuse pain except for parental divorce (16% vs 23%, p = 0.03). Intermittent pain was reported in 117 children (18%) and, compared to subjects with constant pain, they reported less pain (0/10 vs 6/10) and were more functional (FDI 13 vs 25) (both p < 0.0001). CONCLUSIONS: There exists a wide spectrum of pain manifestations among children with amplified pain including limited or diffuse and constant or intermittent pain. Most children who presented to our clinic did not fulfill criteria for fibromyalgia but nonetheless had significant symptoms and disability. Studies focusing on fibromyalgia may miss the full extent of childhood amplified pain. Additionally, research limited to those meeting the fibromyalgia criteria likely underestimate the significant impact of amplified pain among the pediatric population.

Erythema Ab Igne in an Adolescent With Chronic Pain: An Alarming Cutaneous Eruption From Heat Exposure.

Erythema ab igne (EAI) is a cutaneous finding caused by prolonged heat exposure and is characterized by a reticular, brownish-pigmented, often telangiectatic dermatosis. The eruption is reminiscent of livedo reticularis, which is typically seen in the setting of a number of rheumatologic conditions, most prominently vasculitis. Identification of key features distinguishing EAI from livedo reticularis can aid in the diagnosis of EAI and correct elucidation of the underlying etiology. Our patient presented with heating pad-induced EAI in the setting of chronic pain. Only 6 other pediatric cases of EAI associated with heat sources for chronic pain are reported (Acta Derm Venereol. 2014;94:365-367, J Pediatr. 2013;163:1789, Int J Eat Disord. 2013;46:381-383, Arch Dis Child. 2008;93:389, Arch Pediatr Adolesc Med. 2012;166:185-186, Br J Clin Pract. 1990;44:248-251). Our case highlights the need for awareness of this pathognomonic skin eruption in children with chronic pain conditions to help avoid an extensive workup for vasculitis.

Validation of claims-based diagnoses of adult and pediatric neuromyelitis optica spectrum disorder and variations in diagnostic evaluation and treatment initiation.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating disease in need of more studies to determine effective treatment regimens. The rarity of the disorder, however, makes large randomized-controlled trials challenging. Validation of the International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) code for NMO could facilitate the use of large healthcare claims data for future research. We aimed 1) to determine the positive predictive value (PPV) of the ICD-9-CM code for NMO as well as evaluate case-finding algorithms for the identification of patients with NMO/NMOSD and 2) to compare the evaluation of and treatment for pediatric versus adult patients. METHODS: This was a multicenter retrospective cohort study of patients with ≥ 1 ICD-9 code for NMO seen at 3 pediatric and 2 adult United States medical centers from 2001-2016. Using a standardized data entry form, pediatric and adult neurologists and rheumatologists reviewed patients' medical records to determine whether patients fulfilled the 2006 criteria for NMO and/or the 2015 criteria for NMOSD in order to determine the positive predictive value (PPV) for the ICD-9-CM code. Demographic and clinical information was abstracted from patient medical records to ascertain variables then evaluated in case-based finding algorithms for further identification of patients with true NMO/NMOSD. We also evaluated differences in clinical characteristics between pediatric and adult patients using chi-squared or Fisher's exact tests, as appropriate, to assess for treatment variation. RESULTS: A single code for NMO had a PPV of 47% across all sites, with significant site variation (0-77%). The best case-finding algorithm included at least 5 codes as well as a documented hospitalization (PPV = =90% for children and PPV = 92% for adults). Children were more likely to be evaluated by a rheumatologist or ophthalmologist, undergo magnetic resonance imaging of the orbits, and receive immunosuppressive and biologic agents than their adult counterparts. Rituximab was administered similarly among the two groups. CONCLUSION: The ICD-9 code for neuromyelitis optica (NMO) is inaccurate for identification of NMO/NMOSD. Using case-finding algorithms increases the PPV. The initial diagnostic evaluation and treatment of NMOSD differs significantly between children and adults.

Patient-proxy agreement on health-related quality of life in juvenile fibromyalgia syndrome.

BACKGROUND: Health-related quality of life (HRQoL) measures serve as important indicators of pain-related physical and psychosocial function in youth with juvenile fibromyalgia syndrome (JFMS). While the administration of parent-proxy reported HRQoL measures in the assessment of JFMS is common, its added clinical value to patient self-reports is unclear. We aimed to determine the level of agreement on HRQoL among patients with JFMS as well as their parent-proxies and to determine factors associated with this agreement. METHODS: We performed a retrospective, cross-sectional cohort study of children aged 8 to 17 years diagnosed with JFMS and presenting for initial evaluation to a pediatric rheumatology pain clinic between April 2017 and May 2018. All patients and proxies were administered the Pediatric Quality of Life Short Form 15 Generic Core Scales (PedsQL SF-15) as part of routine clinical care. We calculated absolute discrepancy scores (absolute value of parent-proxy score minus patient score) to describe the extent of difference in HRQoL scores between parent-proxies and patients. We examined agreement between parent-proxy report and patient self-report via intraclass correlation coefficients (ICCs), stratified by age and sex, as well as Bland-Altman plots. We also used multivariate regression models to determine factors associated with level of agreement. RESULTS: A total of 65 patient-proxy pairs were included in this study. ICCs demonstrated good to excellent agreement between all parent-proxy and patient measures of HRQoL irrespective of the patient's age or sex. The level of agreement was not associated with pain duration or pain severity but less agreement on psychosocial HRQoL was associated with older patient age (ß = 1.30; p < 0.05). CONCLUSIONS: This study in youth with JFMS demonstrated good to excellent patient-proxy agreement across all domains of the PedsQL SF-15 irrespective of patient's age or sex. Our findings suggest that parent-proxy reports do not provide additional information beyond that obtained from the patient self-report of HRQoL according to the PedsQL SF-15. In order to facilitate children and adolescents with JFMS becoming partners in their own healthcare, and to decrease the burden of multiple questionnaires, we propose focusing on patients' own perceptions of HRQoL in the clinical setting.