A survey assessment of combined pediatric dermatology-rheumatology clinics.

The expertise of both dermatology and rheumatology may be beneficial when managing autoimmune conditions with cutaneous and systemic manifestations in children. This survey study was directed to pediatric dermatologists who participate in combined pediatric dermatology-rheumatology clinics; 13 sites in North America responded. The results provide information regarding clinic operations, benefits, and barriers to establishment. These findings have the potential to help institutions establish or modify combined pediatric dermatology-rheumatology clinics, although further research is needed to determine their impact.

Effects of the SARS-CoV-2 global pandemic on U.S. rheumatology outpatient care delivery and use of telemedicine: an analysis of data from the RISE registry.

The SARS-CoV-2 global pandemic resulted in major disruptions to medical care. We aimed to understand changes in outpatient care delivery and use of telemedicine in U.S. rheumatology practices during this period. Rheumatology Informatics System Effectiveness (RISE) is a national, EHR-enabled registry that passively collects data on all patients seen by participating practices. Included practices were required to have been participating in RISE from January 2019 through August 2020 (N = 213). We compared total visit counts and telemedicine visits during March-August 2020 to March-August 2019 and stratified by locations in states with shelter-in-place (SIP) orders. We assessed characteristics of patients within each practice, including primary rheumatic diagnosis and disease activity scores, where available. We included 213 practices with 945,160 patients. Overall, we found visit counts decreased by 10.9% (from 1,302,455 to 1,161,051) between March and August 2020 compared to 2019; this drop was most dramatic during the month of April (- 22.3%). Telemedicine visits increased from 0% to a mean of 12.1%. Practices in SIP states had more dramatic decreases in visits, (11.5% vs. 5.3%). We found no major differences in primary diagnoses or disease activity across the two periods. We detected a meaningful decrease in rheumatology visits in March-August 2020 during the SARS-CoV-2 global pandemic compared to the year prior with a concomitant increase in the use of telemedicine. Future work should address possible adverse consequences to patient outcomes due to decreased contact with clinicians.

A retrospective study: Impact of consensus treatment plans on systemic therapy of pediatric morphea.

BACKGROUND: Morphea is an inflammatory and fibrosing condition that affects the skin and subcutaneous structures. Morphea is managed by dermatologists, rheumatologists, or both. Prior studies have suggested there is significant variability in approach to treatment. In 2012, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) for pediatric morphea to develop more standardized treatment plans for patients requiring systemic therapy. We aimed to assess whether the publication of CTPs has impacted care of patients with morphea at our institution. METHODS: Data were collected via a retrospective review of medical records of 61 pediatric patients diagnosed with morphea at Seattle Children's Hospital (SCH) from January 1, 2005, to December 12,2017. RESULTS: Prior to the publication of CTPs, 2 out of 24 patients (8.3%) were treated with a regimen that matched a subsequent CTP. After publication of CTPs, 29 out of 37 patients (78.4%) were treated with a regimen that matched a CTP (P < 0.001). A subanalysis was performed to assess the number of patients who needed second- or third-line therapies. Of those who followed a CTP therapy plan (n = 26), 3 patients (11.5%) needed a second-line therapy compared with 11 patients (44%) in the no-CTP followed group (n = 25), (P = 0.012). CONCLUSIONS: The publication of CTPs led to a significant change in treatment approach for patients with morphea requiring systemic therapy at SCH. Patients treated with one of the treatment plans recommended by the CTPs were less likely to need second-line systemic therapy.

Cutaneous polyarteritis nodosa in pediatric patients successfully treated with TNF-α inhibitor and methotrexate: Case series and literature review.

Cutaneous polyarteritis nodosa (CPAN) is a rare necrotizing vasculitis affecting small- to medium-sized arteries. Reported treatments include oral corticosteroids alone or in combination with non-steroidal antiinflammatory drugs, intravenous immunoglobulins, cyclophosphamide, azathioprine, colchicine, or dapsone. However, some patients with CPAN do not respond to such treatments and continue to experience exacerbations over prolonged periods. This series provides support for the use of TNF-α inhibitors in the treatment of recalcitrant CPAN in pediatric patients.

The American English version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the American English language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 315 JIA patients (5.1% systemic, 31.1% oligoarticular, 34% RF negative polyarthritis, 29.8% other categories) and 98 healthy children, were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the American English version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

The central nervous system manifestations of localized craniofacial scleroderma: a study of 10 cases and literature review.

BACKGROUND: Localized craniofacial scleroderma is a rare pediatric disease that involves a spectrum of discoloration, fibrosis and hemiatrophy of the face and scalp. Children with localized craniofacial scleroderma may have neurological symptoms, and in this context often undergo diagnostic imaging of the brain. OBJECTIVE: To catalogue neuroimaging abnormalities in patients with localized craniofacial scleroderma treated at our institution, review their clinical courses and compare this data with prior studies. MATERIALS AND METHODS: Following Institutional Review Board approval, an imaging database search identified 10 patients with localized craniofacial scleroderma and neuroimaging abnormalities treated at our institution. Neuroimaging exams and the electronic medical record were reviewed for each case. RESULTS: The most common indications for neuroimaging were headache or seizure (80% of cases). The most common neuroimaging abnormalities were T2-hyperintense, subcortical white matter lesions ipsilateral to the cutaneous lesion (90% of cases) on magnetic resonance imaging (MRI). Calcifications or blood products (50%), cysts (40%) and abnormal enhancement (20%) were also observed. A positron emission tomography (PET) scan obtained for a single case demonstrated diminished 18F-fluorodeoxyglucose (FDG) avidity corresponding to the dominant focus of signal abnormality on MRI. Progressive neuroimaging abnormalities were present in 30% of cases. There was no consistent relationship between changes in neurological symptoms following treatment and neuroimaging findings. CONCLUSION: Our results are similar to previously published data. In the absence of new or worsening neurological symptoms, the role of neuroimaging for follow-up of localized craniofacial scleroderma is unclear. Knowledge of intracranial neuroimaging abnormalities that are commonly associated with localized craniofacial scleroderma helps to distinguish these lesions from others that have similar appearance.

Congruence between polysomnography obstructive sleep apnea and the pediatric sleep questionnaire: fatigue and health-related quality of life in juvenile idiopathic arthritis.

PURPOSE: To examine the congruence between polysomnography obstructive apnea hypopnea index (OAHI) and parent-reported obstructive sleep apnea (OSA) symptoms in 6- to 11-year-old children with juvenile idiopathic arthritis (JIA) and controls; and to compare fatigue and quality of life in JIA and control children based on OAHI and OSA symptoms. METHODS: Sixty-eight children with JIA and 75 controls and a parent participated. Children underwent one night of polysomnography in a sleep laboratory. Parents completed the sleep-related breathing disorders scale-pediatric sleep questionnaire (PSQ), and both children and parents completed the Pediatric Quality of Life Generic Core Scale and the Multidimensional Fatigue Scale. RESULTS: In JIA, 86% who met the OAHI clinical criteria for OSA (≥1.5) were above the PSQ OSA symptom cut-off score with a sensitivity of 0.86 and a specificity of 0.28. In the control group, 63% who met the OAHI clinical criteria for OSA were above the PSQ OSA symptom cut-off score, with a sensitivity of 0.63 and a specificity of 0.42. All children above both the clinical criteria for OAHI and OSA symptom cut-off score had the most impaired quality of life and greater fatigue compared to those below both the clinical criteria for OAHI and the OSA symptom cut-off score. CONCLUSION: Children who meet clinical criteria for OSA and also scored high on a parent-reported screening tool for OSA symptoms had the most impaired quality of life and more fatigue. The PSQ has potential to identify children at risk for OSA.

Daily Sleep Patterns, Sleep Quality, and Sleep Hygiene Among Parent-Child Dyads of Young Children Newly Diagnosed With Juvenile Idiopathic Arthritis and Typically Developing Children.

OBJECTIVES: Describe daily sleep patterns, sleep quality, and sleep hygiene in 2-5-year-old children newly diagnosed with juvenile idiopathic arthritis (JIA) and their parents in comparison with typically developing (TD) children and parents. METHODS: Participants (13 JIA, 16 TD parent-child dyads) wore actigraphs for 10 days. Parents completed sleep diaries and sleep hygiene survey. RESULTS: Children with JIA had significantly less total sleep time, lower sleep efficiency (SE), and longer naps than TD children. Parents of children with JIA had significantly earlier bedtimes, more wake after sleep onset (WASO) and lower SE than TD parents. Parent-child SE and WASO were interrelated in JIA dyads. Sleep hygiene practices were inconsistent in both groups of children. CONCLUSIONS: Inadequate amounts of sleep and poor sleep quality were common in parent-child dyads. Early interventions to improve sleep duration and promote sleep hygiene practices may alleviate future sleep problems and improve parent and child well-being.

Sleep disturbances and behavior problems in children with and without arthritis.

The study compared sleep disturbances and behavior problems in school-age children with and without juvenile idiopathic arthritis (JIA). Children 6-to-11 years of age, with (n=70) and without (n=46) JIA, and their parent participated. Parents completed questionnaires on sleep habits and behavior problems. Compared to control children, JIA children had significantly higher total sleep disturbances and higher scores on six of eight subscales. Sleep disturbances predicted externalizing behavior problems, controlling for age, medications, study group, and pain. Sleep disturbances such as, sleep disordered breathing are often overlooked or unrecognized in JIA and may contribute to behavioral problems.

Trial of early aggressive therapy in polyarticular juvenile idiopathic arthritis.

OBJECTIVE: To determine whether aggressive treatment initiated early in the course of rheumatoid factor (RF)-positive or RF-negative polyarticular juvenile idiopathic arthritis (JIA) can induce clinical inactive disease within 6 months. METHODS: Between May 2007 and October 2010, a multicenter, prospective, randomized, double-blind, placebo-controlled trial of 2 aggressive treatments was conducted in 85 children ages 2-16 years with polyarticular JIA of <12 months' duration. Patients received either methotrexate (MTX) 0.5 mg/kg/week (maximum 40 mg) subcutaneously, etanercept 0.8 mg/kg/week (maximum 50 mg), and prednisolone 0.5 mg/kg/day (maximum 60 mg) tapered to 0 by 17 weeks (arm 1), or MTX (same dosage as arm 1), etanercept placebo, and prednisolone placebo (arm 2). The primary outcome measure was clinical inactive disease at 6 months. An exploratory phase determined the rate of clinical remission on medication (6 months of continuous clinical inactive disease) at 12 months. RESULTS: By 6 months, clinical inactive disease had been achieved in 17 (40%) of 42 patients in arm 1 and 10 (23%) of 43 patients in arm 2 (χ(2) = 2.91, P = 0.088). After 12 months, clinical remission on medication was achieved in 9 patients in arm 1 and 3 patients in arm 2 (P = 0.053). There were no significant interarm differences in adverse events. CONCLUSION: Although this study did not meet its primary end point, early aggressive therapy in this cohort of children with recent-onset polyarticular JIA resulted in clinical inactive disease by 6 months and clinical remission on medication within 12 months of treatment in substantial proportions of patients in both arms.

Probability of Response in the First Sixteen Weeks After Starting Biologics: An Analysis of Juvenile Idiopathic Arthritis Biologics Trials.

OBJECTIVES: Most juvenile idiopathic arthritis (JIA) biologic disease-modifying antirheumatic drugs (bDMARDs) trials used an open-label run-in period followed by randomized medication withdrawal. We used data from the run-in period of 4 bDMARD trials to 1) delineate early response trajectory to bDMARDs and 2) identify predictors of early response. METHODS: Data from the first 16 weeks of 4 bDMARD trials were used. The primary outcome was the American College of Rheumatology (ACR) Pediatric 50 (Pedi 50) response criteria: clinically significant response defined as ACR Pedi 50 or greater. The secondary outcome was the clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10) minimal disease activity state. Response transition rates and predictors were modeled using an inhomogeneous Markov multistate model. RESULTS: Five hundred thirty-two participants (70% receiving methotrexate, 41% prednisone) were included. By month 4, the probability of attaining ACR Pedi 50 or greater was 0.698. If ACR Pedi 50 or more was not achieved by month 1, the probability of achieving it by month 4 was 0.60. If ACR Pedi 50 or more was not achieved by month 3, the probability of achieving this by month 4 was 0.31. Age at diagnosis, disease duration, baseline rheumatoid factor, and active joint counts predicted ACR and cJADAS state transitions, adjusted for concomitant treatment. CONCLUSIONS: No response ACR Pedi 50 or more by month 1 after treatment was associated with a 0.60 probability of responding by month 4, but not responding by month 3 was associated with a 0.31 probability of response by month 4. Baseline disease duration, rheumatoid factor, and active joint counts predicted early treatment response (ACR and cJADAS10 states).

Disease Recapture Rates After Medication Discontinuation and Flare in Juvenile Idiopathic Arthritis: An Observational Study Within the Childhood Arthritis and Rheumatology Research Alliance Registry.

OBJECTIVE: Children with well-controlled juvenile idiopathic arthritis (JIA) frequently experience flares after medication discontinuation, but the outcomes of these flares have not been well described. The objective of this study was to characterize the rates and predictors of disease recapture among children with JIA who restarted medication to treat disease flare. METHODS: Children with JIA who discontinued conventional synthetic or biologic disease-modifying antirheumatic drugs for well-controlled disease but subsequently experienced a flare and restarted medication were identified from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry. The primary outcome was inactive disease (ID) (physician global assessment <1 and active joint count = 0) 6 months after flare. RESULTS: A total of 333 patients had complete data for ID at 6 months after flare. The recapture rate for the cohort was 55%, ranging from 47% (persistent oligoarthritis) to 69% (systemic arthritis) (P = 0.4). Approximately 67% of children achieved ID by 12 months. In the multivariable model, history and reinitiation of biologic drugs were associated with increased odds of successful recapture (odds ratio [OR] 4.79 [95% confidence interval (95% CI) 1.22-18.78] and OR 2.74 [95% CI 1.62-4.63], respectively). Number of joints with limited range of motion was associated with decreased odds (OR 0.83 per 1 joint increase [95% CI 0.72-0.95]). CONCLUSION: Approximately half of JIA flares post-discontinuation were recaptured within 6 months, but rates of recapture varied across JIA categories. These findings inform shared decision-making for patients, families, and clinicians regarding the risks and benefits of medication discontinuation. Better understanding of biologic predictors of successful recapture in JIA are needed.

Construct validity of Patient-Reported Outcomes Measurement Information System Paediatric measures in juvenile idiopathic arthritis and systemic lupus erythematosus: cross-sectional evaluation.

OBJECTIVES: Evaluate construct validity of Patient-Reported Outcomes Measurement Information System (PROMIS) Paediatric measures of symptoms and functioning against measures of disease activity among youth with juvenile idiopathic arthritis (JIA) or systemic lupus erythematosus (SLE). DESIGN: Cross-sectional associations among PROMIS measures and clinical metrics of disease activity were estimated. SETTING: Seven clinical sites of the Childhood Arthritis and Rheumatology Alliance (CARRA) in the USA. PARTICIPANTS: Youth aged 8-17 years enrolled in the CARRA Registry. INTERVENTION: PROMIS measures were collected and associations with clinical measures of disease activity estimated, by condition, in bivariate and multivariable analyses with adjustment for sociodemographics, insurance status, medications and disease duration. MAIN OUTCOME MEASURES: PROMIS Paediatric measures of mobility, physical activity, fatigue, pain interference, family relationships, peer relationships, depressive symptoms, psychological stress, anxiety, and meaning and purpose, and clinical metrics of disease. RESULTS: Among 451 youth (average age 13.8 years, 71% female), most (n=393, 87%) had a JIA diagnosis and the remainder (n=58, 13%) had SLE. Among participants with JIA, those with moderate/high compared with low/inactive disease had, on average, worse mobility (multivariable regression coefficient and 95% CIs) (-7.40; -9.30 to -5.50), fatigue (3.22; 1.02 to 5.42), pain interference (4.76; 3.04 to 6.48), peer relationships (-2.58; -4.52 to -1.64), depressive symptoms (3.00; 0.96 to 5.04), anxiety (2.48; 0.40 to 4.56) and psychological stress (2.52; 0.68 to 4.36). For SLE, youth with active versus inactive disease had on average worse mobility (-5.07; -10.15 to 0.01) but PROMIS Paediatric measures did not discriminate participants with active and inactive disease in adjusted analyses. CONCLUSIONS: Seven PROMIS Paediatric measures discriminated between active and inactive disease in youth with JIA. Results advance the usefulness of PROMIS for understanding well-being and improving interventions for youth with JIA, but larger studies are needed to determine utility in SLE cohorts. TRIAL REGISTRATION NUMBER: National Institute of Arthritis and Musculoskeletal and Skin Diseases (U19AR069522).

Assessment of Real-World Patient-Reported Outcomes in Patients Initiating Biologic Agents for the Treatment of Autoimmune Diseases: An Observational Study in Four Patient-Powered Research Networks.

Background: The most reliable and meaningful approach for inclusion of patient-reported outcomes (PROs) in the evaluation of real-world clinical effectiveness of biologics in the treatment of autoimmune diseases is u ncertain. This study aimed to assess and compare the proportions of patients who had abnormalities in PROs measuring important general health domains at the initiation of treatment with biologics, as well as the effects of baseline abnormalities on subsequent improvement. Methods: PROs were collected for patient participants with inflammatory arthritis, inflammatory bowel disease, and vasculitis using Patient-Reported Outcomes Measurement Information System instruments. Scores were reported as T-scores normalized to the general population in the United States. Baseline PROs scores were collected near the time of biologic initiation, and follow-up scores were collected 3 to 8 months later. In addition to summary statistics, the proportion of patients with PROs abnormalities (scores ≥5 units worse than the population norm) was determined. Baseline and follow-up scores were compared, and an improvement of ≥5 units was considered significant. Results: There was wide variation across autoimmune diseases in baseline PROs scores for all domains. For example, the proportion of participants with abnormal baseline pain interference scores ranged from 52% to 93%. When restricted to participants with baseline PROs abnormalities, the proportion of participants experiencing an improvement of ≥5 units was substantially higher. Conclusion: As expected, many patients experienced improvement in PROs following initiation of treatment with biologics for autoimmune diseases. Nevertheless, a substantial proportion of participants did not exhibit abnormalities in all PROs domains at baseline, and these participants appear less likely to experience improvement. For PROs to be reliably and meaningfully included in the evaluation of real-world medication effectiveness, more knowledge and careful consideration are needed to select the most appropriate patient populations and subgroups for inclusion and evaluation in studies measuring change in PROs.

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations.

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA.

Latent classes of early response trajectories to biologics initiation in juvenile idiopathic arthritis: an analysis of four trials.

AIMS: 1) To delineate latent classes of treatment response to biologics in juvenile idiopathic arthritis (JIA) patients in the first 16 weeks after initiation. 2) To identify predictors of early disease response. METHODS: The study population was drawn from four biologics trials in polyarticular course JIA: Etanercept 2000, Abatacept 2008, TRial of Early Aggressive Therapy (TREAT) 2012 and Tocilizumab 2014. The outcome was active joint counts (AJC). Semiparametric latent class trajectory analysis was applied to identify latent classes of response to treatment; AJC was transformed for this modelling. We tested baseline disease and treatment characteristics for their abilities to predict class membership of response. RESULTS: There were 480 participants, 74% females. At baseline, 26% were rheumatoid factor positive. 67% were on methotrexate at enrollment. Three latent class solution provided the best fit. Baseline AJC was the sole best predictor of class membership. Participants classified by their highest membership probabilities into high baseline AJC (> 30) and slow response (26.5%), low baseline AJC (< 10), early and sustained response (29.7%), and moderate baseline AJC progressive response (43.8%). Participants were classified into the latent classes with a mean class membership posterior probability of 0.97. Those on methotrexate at baseline were less likely to belong to high baseline AJC class. CONCLUSIONS: Three latent classes of responses were detectable in the first 16 weeks of biologics therapy. Those with the highest baseline AJC demonstrated very slow response in this window and were less likely to be on concomitant methotrexate. TRIALS REGISTRATION: TREAT 2012 (NCT NCT00443430 ) (Wallace et. al, Arthritis Rheum 64:2012-21, 2012), tocilizumab trial 2014 ( NCT00988221 ), abatacept trial 2008 ( NCT00095173 ). Etanercept 2000 from Amgen does not have a trial registration number.

Operational Effect of COVID-19 on Surgical Care at a Tertiary Pediatric Hospital.

The detrimental effects of the coronavirus disease 2019 (COVID-19) pandemic have profoundly disrupted surgical care at health care facilities worldwide. At our tertiary pediatric hospital, we made substantial adjustments to surgical suite utilization and staff member scheduling to account for reductions in surgical volume, increased demand for staff members in other sectors of the hospital, and the highly infectious properties of the virus. Perioperative leaders took advantage of the pandemic's disruption to clinical activities to design and implement a new procedure-scheduling process to rectify the inefficiencies that had accumulated as the previous system evolved. The implementation of said directives was largely facilitated by establishing communication with all involved parties for their input and feedback throughout the process. Although COVID-19 has had varying effects on procedural operations across pediatric health care facilities, we believe our institutional response to the disruptive forces of COVID-19 is of benefit to pediatric hospitals worldwide.

The 2010 American College of Rheumatology/European League Against Rheumatism classification criteria for rheumatoid arthritis: Phase 2 methodological report.

OBJECTIVE: The American College of Rheumatology and the European League Against Rheumatism have developed new classification criteria for rheumatoid arthritis (RA). The aim of Phase 2 of the development process was to achieve expert consensus on the clinical and laboratory variables that should contribute to the final criteria set. METHODS: Twenty-four expert RA clinicians (12 from Europe and 12 from North America) participated in Phase 2. A consensus-based decision analysis approach was used to identify factors (and their relative weights) that influence the probability of "developing RA," complemented by data from the Phase 1 study. Patient case scenarios were used to identify and reach consensus on factors important in determining the probability of RA development. Decision analytic software was used to derive the relative weights for each of the factors and their categories, using choice-based conjoint analysis. RESULTS: The expert panel agreed that the new classification criteria should be applied to individuals with undifferentiated inflammatory arthritis in whom at least 1 joint is deemed by an expert assessor to be swollen, indicating definite synovitis. In this clinical setting, they identified 4 additional criteria as being important: number of joints involved and site of involvement, serologic abnormality, acute-phase response, and duration of symptoms in the involved joints. These criteria were consistent with those identified in the Phase 1 data-driven approach. CONCLUSION: The consensus-based, decision analysis approach used in Phase 2 complemented the Phase 1 efforts. The 4 criteria and their relative weights form the basis of the final criteria set.

Very high serum ferritin levels are associated with increased mortality and critical care in pediatric patients.

OBJECTIVE: To determine whether an elevated serum ferritin level is independently associated with mortality and receipt of critical care in pediatric patients. DESIGN: Retrospective cohort study, open population. SETTING: Seattle Children's Hospital, Seattle, WA, from September 2, 2003, to February 15, 2008. PATIENTS: All patients tested for serum ferritin level from September 2, 2003, to August 16, 2007, with a level ≥1000 ng/mL. INTERVENTIONS: None. MAIN ANALYSIS: Cox regression. MEASUREMENTS AND MAIN RESULTS: The predictor of interest was the patient-specific peak serum ferritin level, dichotomized a priori at 3000 ng/mL. The outcomes were mortality and intensive care unit admission. A total of 171 patients met the inclusion criteria. The observation time without death or intensive care unit admission ranged from 184 to 1621 days. The hazard ratio of death with peak ferritin of >3000 ng/mL was 4.32 (95% confidence interval 2.21-8.47, p < .001) compared to peak ferritin of 1000-3000 ng/mL. The hazard ratio of intensive care unit admission with peak ferritin of >3000 ng/mL was 2.49 (95% confidence interval 1.53-4.05, p < .001) compared to peak ferritin of 1000-3000 ng/mL. Both estimates were adjusted for bone marrow transplant, solid organ transplant, hemoglobinopathy, and existing rheumatologic disease. CONCLUSION: In this pediatric population, with serum ferritin levels of >3000 ng/mL, there was increased risk for both receipt of critical care and subsequent death.

Outcome Measures in Pediatric Rheumatic Disease.

Reliable and responsive outcome measures that accurately detect changes in disease state, activity, and damage are crucial to conducting observational and interventional trials that can directly transform care for children with rheumatic disease. A combination of consensus-based and direct measurement approaches has led to the development of several validated, composite outcome measures in juvenile idiopathic arthritis, juvenile dermatomyositis, childhood-onset systemic lupus erythematosus, and pediatric vasculitis. This review outlines clinician-reported, disease-specific outcome measures developed for these conditions.