Histocompatibility Leukocyte Antigen-A29-Associated Retinal Vasculitis without Choroidal Lesions: A Report of 4 Cases.

This study describes a cohort of patients presenting with histocompatibility leukocyte antigen (HLA)-A29-associated retinal vasculitis without choroidal lesions that may share clinical features with birdshot retinochoroiditis. The methods include a retrospective chart review of patients presenting with HLA-A29-associated retinal vasculitis without choroidal lesions. The data on the patients were entered retrospectively into a new database and analyzed. Four patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 40 years (range: 14-71); 75% were female. At presentation, all four patients had a visual acuity of 20/50 or better in both eyes. All the eyes had mild vitritis, three eyes (37.5%) had cystoid macular edema, and two eyes (25%) had optic disc edema. All the patients required treatment with systemic steroids and immunosuppressive therapy. HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for additional studies.

Development and Validation of a Juvenile Spondyloarthritis Disease Flare Measure: Ascertaining Flare in Patients With Inactive Disease.

OBJECTIVE: Our objective was to develop and validate a composite disease flare definition for juvenile spondyloarthritis (SpA) that would closely approximate the clinical decision made to reinitiate or not reinitiate systemic therapy after therapy de-escalation. METHODS: Retrospective chart reviews of children with SpA who underwent systemic therapy de-escalation of biologic or conventional disease-modifying antirheumatic drugs were used to develop and validate the flare outcome. Data on independent cohorts for development (1 center) and validation (4 centers) were collected from large tertiary health care systems. Core measure thresholds and candidate disease flare outcomes were assessed using sensitivity, specificity, positive predictive values (PPVs) and negative predictive values (NPVs), and the receiver operating characteristic (ROC) area under the curve (AUC), with physician assessment of active disease plus re-initiation of standard dose of systemic therapy as the reference standard. RESULTS: Of the candidate definitions, clinically meaningful worsening in ≥3 of the following 5 core measures performed best: caregiver/patient assessment of well-being; physician assessment of disease activity; caregiver/patient assessment of pain, physical function, and active joint count. The ROC AUC was 0.91, PPV 87.5%, NPV 98.1%, sensitivity 82.4%, and specificity 98.7%. Cronbach's α was 0.81, signifying internal consistency, and factor analysis demonstrated that the outcome measured 1 construct. The Juvenile SpA Flare measure had face validity according to 21 surveyed pediatric rheumatologists. Juvenile SpA Flare had an ROC AUC of 0.85, a PPV of 92.3%, and an NPV of 96.8% in the validation cohort. CONCLUSION: There is initial support for the validity of the Juvenile SpA Flare measure as a tool to identify disease flare in juvenile SpA patients de-escalating therapy, and the measure is potentially applicable in clinical practice, observational studies, and therapeutic trials.

Capturing critical data elements in Juvenile Idiopathic Arthritis: initiatives to improve data capture.

BACKGROUND: Documentation of critical data elements is a focus of the Pediatric Rheumatology Care and Outcomes Improvement Network to aid in clinical care and research for patients with juvenile idiopathic arthritis. We aimed to increase data capture for critical data elements and hypothesized that quality improvement methodology would improve data capture. We also hypothesized that data capture for all critical data elements would be lower for virtual visits compared to in-person visits. METHODS: All visits for patients with JIA between 9/14/2020 and 12/31/2021 at the University of Minnesota were included. We assessed completeness of critical data element capture. Sixteen interventions with providers were conducted, including email reminders, individual discussions, group meetings, and feedback reports. We used statistical process control charts to evaluate change over time. RESULTS: Baseline included 355 patient-visits: 221 (62%) in-person and 134 (38%) virtual with critical data elements entry ranging between 50 and 60%. Post-intervention included 1,596 patient-visits: 1,350 (85%) in-person and 246 (15%) virtual, with critical data elements entry reaching 91%. All providers improved data entry during this study. In-person visits had significantly higher data capture rates than virtual visits for all 4 critical data elements. CONCLUSION: We achieved our aim to increase critical data element documentation by focusing on provider buy-in, frequent reminders, and individualized feedback. We also found that collection of critical data elements occurred significantly less often with virtual visits than with in-person visits. Now that we improved capture of critical data elements, we can shift the focus to efforts aimed at improving outcomes for patients with juvenile arthritis.

Consensus Approach to a Treat-to-target Strategy in Juvenile Idiopathic Arthritis Care: Report From the 2020 PR-COIN Consensus Conference.

OBJECTIVE: Treat to target (T2T) is a strategy of adjusting treatment until a target is reached. An international task force recommended T2T for juvenile idiopathic arthritis (JIA) treatment. Implementing T2T in a standard and reliable way in clinical practice requires agreement on critical elements of (1) target setting, (2) T2T strategy, (3) identifying barriers to implementation, and (4) patient eligibility. A consensus conference was held among Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) stakeholders to inform a statement of understanding regarding the PR-COIN approach to T2T. METHODS: PR-COIN stakeholders including 16 healthcare providers and 4 parents were invited to form a voting panel. Using the nominal group technique, 2 rounds of voting were held to address the above 4 areas to select the top 10 responses by rank order. RESULTS: Incorporation of patient goals ranked most important when setting a treatment target. Shared decision making (SDM), tracking measurable outcomes, and adjusting treatment to achieve goals were voted as the top elements of a T2T strategy. Workflow considerations, and provider buy-in were identified as key barriers to T2T implementation. Patients with JIA who had poor prognostic factors and were at risk for high disease burden were leading candidates for a T2T approach. CONCLUSION: This consensus conference identified the importance of incorporating patient goals as part of target setting and of the influence of patient stakeholder involvement in drafting treatment recommendations. The network approach to T2T will be modified to address the above findings, including solicitation of patient goals, optimizing SDM, and better workflow integration.

Anti-MDA5 juvenile idiopathic inflammatory myopathy with second-degree heart block but no skin or lung involvement: a case report.

BACKGROUND: Patients with idiopathic inflammatory myopathy and autoantibodies directed against melanoma differentiation-associated protein 5 (MDA5) characteristically have interstitial lung disease, severe cutaneous involvement, arthritis, and relatively mild myositis. Cardiac involvement in idiopathic inflammatory myopathy can occur and has been associated with anti-signal recognition particle and anti-polymyositis-scleroderma autoantibodies, but not with anti-MDA5 autoantibodies. CASE PRESENTATION: A 14-year-old male presented with weakness, second-degree heart block, arthritis, and hematologic cytopenias. Imaging and biopsies confirmed the diagnosis of juvenile idiopathic inflammatory myopathy, and he had high titer anti-MDA5 autoantibodies. There were no cutaneous or pulmonary abnormalities. While on prednisone and methotrexate, the patient's heart block improved from second- to first-degree and the cytopenias resolved. Persistent myositis prompted the addition of intravenous immunoglobulin. Seven months into the disease course, the arthritis and myositis are in remission and the patient is no longer taking corticosteroids. CONCLUSIONS: We report a novel case of a patient with juvenile idiopathic myositis who lacked the typical cutaneous and pulmonary findings associated with anti-MDA5 positivity, but who had cardiac conduction defects. This report broadens the clinical spectrum of anti-MDA5-associated inflammatory myopathy.

Environmental variables as potential modifiable risk factors of preterm birth in Philadelphia, PA.

OBJECTIVE: To examine whether variation in neighborhood context is associated with preterm birth (PTB) outcomes and gestational age (GA) at delivery in Philadelphia, and to determine whether these associations might persist when considering relevant individual-level variables. STUDY DESIGN: We analyzed individual-level data collected for a prospective cohort study of singleton pregnancies with preterm labor. We merged block-group level data to each individual's home address. Unadjusted analyses were performed to determine the association between block-group variables and individual-level outcomes. Block-group variables identified as potential risk factors were incorporated into multivariable individual-level models to determine significance. RESULTS: We analyzed data for 817 women. The prevalence of PTB <37 weeks was 41.5%. Although in unadjusted analyses several block-group variables were associated with PTB and GA at delivery, none retained significance in individual-level multivariable models. CONCLUSION: Block-group level data were not associated with PTB outcomes or GA at delivery in Philadelphia.

Prenatal inflammation is associated with adverse neonatal outcomes.

OBJECTIVE: The purpose of this study was to determine whether prenatal inflammation (as assessed by clinical chorioamnionitis, maternal temperature >38°C, or histologic chorioamnionitis) is associated with a composite adverse neonatal outcome. STUDY DESIGN: We performed a prospective cohort study of women at 22 weeks to 33 weeks 6 days' gestation with symptoms of labor (April 2009 to March 2012). Relevant maternal and neonatal exposures and outcomes were recorded. Multivariable logistic regression was performed to determine the association between prenatal inflammation and neonatal outcomes that were controlled for potential confounders. RESULTS: We analyzed 871 mother-infant pairs. The preterm birth rate was 42.0%. When we controlled for infant sex and modified the data by gestational age at delivery, prenatal inflammation remains a significant risk factor for adverse neonatal outcomes, despite advancing gestational age: clinical chorioamnionitis at 32 weeks' gestation (odds ratio [OR], 3.12; 95% confidence interval [CI], 1.02-9.52], at 36 weeks' gestation (OR, 8.88; 95% CI, 4.32-18.25), and at 40 weeks' gestation (OR, 25.30; 95% CI, 9.25-69.19); maternal temperature >38°C at 32 weeks' gestation (OR, 3.18; 95% CI, 0.66-15.42), at 36 weeks gestation (OR, 8.40; 95% CI, 3.60-19.61), and at 40 weeks gestation (OR, 22.19; 95% CI, 8.15-60.44); histologic chorioamnionitis at 32 weeks gestation (OR, 1.25; 95% CI, 0.64-2.46), at 36 weeks gestation (OR, 2.56; 95% CI, 1.54-4.23), and at 40 weeks gestation (OR, 5.23; 95% CI, 1.95-13.99). CONCLUSION: The protective association with advancing gestational age is diminished when prenatal inflammation is present.

Biomarkers and cervical length to predict spontaneous preterm birth in asymptomatic high-risk women.

OBJECTIVE: To investigate whether biomarkers from different pathways of spontaneous preterm birth (cervical membrane degradation [fetal fibronectin], cervical remodeling [soluble E-cadherin], and inflammation (elafin, surfactant protein-D, interleukin-6 [IL-6]) were superior to one biomarker alone in predicting preterm birth. Our secondary objective was to examine the association of these biomarkers with cervical length in predicting preterm birth. METHODS: We performed a single-center, prospective cohort study from August 2011 to November 2012 of asymptomatic women at risk for spontaneous preterm birth as a result of obstetric and gynecologic history. Cervicovaginal fluid and cervical length measurements were collected at two time points (20-23 6/7 weeks and 24-27 6/7 weeks of gestation). RESULTS: Among the 104 women with complete data, the preterm birth rate was 24.5%. Prior preterm birth (P=.006) and cervical length at visit 1 (P=.003) were significantly associated with preterm birth, whereas fetal fibronectin and median biomarker levels (elafin, soluble E-cadherin, IL-6) were not. Median surfactant protein-D levels at visit 1 by preterm birth status were statistically but not clinically different (0.44 ng/mL compared with 0.40 ng/mL, P<.001). Analyses of biomarkers from more than one pathway were not superior to single biomarker analyses in predicting prematurity. Neither inclusion of biomarkers nor fetal fibronectin improved the predictive ability of cervical length alone. CONCLUSION: Cervical length assessment and obstetric history but not fetal fibronectin or biomarkers were useful in the risk stratification of women identified to be at greatest risk for spontaneous preterm birth. LEVEL OF EVIDENCE: II.