First name
R
Last name
Xiao

Title

Development and validation of the juvenile spondyloarthritis disease flare (JSpAflare) measure: Ascertaining flare in patients with inactive disease.

Year of Publication

2021

Date Published

2021 Aug 07

ISSN Number

2151-4658

Abstract

<p><strong>OBJECTIVE: </strong>Our objective was to develop and validate a composite disease flare definition for juvenile spondyloarthritis that would closely approximate the clinical decision made to reinitiate/not reinitiate systemic therapy after therapy de-escalation.</p>

<p><strong>METHODS: </strong>Retrospective chart reviews of children with spondyloarthritis who underwent systemic therapy de-escalation of biologic or conventional disease-modifying antirheumatic drugs (bDMARDs; cDMARDs) were used to develop and validate the flare outcome. Independent cohorts for development (1 center) and validation (4 centers) were collected from large tertiary healthcare systems. Core measure thresholds and candidate disease flare outcomes were assessed using sensitivity, specificity, positive (PPV) and negative predictive values (NPV), and area under the receiver operating characteristic (AUROC) curve with physician assessment of "active disease" plus re-initiation of standard dose of systemic therapy as the reference standard.</p>

<p><strong>RESULTS: </strong>Of the candidate definitions, clinically meaningful worsening in ≥3 of the following five 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. AUROC 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 the outcome measured one construct. "JSpAflare" had face validity according to 21 surveyed pediatric rheumatologists. JSpAflare had AUROC 0.85, PPV 92.3%, and NPV 96.8% in the validation cohort.</p>

<p><strong>CONCLUSIONS: </strong>There is initial support for the validity of JSpAflare as a tool to identify disease flare in juvenile spondyloarthritis patients de-escalating therapy and is potentially applicable in clinical practice, observational studies, and therapeutic trials.</p>

DOI

10.1002/acr.24763

Alternate Title

Arthritis Care Res (Hoboken)

PMID

34363343

Title

Child-onset systemic lupus erythematosus is associated with a higher incidence of myopericardial manifestations compared to adult-onset disease.

Year of Publication

2018

Number of Pages

2146-2154

Date Published

2018 Nov

ISSN Number

1477-0962

Abstract

<p>Objectives There are no population-based estimates of the incidence or risk factors for acute cardiac manifestations in children with systemic lupus erythematosus (SLE) to guide screening and diagnostic imaging practices. We estimated the incidence and prevalence of acute cardiac manifestations of child-onset SLE compared to adult-onset SLE and identified factors associated with cardiac diagnoses. Methods We identified children (5-17 years) and adults (18-64 years) with incident SLE (≥3 International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 CM) code 710.0, &gt; 30 days apart) using Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) deidentified United States administrative claims (2000-2013). We calculated incidence and prevalence of three outcomes: ≥ 1 diagnosis code for (1) pericarditis and/or myocarditis, (2) endocarditis, or (3) valvular insufficiency. Negative binomial regression was used to identify characteristics associated with cardiac diagnoses in children and determine whether SLE onset in childhood vs adulthood was independently associated with cardiac involvement. Results There were 297 children and 6927 adults with new-onset SLE. A total of 17.8% of children had ICD-9 CM codes for acute cardiac diagnoses, the incidence of which were highest in the first year after SLE diagnosis (12.2 per 100 person-years). African American race (incidence rate ratio (IRR) 6.6, 95% confidence interval (CI) (2.9, 15.0), p &lt; 0.01) and nephritis (IRR 7.0, 95% CI (2.6, 18.6), p &lt; 0.01) were associated with acute cardiac diagnoses in children. Child-onset disease was independently associated with a 4.4-fold higher rate of pericarditis or myocarditis compared to adult-onset SLE after adjustment for other disease and demographic characteristics (95% CI (2.4, 8.0), p &lt; 0.01). Conclusion This study establishes baseline estimates of the incidence and prevalence of pericarditis and myocarditis in child-onset SLE, which is substantially higher than that of adult-onset SLE. Prospective echocardiographic evaluations are needed to validate incidence measures and characterize the natural history of acute cardiac manifestations in child-onset SLE, as well as identify risk factors for poor cardiac outcomes to inform screening and management.</p>

DOI

10.1177/0961203318804889

Alternate Title

Lupus

PMID

30318995

Title

Use of echocardiography at diagnosis and detection of acute cardiac disease in youth with systemic lupus erythematosus.

Year of Publication

2018

Number of Pages

1348-1357

Date Published

2018 Jul

ISSN Number

1477-0962

Abstract

<p>Objectives There are no guidelines on the use of echocardiography to detect cardiac manifestations of childhood-onset systemic lupus erythematosus (SLE). We quantify the prevalence of acute cardiac disease in youth with SLE, describe echocardiogram utilization at SLE diagnosis, and compare regional echocardiogram use with incident cardiac diagnoses. Methods Using the Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) de-identified United States administrative database from 2000 to 2013, we identified youth ages 5-24 years with new-onset SLE (≥3 ICD-9 SLE codes 710.0, &gt; 30 days apart) and determined the prevalence of diagnostic codes for pericardial disease, myocarditis, endocarditis, and valvular insufficiency. Multiple logistic regression was used to identify factors associated with echocardiography during the baseline period, up to one year before or six months after SLE diagnosis. We calculated a regional echocardiogram utilization index, which is the ratio of observed use over the mean predicted probability based on all available baseline characteristics. Spearman's rank correlation coefficient was used to evaluate the association between regional echocardiogram utilization indices and percentage of imaged youth diagnosed with their first cardiac manifestation following echocardiography. Results Among 699 youth with new-onset SLE, 18% had ≥ 1 diagnosis code for acute cardiac disease, of which valvular insufficiency and pericarditis were most common. Twenty-five percent of all youth underwent echocardiogram during the baseline period. Regional echocardiogram use was positively correlated with the percentage of imaged youth found to have cardiac disease (ρ = 0.71, p = 0.05). There was up to a five-fold difference in adjusted odds of baseline echocardiography between low- and high-utilizing regions (OR = 0.19, p = 0.007). Conclusion Nearly one-fifth of youth with new-onset SLE have acute cardiac manifestations; however, use of echocardiograms at SLE diagnosis is highly variable. There may be incremental diagnostic value to early use of echocardiography, but prospective studies are needed to determine whether greater use of echocardiograms modifies outcomes.</p>

DOI

10.1177/0961203318772022

Alternate Title

Lupus

PMID

29688145

Title

Variation in treatment of children hospitalized with antineutrophil cytoplasmic antibody-associated vasculitis in the United States

Year of Publication

2016

Date Published

2016 Nov 3

Abstract

OBJECTIVE:

There are few reports on treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in children. This study characterizes the use of cyclophosphamide, rituximab, and plasma exchange in children hospitalized with AAV in the United States.

METHODS:

We conducted a retrospective cohort study of children hospitalized with AAV from 2004-2014 utilizing an administrative and billing database from 47 tertiary care pediatric hospitals. All patients had an ICD-9-CM discharge code of 446.4 and ≥1 charge for glucocorticoids. Treatment receipt was determined using billing data. Mixed effects logistic regression evaluated factors associated with the likelihood of receipt of each of the three treatments.

RESULTS:

During the 11 year study period there were 1290 admissions for 393 children. Median age at index admission was 14.6 years and 61% were female. Sixteen percent and 17% of children required dialysis or mechanical ventilation, respectively. The median length of stay was 9 days. Fifty-seven percent, 21%, and 10% of children received cyclophosphamide, rituximab, or both, respectively. Twenty-two percent received plasma exchange. Mechanical ventilation was associated with receipt of cyclophosphamide and plasma exchange, but not rituximab. There was an increasing trend in use of rituximab over time during the study period (p<0.05), and a decreasing trend in use of cyclophosphamide (p<0.05). Treatment use varied significantly between hospitals, especially for plasma exchange.

CONCLUSION:

The treatment of children with severe AAV is shifting from cyclophosphamide to rituximab and their need for dialysis, mechanical ventilation, and prolonged hospitalization remains common. Use of plasma exchange is highly variable. This article is protected by copyright. All rights reserved.

PMID

27813340

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