Erica F. Lawson

Self-management skills in adolescents with chronic rheumatic disease: A cross-sectional survey

BackgroundFor adolescents with a diagnosis of lifelong chronic illness, mastery of self-management skills is a critical component of the transition to adult care. This study aims to examine self-reported medication adherence and self-care skills among adolescents with chronic rheumatic disease.MethodsCross-sectional survey of 52 adolescent patients in the Pediatric Rheumatology Clinic at UCSF. Outcome measures were self-reported medication adherence, medication regimen knowledge and independence in health care tasks. Predictors of self-management included age, disease perception, self-care agency, demographics and self-reported health status. Bivariate associations were assessed using the Students t-test, Wilcoxon rank sum test and Fisher exact test as appropriate. Independence in self-management tasks were compared between subjects age 13-16 and 17-20 using the chi-squared test.ResultsSubjects were age 13-20 years (mean 15.9); 79% were female. Diagnoses included juvenile idiopathic arthritis (44%), lupus (35%), and other rheumatic conditions (21%). Mean disease duration was 5.3 years (SD 4.0). Fifty four percent reported perfect adherence to medications, 40% reported 1-2 missed doses per week, and 6% reported missing 3 or more doses. The most common reason for missing medications was forgetfulness. Among health care tasks, there was an age-related increase in ability to fill prescriptions, schedule appointments, arrange transportation, ask questions of doctors, manage insurance, and recognize symptoms of illness. Ability to take medications as directed, keep a calendar of appointments, and maintain a personal medical file did not improve with age.ConclusionsThis study suggests that adolescents with chronic rheumatic disease may need additional support to achieve independence in self-management.

Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.

Objective. Granulomatosis with polyangiitis (Wegener’s; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. Methods. Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). Results. MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. Conclusion. EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified.

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

Perception of transition readiness and preferences for use of technology in transition programs: teens' ideas for the future.

Abstract Background: Efforts to facilitate transition of care to adult providers for adolescents with chronic disease are not uniformly successful and many patients encounter challenges. The goal of this study was to assess transition readiness and preferences for tools to aid in the transition process with an emphasis on technology and social media. Methods: We surveyed and performed focus groups on patients aged 13–21 years from a pediatric university-based rheumatology and general pediatric practice. Demographics and transition readiness were assessed using a questionnaire. Transition readiness was assessed by examining patient knowledge and independence with care. Focus groups were conducted to elicit perspectives about desirable features of a transition program and useful tools. Results: Thirty-five patients completed surveys; and 20 patients and 13 of their parents participated in a focus group. The median patient age was 17 years and 74.3% were female. A Likert scale (0–10, 10=most) was used to evaluate concern over changing to an adult medical provider, (mean=6.4, SD=2.6), preparedness for disease self-management (mean=6.0, SD=2.8), and perceived importance of self-managing their condition (mean=7.1, SD=3.1). Themes that emerged from focus groups included a desire for support groups with other teens, a preference for using text messaging for communication and a desire for an online health management program. Conclusions: Teens with chronic disease are able to identify health maintenance tasks and strategies that will aid in developing independence with healthcare management. These findings support the idea that developing engaging applications and support groups will assist teens in the transitioning.

Comparing presenting clinical features of 48 children with microscopic polyangiitis (MPA) against 183 having granulomatosis with polyangiitis (GPA). An ARChiVe study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

Assessing the performance of the Birmingham vasculitis activity score at diagnosis for Children with antineutrophil cytoplasmic antibody-associated vasculitis in a registry for childhood vasculitis (ARChiVe)

Objective. There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician’s global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods. Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman’s rank correlation coefficient (rs) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. Results. A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0–40). The BVAS v.3 correlations were rs = 0.379 (95% CI 0.233 to 0.509) with PGA, rs = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and rs = 0.403 (95% CI 0.253 to 0.533) with ESR. Conclusion. Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.

Reasons for failure to receive pneumococcal and influenza vaccinations among immunosuppressed patients with systemic lupus erythematosus

OBJECTIVE To better understand why immunosuppressed individuals with systemic lupus erythematosus (SLE) fail to receive influenza and pneumococcal vaccines. METHODS These cross-sectional data were derived from the 2009 cycle of the Lupus Outcomes Study (LOS), an annual longitudinal telephone survey of individuals with confirmed SLE. Respondents were included in the analysis if they had taken immunosuppressive medications in the past year. We assessed any prior receipt of pneumococcal vaccine and influenza vaccine in the past year, and then elicited reasons for not receiving vaccination. We used bivariate statistics and multivariate logistic regression to assess frequency and predictors of reported reasons for not obtaining influenza or pneumococcal vaccines. RESULTS Among 508 respondents who received immunosuppressants, 485 reported whether they had received vaccines. Among the 175 respondents who did not receive an influenza vaccine, the most common reason was lack of doctor recommendation (55%), followed by efficacy or safety concerns (21%), and lack of time (19%). Reasons for not receiving pneumococcal vaccine (N = 159) were similar: lack of recommendation (87%), lack of time (7%), and efficacy or safety concerns (4%). Younger, less-educated, non-white patients with shorter disease duration, as well as those immunosuppressed with steroids alone, were at the greatest risk for not receiving indicated vaccine recommendations. CONCLUSIONS The most common reason why individuals with SLE did not receive pneumococcal and influenza vaccines was that physicians failed to recommend them. Data suggest that increasing vaccination rates in SLE will require improved process quality at the provider level, as well as addressing patient concerns and barriers.

Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? An analysis of ARChiVe study treatment decisions.

Objective. To determine whether adult disease severity subclassification systems for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are concordant with the decision to treat pediatric patients with cyclophosphamide (CYC). Methods. We applied the European Vasculitis Study (EUVAS) and Wegener’s Granulomatosis Etanercept Trial (WGET) disease severity subclassification systems to pediatric patients with AAV in A Registry for Childhood Vasculitis (ARChiVe). Modifications were made to the EUVAS and WGET systems to enable their application to this cohort of children. Treatment was categorized into 2 groups, “cyclophosphamide” and “no cyclophosphamide.” Pearson’s chi-square and Kendall’s rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis. Results. In total, 125 children with AAV were studied. Severity subgroup was associated with treatment group in both the EUVAS (chi-square 45.14, p < 0.001, Kendall’s tau-b 0.601, p < 0.001) and WGET (chi-square 59.33, p < 0.001, Kendall’s tau-b 0.689, p < 0.001) systems; however, 7 children classified by both systems as having less severe disease received CYC, and 6 children classified as having severe disease by both systems did not receive CYC. Conclusion. In this pediatric AAV cohort, the EUVAS and WGET adult severity subclassification systems had strong correlation with physician choice of treatment. However, a proportion of patients received treatment that was not concordant with their assigned severity subclass.

A Population‐Based Study of Infection‐Related Hospital Mortality in Patients With Dermatomyositis/Polymyositis

Dermatomyositis (DM) and polymyositis (PM) are debilitating inflammatory myopathies associated with significant mortality. We evaluated the relative contribution of infection to hospital mortality in a large population‐based study of individuals with PM/DM.

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

To characterize the early disease course in childhood‐onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12‐month outcomes in children with AAV.