Childhood‐onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus

Hersh, Aimee O.; Trupin, Laura; Yazdany, Jinoos; Panopalis, Peter; Julián, Laura; Katz, Patricia P.; Criswell, Lindsey A.; Yelin, Edward H.

doi:10.1002/acr.20179

Cited by 141 publications

(127 citation statements)

References 29 publications

(41 reference statements)

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“…Mortality rates among young people with SLE remain alarmingly high, however, with a 20-fold mortality risk among adolescents with lupus compared to the general population, and significantly higher than the 2.4-fold mortality risk among lupus patients of all ages (15,19,21). The mortality rate in our cohort was lower, at 4% over a mean followup period of 5 years.…”

Section: Discussioncontrasting

confidence: 54%

“…As a result of significant medical advances over the past decades, the vast majority of children with chronic diseases, including pediatric-onset systemic lupus erythematosus (SLE), are now surviving well into adulthood (15). Thus, transition of care has become an increasingly relevant issue for both patients and health care providers.…”

Section: Introductionmentioning

confidence: 99%

“…In adult cohorts, patients with SLE have been found to face a 2-to 3-fold increase in mortality if their disease onset occurred during childhood (15,19), and mortality rates in pediatric-onset SLE are known to peak among young adults (21). Continued damage accrual through adulthood results in chronic medical problems (18,22), inferior academic outcomes (23), and disease-related absence from work (24) and may contribute to the comparatively higher unemployment rates among adult patients with pediatric-onset SLE (25).…”

Section: Introductionmentioning

confidence: 99%

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Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

Felsenstein

Reiff

Ramanathan

2015

Arthritis Care & Research

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Objective. Transition from pediatric to adult care is a complex process and can negatively impact patients with chronic disease. We describe the transition experience of patients with pediatric-onset systemic lupus erythematosus (SLE) and its associated outcomes in adulthood. Methods. A telephone survey of 41 pediatric-onset SLE patients was conducted following their transition to adult care. Data on medical and social outcomes during and after the transition were collected. Health status was compared to retrospectively collected baseline data at pediatric discharge.Results. The mean 6 SD followup interval was 5 6 3.7 years; the mean 6 SD age at followup was 24 6 4.2 years. More than half of patients (22 of 41) experienced transition difficulties, primarily due to loss of insurance and emotional readjustment, which were associated with poor symptom control (P 5 0.03) and multiple organ system involvement (P 5 0.05) at followup. After the transition, most patients (35 of 41) were followed by an adult-care rheumatologist, and the majority (37 of 41) reported recent symptoms of active disease; 41% (13 of 29) had developed symptoms suggestive of new renal manifestations following transition. One-third (15 of 41) reported new or ongoing neuropsychiatric symptoms. Both renal and neuropsychiatric manifestations were associated with unemployment (P < 0.05). Direct referral by a pediatric rheumatologist was associated with fewer hospitalizations following transition (P 5 0.04). Conclusion. The majority of patients transitioned successfully to adult rheumatologic care. Major challenges were loss of insurance and attachment to pediatric providers, highlighting the importance of a structured transition process that focuses on providing emotional and financial guidance. Disease activity in pediatric-onset SLE remains high throughout adulthood, with morbidity primarily related to renal and neuropsychiatric manifestations.

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Section: Discussioncontrasting

confidence: 54%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

Felsenstein

Reiff

Ramanathan

2015

Arthritis Care & Research

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“…Children with SLE tend to have more severe disease at presentation and more disease activity over time compared with their adult counterparts. [1][2][3][4] Hersh et al 5 reported that childhood onset of SLE is a predictor of mortality among adult patients with SLE.…”

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confidence: 99%

Outcomes in Hospitalized Pediatric Patients With Systemic Lupus Erythematosus

Son¹,

Johnson

Hersh

et al. 2014

Pediatrics

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OBJECTIVE: Disparities in outcomes among adults with systemic lupus erythematosus (SLE) have been documented. We investigated associations between sociodemographic factors and volume of annual inpatient hospital admissions with hospitalization characteristics and poor outcomes among patients with childhood-onset SLE. METHODS: By using the Pediatric Health Information System, we analyzed admissions for patients aged 3 to <18 years at index admission with ≥1 International Classification of Diseases, Ninth Revision code for SLE from January 2006 to September 2011. Summary statistics and univariable analyses were used to examine demographic characteristics of hospital admissions, readmissions, and lengths of stay. We used multivariable logistic regression analyses, controlling for patient gender, age, race, ethnicity, insurance type, hospital volume, US census region, and severity of illness, to examine risk factors for poor outcomes. RESULTS: A total of 10 724 admissions occurred among 2775 patients over the study period. Hispanic patients had longer lengths of stay, more readmissions, and higher in-hospital mortality. In multivariable analysis, African American race was significantly associated with ICU admission. African American race and Hispanic ethnicity were associated with end-stage renal disease and death. Volume of patients with SLE per hospital and hospital location were not significantly associated with outcomes. CONCLUSIONS: In this cohort of hospitalized children with SLE, race and ethnicity were associated with outcomes. Further studies are needed to elucidate the relationship between sociodemographic factors and poor outcomes in patients with childhood-onset SLE.

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“…Juvenile SLE (JSLE) is characterized by an exaggerated immune response with highly activated autoreactive B lymphocytes, dysregulated immune regulatory pathways, and frequent involvement of the renal, hematological and central nervous systems (3)(4)(5). Children with JSLE also have a higher disease severity and a significantly higher mortality risk compared to patients with adult onset disease (6,7). Timely diagnosis and treatment have been shown to significantly improve the prognosis and the clinical management of JSLE (8).…”

mentioning

confidence: 99%

Clinical applications of the indirect immunofluorescence assay for detection of anticell membrane-associated DNA antibodies in juvenile systemic lupus erythematosus

Zhao

Xie

et al. 2014

Pediatr Res

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Background: Juvenile-onset systemic lupus erythematosus (JSLE) has a higher mortality risk compared to adultonset SLE. We compared the diagnostic value of anti-cmDNA antibodies with that of antinucleosome antibodies (AnuA), anti-Sm antibodies, and anti-dsDNA antibodies and human B lymphocyte Raji cells with that of human promyelocytic leukemia HL60 cells as substrates in an indirect immunofluorescence assay to detect anti-cmDNA antibodies in JSLE patients. Methods: We recruited 92 JSLE patients and 71 patients with other juvenile-onset rheumatic diseases. Anti-cmDNA antibodies and antinuclear antibodies (ANA) were detected in patient sera using indirect immunofluorescence assays. Anti-dsDNA antibodies were detected by combining ELISA and indirect immunofluorescence. Anti-Sm antibodies were detected by double immunodiffusion assay and immunoblotting, while AnuA were detected by ELISA. results: The JSLE group had a significantly higher percentage of patients positive for anti-cmDNA compared to patients with other rheumatoid diseases. Using one antibody for diagnosis, anti-cm DNA antibodies had the highest accuracy at 84.0%; using two antibodies, the combination of anti-cm DNA and anti-dsDNA antibodies had 90.8% accuracy. Raji cells used as substrate demonstrated a stronger intensity of fluorescent patterns compared to HL60 cells. conclusion: The high sensitivity, specificity, and accuracy of detection of anti-cmDNA antibodies make it a valuable diagnostic tool for JSLE. s ystemic lupus erythematosus (SLE) is an autoimmune disease characterized by the presence of autoimmune antibodies against a number of nuclear and nonnuclear antigens, resulting in immune complex-mediated inflammation and systemic, multiple organ failure (1). Almost 15-20% of all SLE patients are below the age of 18 (ref. 2). Juvenile SLE (JSLE) is characterized by an exaggerated immune response with highly activated autoreactive B lymphocytes, dysregulated immune regulatory pathways, and frequent involvement of the renal, hematological and central nervous systems (3)(4)(5). Children with JSLE also have a higher disease severity and a significantly higher mortality risk compared to patients with adult onset disease (6,7). Timely diagnosis and treatment have been shown to significantly improve the prognosis and the clinical management of JSLE (8).Disease activity in SLE patients is currently evaluated using the SLE activity index (SLEDAI), the systemic lupus activity measure, and the British Isles Lupus Assessment Group (9). Recently, the anti-Smith (Sm) antibodies, anticell membrane DNA (cmDNA) antibodies, antidouble strand DNA (dsDNA) antibodies, antinuclear antigen (ANA) antibodies, antinucleosome (AnuA) antibodies, anti-C1q antibodies, and antiphospholipid antibodies have been proposed as useful biomarkers of JSLE (10-16). Anti-dsDNA antibodies, anti-Sm antibodies and antiphospholipid antibodies are included in the diagnostic criteria outlined by the American College of Rheumatology (10). JSLE patients were shown to be more frequently positive ...

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Childhood‐onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus

Cited by 141 publications

References 29 publications

Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

Transition of Care and Health‐Related Outcomes in Pediatric‐Onset Systemic Lupus Erythematosus

Outcomes in Hospitalized Pediatric Patients With Systemic Lupus Erythematosus

Clinical applications of the indirect immunofluorescence assay for detection of anticell membrane-associated DNA antibodies in juvenile systemic lupus erythematosus

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