Outcomes in juvenile onset lupus: single center cohort from a developing country

Aggarwal, Amita; Phatak, Sanat; Srivastava, Puja; Lawrence, Able; Agarwal, Vikas; Misra, Ramnath

doi:10.1177/0961203318791046

Cited by 31 publications

(36 citation statements)

References 43 publications

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“…Our finding that 39% of youth who died had infectious diseases as their primary diagnosis demonstrates once again that infection is a major complication of the rheumatic diseases. The rate of infection we found was higher than reported by Butt et al in a Danish cohort with systemic sclerosis [37] and by Aggarwal et al in an Indian cohort of youth with lupus [38] but similar to the Chinese lupus cohort of all ages by Mu et al [39] We also found that patients with vasculitis and scleroderma were at higher risk of dying in the hospital relative to patients with other rheumatic diseases (of which lupus was the most frequent comparator diagnosis). While this can be explained by the increased severity of these diseases, they are understudied in the transition literature [40,41].…”

Section: Discussionsupporting

confidence: 74%

Inpatient mortality in transition-aged youth with rheumatic disease: an analysis of the National Inpatient Sample

Jensen¹,

Koh²,

Cash³

et al. 2020

Pediatr Rheumatol

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Background: Transition from pediatric to adult care is a vulnerable time for youth with chronic diseases. In youth with rheumatic disease, studies show high rates of loss to follow up and increased disease activity. However, mortality data are lacking. In this study, we assessed whether transitional age is a risk factor for inpatient mortality. Methods: We analyzed the 2012-2014 National Inpatient Sample database, a representative sample of discharges in the United States. Individuals with rheumatic diseases were identified by International Statistical Classification of Disease-9 (ICD-9) codes at time of discharge. Youth were categorized into three age groups: pre-transitional (11-17), transitional (18-24) and post transitional (25-31). We fitted univariable and multivariable logistic regression models to assess whether transitional age was a risk factor for inpatient mortality. Results: There were 30,269 hospital discharges which met our inclusion criteria of diagnosis and age. There were 195 inpatient deaths (0.7%). The most common causes of death were infection (39.5%), pulmonary disease (13.8%), and cardiac disease (11.2%). The Odds ratio for inpatient mortality of a transitional-aged individual was 1.18 compared to controls (p = 0.3). Black race (OR = 1.4), male sex (OR = 1.75), and a diagnosis of systemic sclerosis (OR = 4.81) or vasculitis (OR = 2.85) were the greatest risk factors of inpatient mortality. Conclusion: Transitional age was not a risk factor for inpatient mortality in this study. We did identify other risk factors other than age. Further studies are required to assess if there is an increased risk of mortality in outpatients of the transitional age group.

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Section: Discussionsupporting

confidence: 74%

Inpatient mortality in transition-aged youth with rheumatic disease: an analysis of the National Inpatient Sample

Jensen¹,

Koh²,

Cash³

et al. 2020

Pediatr Rheumatol

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“…There is also a markedly increased risk of cardiovascular disease in adulthood [ 84 , 85 ]. Ten-year survival for JSLE in LRIC and MRIC is approximately 80% [ 2 , 80 , [86] , [87] , [88] , [89] , [90] ] compared with 99% in HRIC [ 85 ], with lupus nephritis and infection, the major causes of death [ 91 , 92 ].…”

Section: Impact Described Through Selected Msk Conditionsmentioning

confidence: 99%

“…Diagnosis can be challenging where infections (such as TB) are often mimics of rheumatic diseases and diagnostic capabilities (including availability of autoantibody tests) are often limited in low resource settings [ 5 , 95 ]. Delayed diagnosis and access to specialist care in JSLE with worse clinical outcomes is widely reported across the world [ 79 , 88 , [96] , [97] , [98] ], often associated with more severe disease activity and organ damage at presentation [ 75 , 99 , 100 ]. Many of the innovations that have improved survival in HRIC remain unavailable in most of the world [ 77 ] and access to renal replacement therapy may be severely limited or non-existent [ 101 ].…”

Section: Impact Described Through Selected Msk Conditionsmentioning

confidence: 99%

Improving musculoskeletal health for children and young people – A ‘call to action’

Foster¹,

Scott

Tiderius

et al. 2020

Best Practice & Research Clinical Rheumatology

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This chapter describes the musculoskeletal (MSK) context in children and young people as an important contributor to the global non-communicable disease burden. Through selected MSK conditions, we describe the impact on patients, families and communities and highlight the challenges that need to be addressed. We focus on opportunities for better working together and describe exemplar initiatives to raise awareness, workforce capacity building, models of care and research agendas to have a greater global context.

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“…Advancements of immunosuppressive therapy in systemic lupus erythematosus (SLE) improve patient survival. 1 However, the use of potent immunosuppressive medications is associated with increased rates of infection-related morbidity and mortality. 2 Infection contributes to 30% of deaths in the first 5 years of disease and infection increases standardized mortality ratio in patients with SLE five-fold.…”

Section: Introductionmentioning

confidence: 99%

“…Children account for 10–20% of patients with SLE and 50–70% of them develop lupus nephritis (LN) with class IV the most common. 1 Infection is the leading cause of death in childhood-onset SLE as children often require intense immunosuppression to control the disease. 4 A recent study has shown that severe sepsis is the only independent risk factor of death in childhood-onset SLE and associated with nearly a 100-fold increase in the risk of death.…”

Section: Introductionmentioning

confidence: 99%

Risk factors of infection-associated mortality in children with lupus nephritis in under-resourced areas

Rianthavorn

Prurapark

2019

Lupus

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Introduction Treatment of lupus nephritis class III, IV and V with immunosuppressive therapy increases patient survival but poses a risk of infection-related mortality. This study was conducted to evaluate risk factors for fatal infection in children with lupus nephritis in under-resourced areas. Methods Medical records of patients, who were admitted to a tertiary-care university-based hospital from January 2002 to July 2018 with the diagnosis of systemic lupus erythematosus, were reviewed. Only patients aged less than 18 years with lupus nephritis and documented infection were included in the study. The primary outcome was infection-associated mortality. The logistic regression model was used to identify independent variables associated with fatal infection. Predicted probabilities of infection-related mortality adjusted for factors significant in multivariate analysis were calculated using marginal effects at representative values. Results Infection-related deaths occurred in 27 of 179 patients (15.1%). Hospital-acquired infections occurred in 72 of 375 episodes of hospital admissions (19.2%) and 13 hospital-acquired infections (18.1%) resulted in fatal infection. Invasive fungal infections were the leading cause of death (44.4%) and pulmonary infections were the predominant site (55.5%). Haemoglobin levels and glomerular filtration rates were significantly lower in deceased versus surviving patients. Percentages of patients with hospital-acquired infections, invasive fungal infections and pulmonary infections were significantly higher in deceased than surviving patients. Urine protein, the neutrophil-to-lymphocyte ratio, cumulative methylprednisolone dose and daily prednisolone dose were significantly higher in deceased than surviving patients. In multivariate analysis, a neutrophil-to-lymphocyte ratio more than 20, invasive fungal infections and high daily prednisolone dose were independently predictive of fatal infection with adjusted odds ratio of 3.02 (95% confidence interval 1.02–8.97, p = 0.04), 15.08 (95% confidence interval 4.72–48.24, p < 0.001) and 1.03 (95% confidence interval 1.01–1.06, p = 0.04), respectively. A high daily prednisolone dose intensified the impact of invasive fungal infections and high neutrophil-to-lymphocyte ratio on predicted probability of infection-associated mortality. Conclusions Prevention of invasive fungal infections and minimization of daily prednisolone should be emphasized in routine clinical practice of children with lupus nephritis in under-resourced areas to achieve better survival. Children with lupus nephritis and a high neutrophil-to-lymphocyte ratio should be under cautious surveillance for infection.

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Outcomes in juvenile onset lupus: single center cohort from a developing country

Cited by 31 publications

References 43 publications

Inpatient mortality in transition-aged youth with rheumatic disease: an analysis of the National Inpatient Sample

Inpatient mortality in transition-aged youth with rheumatic disease: an analysis of the National Inpatient Sample

Improving musculoskeletal health for children and young people – A ‘call to action’

Risk factors of infection-associated mortality in children with lupus nephritis in under-resourced areas

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