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Risk factors associated with the death of patients hospitalized for juvenile systemic lupus erythematosus
M M M Faco1, C Leone, L M A Campos
1Unidades de Reumatologia e Infectologia Pediátricas, Instituto da Criança, Departamento de Pediatria, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Insights
Severe sepsis significantly increases mortality risk in hospitalized juvenile systemic lupus erythematosus (JSLE) patients. Autopsies are crucial for accurate diagnosis, revealing discrepancies in 60% of cases.
Area of Science:
- Pediatric Rheumatology
- Critical Care Medicine
- Pathology
Background:
- Juvenile Systemic Lupus Erythematosus (JSLE) is a chronic autoimmune disease with significant morbidity and mortality.
- Hospitalization for JSLE carries inherent risks, necessitating identification of key factors contributing to adverse outcomes.
Purpose of the Study:
- To identify risk factors associated with mortality in hospitalized JSLE patients.
- To evaluate the role of autopsy in determining causes of death in JSLE.
Main Methods:
- Retrospective analysis of 71 JSLE patients with 169 hospitalizations between 1994-2003.
- Comparison of clinical data and autopsy reports for patients who died during hospitalization.
- Univariate, stratified, and multivariate logistic regression analyses to identify independent predictors of mortality.
Main Results:
- The primary causes of hospitalization were JSLE activity with infection (52%) and isolated JSLE activity (44%).
- Severe sepsis emerged as the strongest independent predictor of death (OR = 98), followed by high SLEDAI scores.
- Autopsy revealed diagnostic discrepancies in 6 out of 10 cases, highlighting its importance.
Conclusions:
- Severe sepsis is a critical determinant of mortality in hospitalized JSLE patients.
- Autopsy plays a vital role in clarifying the cause of death and improving diagnostic accuracy in JSLE mortality cases.
Abstract:
We assessed the risk factors associated with death in patients hospitalized for juvenile systemic lupus erythematosus (JSLE) and evaluated the autopsy reports. A total of 57,159 hospitalizations occurred in our institution from 1994 to 2003, 169 of them involving 71 patients with JSLE. The most recent hospitalization of these patients was evaluated. Patients were divided into two groups based on mortality during hospitalization: those who survived (N = 53) and those who died (N = 18). The main causes of hospitalization were JSLE activity associated with infection in 52% and isolated JSLE activity in 44%. Univariate analysis showed that a greater risk of death was due to severe sepsis (OR = 17.8, CI = 4.5-70.9), systemic lupus erythematosus disease activity index (SLEDAI) >or=8 (OR = 7.6, CI = 1.1-53.8), general infections (OR = 6.1, CI = 1.5-25), fungal infections (OR = 5.4, CI = 3.2-9), acute renal failure (OR = 5.1, CI = 2.5-10.4), acute thrombocytopenia (OR = 3.9, CI = 1.9-8.4), and bacterial infections (OR = 2.3, CI = 1.2-7.5). Stratified analysis showed that severe sepsis and SLEDAI >or=8 were not confounder variables. In the multivariate analysis, logistic regression showed that the only independent variable in death prediction was severe sepsis (OR = 98, CI = 16.3-586.2). Discordance between clinical diagnosis and autopsy was observed in 6/10 cases. Mortality of hospitalized JSLE patients was associated with severe sepsis. Autopsy was important to determine events not detected or doubtful in dead patients and should always be requested.
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