Abstract
Background: Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to improve the diagnostic awareness and management of pSLE patients.Methods: This retrospective study included 25 patients diagnosed with pSLE and followed at the University of Pisa. We collected data about clinical profile at disease onset and during a long-term follow-up, including disease activity, organ damage development, and treatments received.Results: The mean patient age at disease onset was 14.6 ± 1.6 years, and the mean follow-up period was 14.17 ± 8.04 years. The most common initial manifestations were arthritis, malar rash, and cytopenias. The median time to diagnosis since the first symptoms was 6 months, and was significantly longer in patients with hematological onset (54 months). During follow-up, the number of patients with renal involvement showed a significant increase, from 36% at diagnosis to 72.2% after 10 years of disease evolution. Patients who developed chronic organ damage maintained a higher time-averaged disease activity during follow-up and received a significantly higher dose of corticosteroids.Conclusion: Patients with immune cytopenia represent a group deserving strict clinical follow-up for the risk of evolution to SLE. Intense surveillance of renal function, early treatment and steroid-sparing strategies should be strongly considered in the management of pSLE patients.
Highlights
Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic involvement and a chronic-relapsing course.According to the definition proposed by Silva et al, pediatric systemic lupus erythematosus identifies a subset of patients with disease onset of SLE prior to the 18th birthday [1], as this threshold reflects differences in clinical expression, disease activity and gender distribution.The mean age at the onset of SLE symptoms is typically between 20 and 40 years, and pSLE is a rare condition, representing approximately 10% of SLE cases
The aim of the present study is to identify variables that may improve the diagnostic sensibility and management of pSLE patients, by the analysis of the most common patterns observed at disease onset, the manifestations occurring during the clinical course, the factors associated with the development of organ damage and the effects of treatment received
We investigated the role of the overall disease activity and of the use of corticosteroids in the development of organ damage: the population of patients with at least 10 years of follow-up was divided according to the presence of organ damage (“damage group” and “non-damage group”), and time-averaged SLEDAI scores and mean annual dose of prednisone were compared between the two groups of patients
Summary
Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic involvement and a chronic-relapsing course.According to the definition proposed by Silva et al, pediatric systemic lupus erythematosus (pSLE) identifies a subset of patients with disease onset of SLE prior to the 18th birthday [1], as this threshold reflects differences in clinical expression, disease activity and gender distribution.The mean age at the onset of SLE symptoms is typically between 20 and 40 years, and pSLE is a rare condition, representing approximately 10% of SLE cases. Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystemic involvement and a chronic-relapsing course. According to the definition proposed by Silva et al, pediatric systemic lupus erythematosus (pSLE) identifies a subset of patients with disease onset of SLE prior to the 18th birthday [1], as this threshold reflects differences in clinical expression, disease activity and gender distribution. Concerning the clinical phenotype, most studies have reported substantial differences between pSLE and adult-onset disease, showing a higher frequency of renal, hematological and neuropsychiatric involvement in pediatric patients, while chronic cutaneous lupus is more commonly diagnosed in patients with aSLE. Patients with pSLE have higher disease activity compared to aSLE patients and frequently develop more organ damage, showing worse outcomes [11, 12]. Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to improve the diagnostic awareness and management of pSLE patients
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