Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood
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 diagn...
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doaj-1ff5074fe64c403bbdcc08312e6eef152020-11-24T21:07:35ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602018-05-01610.3389/fped.2018.00144377029Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to AdulthoodGiorgio Costagliola0Marta Mosca1Paola Migliorini2Rita Consolini3Laboratory of Immunology, Division of Pediatrics, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, ItalyRheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, ItalyClinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, ItalyLaboratory of Immunology, Division of Pediatrics, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, ItalyBackground: 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.http://journal.frontiersin.org/article/10.3389/fped.2018.00144/fullsystemic lupus erythematosuschildrendisease activityorgan damagecorticosteroidsthrombocytopenia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Giorgio Costagliola Marta Mosca Paola Migliorini Rita Consolini |
spellingShingle |
Giorgio Costagliola Marta Mosca Paola Migliorini Rita Consolini Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood Frontiers in Pediatrics systemic lupus erythematosus children disease activity organ damage corticosteroids thrombocytopenia |
author_facet |
Giorgio Costagliola Marta Mosca Paola Migliorini Rita Consolini |
author_sort |
Giorgio Costagliola |
title |
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood |
title_short |
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood |
title_full |
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood |
title_fullStr |
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood |
title_full_unstemmed |
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood |
title_sort |
pediatric systemic lupus erythematosus: learning from longer follow up to adulthood |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Pediatrics |
issn |
2296-2360 |
publishDate |
2018-05-01 |
description |
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. |
topic |
systemic lupus erythematosus children disease activity organ damage corticosteroids thrombocytopenia |
url |
http://journal.frontiersin.org/article/10.3389/fped.2018.00144/full |
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