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Outcome of primary antiphospholipid syndrome in childhood
M Gattorno1, F Falcini, A Ravelli
1Dipartimento di Pediatria, Università di Genova, Pediatria II, Istituto G. Gaslini, Italy. marcogattorno@ospedale-gaslini.ge.it
Insights
Pediatric primary antiphospholipid syndrome (APS) can evolve. Some children diagnosed with APS may later develop systemic lupus erythematosus (SLE) or lupus-like syndromes, highlighting the need for long-term monitoring.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Thrombosis
Background:
- Primary antiphospholipid syndrome (APS) in children is rare.
- Long-term outcomes and potential evolution of pediatric APS are not well-established.
Purpose of the Study:
- To investigate the long-term outcomes of primary APS in pediatric patients.
- To determine the rate of progression to systemic lupus erythematosus (SLE) or lupus-like syndromes.
Main Methods:
- Retrospective analysis of unselected pediatric patients with primary APS onset before age 16.
- Review of clinical and laboratory manifestations over a median follow-up of six years.
- Assessment of diagnostic evolution from primary APS to SLE or lupus-like syndrome.
Main Results:
- Fourteen pediatric patients (median age at onset 9 years) were analyzed.
- Vascular events included deep vein thrombosis and cerebral stroke.
- At follow-up, 10 patients remained primary APS, two developed SLE, and one developed a lupus-like syndrome.
Conclusions:
- Pediatric primary APS can progress to SLE or lupus-like syndromes.
- Long-term surveillance is crucial for children diagnosed with primary APS.
- Understanding the evolution of pediatric APS informs clinical management and prognosis.
Abstract:
The objective of this paper is to investigate the long-term outcome of primary antiphospholipid syndrome (APS) in the paediatric age. The features of unselected patients with primary APS who had disease onset before the age of 16 years were retrospectively analysed in three Italian referralcentres. Clinical and laboratory manifestations were assessed to establish whether, at the end of follow-up, the final diagnosis was still primary APS or whether they had developed definite SLE or lupus-like syndrome. Fourteen patients, nine boys and five girls, who had the presenting clinical manifestation of APS between three and 13 years of age (median nine years) and were followed for two to 16 years (median six years). Six patients presented with deep vein thrombosis, five with cerebral stroke, two with peripheral artery occlusion and onewith myocardial infarction. During follow-up, four patients had one or more recurrences of vascular thrombosis. At last observation, 10 patients could still be classified as having primary APS, two had developed SLE, one lupus-like syndrome and one Hodgkin's lymphoma. In conclusion; our analysis suggests that some children who present with the features of primary APS may progress to develop SLE or lupus-like syndrome.