Elsevier

Autoimmunity Reviews

Volume 13, Issue 2, February 2014, Pages 157-162
Autoimmunity Reviews

Review
Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS Registry”

https://doi.org/10.1016/j.autrev.2013.10.004Get rights and content

Abstract

Given the lack of information about catastrophic antiphospholipid syndrome (APS) in pediatric patients, the objective of the current study was to describe the clinical characteristics, laboratory features, treatment, and outcome of pediatric patients with catastrophic APS and compare them with the adult patients with catastrophic APS.

We identified patients who were under 18 years of age at time of catastrophic APS diagnosis included in the international registry of patients with catastrophic APS (CAPS Registry). Their main demographic and clinical characteristics, laboratory features, treatment, and outcome were described and compared with those of adult patients with catastrophic APS.

From the 446 patients included in the CAPS Registry as of May 2013, 45 (10.3%) patients developed 46 catastrophic events before 18 years of age (one patient presented two episodes). Overall, 32 (71.1%) patients were female and the mean age was 11.5 ± 4.6 years (range, 3 months–18 years). A total of 31 (68.9%) patients suffered from primary APS and 13 (28.9%) from systemic lupus erythematosus (SLE). The main differences between the two groups of patients were the higher prevalence of infections as precipitating factor for catastrophic event in the pediatric population (60.9% versus 26.8% in the adult population, p < 0.001) and of peripheral vessel thrombosis (52.2% versus 34.3%, p = 0.017). In addition, catastrophic APS was the first manifestation of APS more frequently in pediatric patients (86.6% versus 45.2%, p < 0.001). Interestingly, pediatric patients showed a trend of lower mortality, although the difference was not statistically significant (26.1% versus 40.2%; odds ratio, 1.9; 95% confidence interval, 0.96–3.79; p = 0.063). No differences were found neither in the laboratory features nor in the isolated or combination treatments between groups.

Catastrophic APS in pediatric patients is a rare disease. There are minimal differences in the clinical and laboratory features, treatment, and outcome of pediatric and adult catastrophic APS patients.

Introduction

The catastrophic antiphospholipid syndrome (APS) is characterized by multi-organ thrombosis developing over a short period of time [1]. This life-threatening variant of the APS that represents less than 1% of all patients with APS, was first described more than 20 years ago [2]. Since then, more than 400 cases have been collected in the catastrophic APS international registry (CAPS Registry). This registry was created by the European Forum on Antiphospholipid Antibodies, a study group devoted to the development of multi-center projects with large populations of APS patients [3]. The analysis of this registry has generated a cluster of knowledge of the main clinical, and laboratory features as well as the outcome of patients with catastrophic APS [4]. In addition, classification criteria and treatment guidelines for its management have been established [1], [5], [6].

Age at onset might modulate the clinical expression and prognosis of patients with different autoimmune diseases [7]. Particularly for classic APS, a review of 121 patients with pediatric APS, defined as the onset of APS before the patient's 18th birthday, demonstrated some important differences between the pediatric and adult APS patients [8]. In fact, the high female preponderance in adult patients (female/male ratio of 5:1) with classic APS does not occur in the pediatric subset (1.2:1). Deep venous thrombosis in the legs was the most frequent venous thrombotic event both in pediatric and adult APS patients (40% versus 32%, respectively). However, cerebral sinus venous thrombosis and ischemic stroke as a first manifestation of APS were significantly more frequent in the pediatric APS patients. In addition, the presence of lupus anticoagulant (LAC) was also more frequent in patients with pediatric APS [8].

In the present article, we describe the clinical characteristics, laboratory features, and outcomes of patients who presented with catastrophic APS in the pediatric age and compared them with those of adult patients with catastrophic APS.

Section snippets

Data collection

We identified the patients with catastrophic APS in the pediatric age included in the CAPS Registry, the website-based international registry of patients with catastrophic APS. The sources of information for the CAPS Registry are: a) periodic Medline search of published catastrophic APS reports; and b) personal communication with physicians in charge of catastrophic APS patients for the unpublished cases (physicians fill out a standardized data form collecting demographic, clinical,

General characteristics

From the 446 patients included in the CAPS Registry since May 2013, 45 (10.3%) patients developed 46 catastrophic events before 18 years of age (one patient presented two episodes). Overall, 32 (71.1%) patients were female and the mean age was 11.5 ± 4.6 years (range, 3 months–18 years) (Table 1). A total of 31 (68.9%) patients suffered from primary APS, 13 (28.9%) from systemic lupus erythematosus (SLE) and one from a lupus-like disease (2.2%).

Precipitating factors and clinical presentation

Precipitating factors were identified in 35 (76.1%)

Discussion

In the current study we have demonstrated that the clinical, laboratory and treatment features between the pediatric and adult patients with catastrophic APS were generally similar. The main differences between the two groups of patients were the higher prevalence of infections as precipitating factor for catastrophic event and of peripheral vessel thrombosis in the pediatric population, and the fact that catastrophic APS was the first manifestation of APS in a higher number of pediatric

Conclusion

The current study is the first description of pediatric patients with catastrophic APS, corresponding to 10% of patients included in the CAPS Registry. The clinical and laboratory features, and treatment between catastrophic APS in pediatric patients and those cases in adults were similar. Catastrophic APS was the first manifestation of APS in a higher number of pediatric patients. In addition, infections as precipitating factor and peripheral vessel thrombosis were more frequent in the

Take-home messages

  • There is no information about catastrophic antiphospholipid syndrome of patients in the pediatric age.

  • The current study is the first description of pediatric patients with catastrophic APS, corresponding to 10% of patients included in the CAPS Registry.

  • There were minimal differences in the clinical and laboratory features, and treatment between catastrophic APS in pediatric patients and those cases in adults.

References (14)

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1

These two authors contributed equally to this study.

2

See complete list of members at the appendix at the end of article.

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