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Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome

Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women’s Medical University, Tokyo 162-8666, Japan
Author to whom correspondence should be addressed.
Academic Editor: Philippe Guilpain
J. Clin. Med. 2021, 10(6), 1240;
Received: 19 January 2021 / Revised: 10 March 2021 / Accepted: 12 March 2021 / Published: 17 March 2021
(This article belongs to the Special Issue Pediatric and Adolescent Rheumatology: A Rapidly Developing Field)
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder mainly characterised by increased risks of thrombosis and pregnancy morbidity and persistent positive test results for antiphospholipid antibodies (aPLs). The criteria for diagnosing juvenile APS have yet to be validated, while the Sydney classification criteria do not contain several non-thrombotic clinical manifestations associated with the presence of aPLs. As such, difficulties have been encountered in the diagnosis of patients who have no certain thrombotic occlusions. Moreover, extra-criteria manifestations (i.e., clinical manifestations not listed in the classification criteria), including neurologic manifestations (chorea, myelitis and migraine), haematologic manifestations (thrombocytopenia and haemolytic anaemia), livedo reticularis, nephropathy and valvular heart disease have been reported, which suggests that the clinical spectrum of aPL-related manifestations extends beyond that indicated in the classification criteria. Studies have demonstrated that more than 40% of children with aPLs demonstrated non-thrombotic aPL-related clinical manifestations alone. Moreover, our results showed that the pathogenesis of non-criteria manifestations is characterised by “APS vasculopathy”. The present review introduces the characteristics and findings of non-criteria manifestations observed in juvenile APS. View Full-Text
Keywords: antiphospholipid antibodies; children; chorea; systemic lupus erythematosus antiphospholipid antibodies; children; chorea; systemic lupus erythematosus
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MDPI and ACS Style

Miyamae, T.; Kawabe, T. Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome. J. Clin. Med. 2021, 10, 1240.

AMA Style

Miyamae T, Kawabe T. Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome. Journal of Clinical Medicine. 2021; 10(6):1240.

Chicago/Turabian Style

Miyamae, Takako, and Tomohiro Kawabe. 2021. "Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome" Journal of Clinical Medicine 10, no. 6: 1240.

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