川邊 智宏
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Assistant Professor
Article types Review article
Language English
Peer review Peer reviewed
Title Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome.
Journal Formal name:Journal of clinical medicine
Abbreviation:J Clin Med
ISSN code:20770383/20770383
Domestic / ForeginForegin
Volume, Issue, Page 10(6),pp.1-1240
Author and coauthor Miyamae Takako, Kawabe Tomohiro
Authorship Last author
Publication date 2021/03
Summary 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.
DOI 10.3390/jcm10061240
PMID 33802787