ヌノムラ タカコ   Nunomura Takako
  布村 多佳子
   所属   医学部 医学科(東京女子医科大学病院)
   職種   准教授
論文種別 総説
言語種別 英語
査読の有無 査読あり
表題 Non-Criteria Manifestations of Juvenile Antiphospholipid Syndrome.
掲載誌名 正式名:Journal of clinical medicine
略  称:J Clin Med
ISSNコード:20770383/20770383
掲載区分国外
巻・号・頁 10(6),pp.1-1240
著者・共著者 Miyamae Takako, Kawabe Tomohiro
担当区分 筆頭著者
発行年月 2021/03
概要 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