Article

Clinical Reviews in Allergy & Immunology

, Volume 36, Issue 2, pp 109-125

First online:

Microthrombotic/Microangiopathic Manifestations of the Antiphospholipid Syndrome

  • Sonja PraprotnikAffiliated withClinical Department of Rheumatology, University Medical Center Ljubljana
  • , Dušan FerlugaAffiliated withInstitute of Pathology, Faculty of Medicine University of Ljubljana
  • , Alenka VizjakAffiliated withInstitute of Pathology, Faculty of Medicine University of Ljubljana
  • , Anastazija HvalaAffiliated withInstitute of Pathology, Faculty of Medicine University of Ljubljana
  • , Tadej AvčinAffiliated withDepartment of Allergology, Rheumatology and Clinical Immunology, University Children’s Hospital, University Medical Center Ljubljana
  • , Blaž RozmanAffiliated withClinical Department of Rheumatology, University Medical Center Ljubljana Email author 

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Abstract

The paper presents an overview of clinical manifestations and histopathologic findings in different organs in microvascular thrombotic and microangiopathic antiphospholipid syndrome (MAPS). Subsets of antiphospholipid syndrome (APS) are presented and defined. Clinico-pathologic correlations seem insufficient so far, because of a lack of detailed systematic studies of the histopathology in different organs. Based on their own autopsy and biopsy studies, the authors propose a novel categorization of histopathologic lesions that occur in patients with classic and catastrophic APS. In addition to the already accepted category of a microvascular thrombotic type of lesions, microangiopathic lesions consistent with thrombotic microangiopathy are proposed to be included in new revised classification criteria for definite APS. Microvascular thrombotic and so far underestimated microangiopathic histopathologic lesions have been shown to appear in various combinations and of different ages in patients with both classic and catastrophic APS, which fits into the concept of MAPS. These preliminary findings of our studies are also in line with the most recent hypothesis of two main mechanisms in the pathogenesis of APS, emphasizing a key role of endothelial cell affection induced by aPL on the one hand and interference with coagulation cascade on the other side.

Keywords

Antiphospholipid syndrome Microvascular thrombotic/microangiopathic antiphospholipid syndrome Clinical manifestations Diagnostic criteria Thrombotic microangiopathy Histopathology