Clinical Reviews in Allergy & Immunology

, Volume 36, Issue 2, pp 109–125

Microthrombotic/Microangiopathic Manifestations of the Antiphospholipid Syndrome

  • Sonja Praprotnik
  • Dušan Ferluga
  • Alenka Vizjak
  • Anastazija Hvala
  • Tadej Avčin
  • Blaž Rozman
Article

DOI: 10.1007/s12016-008-8104-z

Cite this article as:
Praprotnik, S., Ferluga, D., Vizjak, A. et al. Clinic Rev Allerg Immunol (2009) 36: 109. doi:10.1007/s12016-008-8104-z

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 syndromeMicrovascular thrombotic/microangiopathic antiphospholipid syndromeClinical manifestationsDiagnostic criteriaThrombotic microangiopathyHistopathology

Copyright information

© Humana Press Inc. 2008

Authors and Affiliations

  • Sonja Praprotnik
    • 1
  • Dušan Ferluga
    • 2
  • Alenka Vizjak
    • 2
  • Anastazija Hvala
    • 2
  • Tadej Avčin
    • 3
  • Blaž Rozman
    • 1
  1. 1.Clinical Department of RheumatologyUniversity Medical Center LjubljanaLjubljanaSlovenia
  2. 2.Institute of Pathology, Faculty of Medicine University of LjubljanaLjubljanaSlovenia
  3. 3.Department of Allergology, Rheumatology and Clinical ImmunologyUniversity Children’s Hospital, University Medical Center LjubljanaLjubljanaSlovenia