RESEARCH - Pathogenesis of the antiphospholipid syndrome: an additional example of the mosaic of autoimmunity

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J Autoimmun. 2008 Feb-Mar;30(1-2):99-103.

Pathogenesis of the antiphospholipid syndrome: An additional example of the

mosaic of autoimmunity.

Meroni PL.

Department of Internal Medicine, University of Milan, IRCCS Istituto

Auxologico Italiano, Milan, Italy.

The antiphospholipid syndrome (APS) is a systemic autoimmune disease

characterized by an adaptive immune response against self-PL-binding

proteins ending in the production of specific autoantibodies.

Antiphospholipid antibodies (aPL; and in particular anti-beta2 glycoprotein

I antibodies) are formal diagnostic markers and pathogenic antibodies.

Although APS may be considered as an autoantibody-mediated disease, there is

now evidence that aPL are necessary but not sufficient to trigger some of

the clinical manifestations of the syndrome. For example, additional

factors, such as mediators of the innate immunity are now recognized to play

a key role as second hits able to induce the thrombotic events in the

presence of the autoantibodies. The APS scenario is also supplemented by the

influence of genetically determined factors. Finally, environmental agents -

in particular infectious ones - were reported to act as triggers for the

production of autoantibodies cross-reacting with PL-binding proteins as well

as inflammatory stimuli that potentiate the aPL thrombogenic effect.

Altogether these findings do support the concept of a mosaic of factors that

participate to the pathogenesis of the syndrome at different levels.

PMID: 18194729

http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve & db=PubMed & list_uids=18

194729 & dopt=AbstractPlus

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