REVIEW - The physiopathology of the catastrophic antiphospholipid (Asherton's) syndrome: compelling evidence

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J Autoimmun. 2009 Feb;32(1):1-6. Epub 2008 Dec 6.

The physiopathology of the catastrophic antiphospholipid (Asherson's)

syndrome: compelling evidence.

Ortega- OD, Agmon-Levin N, Blank M, Asherson RA, Shoenfeld Y.

Department of Medicine 'B' and Research Center for Autoimmune

Diseases, Sheba Medical Center, Tel-Hashomer 52621, Israel.

Catastrophic antiphospholipid (Asherson's) syndrome (cAPS) was

described in the past as a severe variant of the antiphospholipid

syndrome (APS). Currently growing evidence suggests it is a unique

condition. This statement is based on several clinical and

physiopathological features that although not well understood define

cAPS by itself. The remarkable features of cAPS are the presence of

antiphospholipid antibodies (aPLAs) and microthromboses. Additional

physiopathological features are the presence of anemia and

thrombocytopenia, which are also often described in similar autoimmune

conditions. A strong association with concomitant infection is thought

to act as the main trigger of microthromboses in cAPS. Several

theories have been proposed to explain these physiopathological

features. Some of them suggest the possibility of molecular mimicry

between components of infectious microorganisms and natural

anticoagulants, which might be involved in the production of

cross-reacting aPLAs in cAPS. Some genetic risk factors have also been

suggested to be implicated in the onset of cAPS, however they have not

been defined yet. Herein, we review the remarkable physiopathological

features commonly described in cAPS hitherto. We concluded that

although they are not completely understood, it is possible to

differentiate them from similar conditions. Nevertheless further

studies on these physiopathological mechanisms of the disease are

needed.

PMID: 19059760

http://www.ncbi.nlm.nih.gov/pubmed/19059760

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