EDITORIAL - Microangiopathic antiphospholipid-associated syndromes revisited - new concepts

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Journal of Rheumatology

September 2007

Editorial

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Microangiopathic Antiphospholipid-Associated Syndromes Revisited - New

Concepts Relating to Antiphospholipid Antibodies and Syndromes

RONALD A. ASHERSON, MD, FRCP, FACP, FCP, FACR,

Professor of Immunology (Hon),

Division of Immunology, School of Pathology,

University of the Witwatersrand,

14 Sturdee Avenue, Rosebank,

Johannesburg, Gauteng, South Africa 2196;

SYLVIA PIERANGELI, MD, PhD,

Division of Rheumatology,

University of Texas,

Galveston, Texas, USA;

RICARD CERVERA, MD, FRCP, PhD,

Department of Autoimmune Diseases,

Hospital Clinic,

Barcelona, Catalonia, Spain

Address reprint requests to Dr. Asherson. E-mail: ashron@....

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In a recent issue of ls of the Rheumatic Diseases1 we proposed a case

for a " microangiopathic antiphospholipid syndrome " (MAPS) encompassing

several conditions mainly affecting the microvasculature of selected organs:

the liver in the HELLP syndrome (hemolysis, elevated liver enzymes, and low

platelet count); kidney, brain, and skin in thrombotic thrombocytopenic

purpura (TTP) and related syndromes; the bowel where it is predominant in

patients with catastrophic antiphospholipid syndrome (CAPS, Asherson's

syndrome) in whom no large-vessel occlusions are manifest (approximately

70%) and in patients with disseminated intravascular coagulation (DIC)

whatever the cause (usually sepsis) who might also demonstrate

antiphospholipid antibody (aPL) positivity. It was our intention initially

to include those patients who might also demonstrate evidence of a

microangiopathic hemolytic process (and in whom schistocytes might be

demonstrable) as well as those patients who, in addition to demonstrating

aPL positivity, did not demonstrate large-vessel occlusions so typical of

classic APS. Moreover, we have since analyzed a small group of patients with

relapsing CAPS2 who resemble TTP and are thus " TTP-like " and have concluded

that a group of patients may exist in whom the presence of aPL positivity

might not imply that they are in fact pathogenic but that their appearance

might be consequent on endothelial cell damage/injury. Pulmonary

hypertension (PHT) is one of these conditions and will be discussed further.

Infection-induced aPL may also be included, but a detailed discussion of

this topic is beyond the scope of this editorial. A minority only of

infections with aPL positivity may be accompanied by manifestation of the

APS. This might be because of individual susceptibility to form aPL (?

genetic) and, perhaps, only certain of the antibody populations primarily

induced may be pathogenic, affecting coagulation factors, and cells leading

to large-vessel occlusions, thrombocytopenia, etc.

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