The Primary, Secondary, Catastrophic, and Seronegative Variants of the Antiphospholipid Syndrome: A Personal History Long in the Making

ABSTRACT

Although many of the clinical features accompanying lupus anticoagulant positivity were documented in the early 1960s and many “non–lupus patients” were also published, it was not until the discovery of antibodies to cardiolipin in the 1980s that the existence and true ramifications of a distinct antiphospholipid syndrome was defined. A primary syndrome was in fact recognized in 1985 by the author while at the Hammersmith Hospital and comprised 25 patients who conformed to this new subset of disease, which has now overtaken lupus-associated (secondary) antiphospholipid syndromes in frequency. However, publication of this important milestone was in fact prevented, because of the purveying dogma at that time that “these patients were all suffering from ‘lupus,’” which history has since proved to be incorrect. The syndrome was therefore only clearly defined and published in 1988. Subsequently, in the following year, a new and more comprehensive multicenter series comprising 70 patients was documented (including the original 25 patients from 1985) as well as two smaller series by other units. The catastrophic variant of the syndrome with distinct triggering factors, clinical features, and a generally poor prognosis was then defined in 1992, with more than 300 patients with this devastating condition now summarized on the University of Barcelona online registry. The existence of a seronegative syndrome has also been suggested, but whether this is related to the presence of undetectable antiphospholipid antibodies or perhaps represents a similar type of vasculopathy or endotheliopathy is unclear at the present time. This article documents a personal account of the events that took place in relation to the description of these syndromes.

Primary antiphospholipid syndrome - PAPS - catastrophic antiphospholipid (Asherson's) syndrome - CAPS - seronegative antiphospholipid syndrome - SNAPS

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