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PubMed · 9682289

The antiphospholipid syndrome.

A Muir
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Abstract

The antiphospholipid syndrome is now well established as an important, acquired prothrombotic disorder. Advances are being made in the understanding of the condition and the paradox of the in vitro anticoagulation, and the in vivo prothrombotic tendency. Newer and better assays for the diagnosis of antiphospholipid syndrome can be expected in the near future. Large, multi-centre, controlled trials to determine the optimum treatment of patients who test positive for the antiphospholipid syndrome, but have not suffered a thrombotic event, are required.

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Antibodies, AntiphospholipidAntiphospholipid SyndromeClinical Trials as TopicFemaleHumansIncidenceMalePrognosisRisk FactorsScotlandJournal ArticleReview

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BibTeXRIS

A Muir. 1998. The antiphospholipid syndrome.. https://doi.org/10.1177/003693309804300302

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PubMed2007

Binding of antiphospholipid antibodies to discontinuous epitopes on domain I of human beta(2)-glycoprotein I: mutation studies including residues R39 to R43.

OBJECTIVE: Pathogenic antiphospholipid antibodies (aPL) bind the self antigen N-terminal domain (domain I) of beta(2)-glycoprotein I (beta(2)GPI), with residues G40-R43 being important. However, peptides homologous to other regions of domain I have also been shown to bind aPL. Furthermore, there are no published reports of the effects of altering R39, which has greater surface exposure than the G40-R43 residues. METHODS: We used a novel, efficient method of production and purification of human domain I by Escherichia coli to create multiple mutants of domain I. These domain I mutants were then screened for binding to a range of polyclonal IgG purified from patients with antiphospholipid syndrome, using both solid-phase and fluid-phase assays. RESULTS: E coli-expressed purified domain I selectively bound IgG derived from patients with antiphospholipid syndrome. In region R39-R43, the R39S mutation had the greatest effect in terms of reducing binding to a panel of aPL in the fluid phase (mean +/- SD inhibition 14 +/- 18.5% versus 44.1 +/- 31.7% for G40E and 62.9 +/- 25.7% for wild-type domain I). Conversely, altering both D8 and D9 to S8 and G9, respectively, had the effect of enhancing binding to aPL in the fluid phase. Adding the remainder of the domain I-II interlinker resulted in enhanced binding over wild-type in the solid phase but not the fluid phase. CONCLUSION: The binding of aPL to beta(2)GPI domain I is complex and likely to involve discontinuous epitopes that include R39 in addition to G40-R43, the domain I-II interlinker, and possibly D8 and D9. Domain I variants with enhanced binding to aPL compared with wild-type domain I may aid in the development of novel therapies.

Antibodies, Antiphospholipid↗
PubMed2006

A case of Churg-Strauss syndrome associated with antiphospholipid antibodies.

Churg-Strauss syndrome (CSS) is a systemic vasculitis affecting both small- and medium-sized blood vessels, almost invariably affecting the lung, and frequently associated with cutaneous involvement. Microvascular vaso-occlusion leading to digital gangrene is not a feature of CSS. We report an unusual case of a patient with CSS with antiphospholipid antibodies who developed severe digital gangrene in addition to cutaneous vasculitis. The presence of antiphospholipid antibodies is not a feature usually seen in association with CSS. While the full clinical spectrum of CSS is still being defined, the identification of additional features associated with this syndrome might help to better understand the pathogenesis of the disease and to have an impact on both management and prognosis.

Antibodies, Antiphospholipid↗