The Antiphospholipid Syndrome

Levine, Jerrold S.; Branch, D. Ware; Rauch, Joyce

doi:10.1056/nejmra002974

Cited by 1,381 publications

(1,055 citation statements)

References 85 publications

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“…In our experience, APLA were detected in 64% of SLE patients, 41% of patients with ITP, and 27% of patients with AIHA (personal data). These results are close to those reported in the literature as the prevalence of APLA ranges from 18% to 65% in SLE patients, from 40% to 50% in patients with ITP, and from 14% to 23% in patients with AIHA [34,35]. In Senecal's report, the incidence of APLA was significantly higher in anti-lamin B 1 positive as compared to anti-lamin B 1 negative SLE patients (62.5% vs. 19.8%).…”

Section: Discussionsupporting

confidence: 87%

Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides

et al. 2004

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A subset of anti-nuclear autoantibodies (ANA) are directed against nuclear envelope (NE) polypeptides and display by indirect immunofluorescence (IIF) a ring-like fluorescent pattern. We report herein 19 patients with autoimmune cytopenias associated with antibodies (Abs) to NE polypeptides. Anti-NE specificity was determined by immunoblot, using NE preparations and purified lamina fractions. Eleven sera reacted with lamin B 1 , and two reacted with both lamin B 1 and an unidentified 150-kDa protein (p150). One serum reacted with only p150. Four sera reacted with lamins A and C, and one reacted with and an unidentified 52-kDa NE polypeptide (p52). Autoimmune cytopenias included hemolytic anemia (7 cases), thrombocytopenia (13 cases), and neutropenia (6 cases). Five patients had 2 (3 cases) or 3 (2 cases) different cytopenias. Antiphospholipid antibodies (APLA) were detected in 14 patients, 2 of whom experienced thromboembolic events. A liver disorder was present in 7 patients. Systemic lupus erythematosus and lupus-like syndrome were diagnosed in 11 and 2 patients, respectively. Cytopenias responded to steroids alone (13 patients), or together with intravenous immunoglobulins (2 patients), or cyclophosphamide (2 patients). Two patients did not require treatment. Our results suggest that anti-NE Abs need to be sought for in patients with peripheral cytopenias, particularly when they are associated with APLA and/or liver disorders. Their detection strongly suggests an autoimmune process. Such cytopenias are often manifestations of a lupus or lupus-like disease and are responsive to steroids. Am.

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Section: Discussionsupporting

confidence: 87%

Autoimmune cytopenias associated with autoantibodies to nuclear envelope polypeptides

et al. 2004

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“…We and others have shown that aPL is associated with thrombovascular events (21). In murine models of APS, aPL promotes thrombus formation in the presence of vascular injury and/or endothelial activation, but does not itself initiate formation of the thrombus (35,36).…”

Section: Discussionmentioning

confidence: 85%

“…We further hypothesized that anti-Hsp60 might increase the risk of vascular events in individuals with known thrombovascular risk factors, such as antiphospholipid antibodies (aPL). Antiphospholipid antibodies (LAC, anticardiolipin antibody [aCL], and anti-␤ 2 -glycoprotein I [anti-␤ 2 GPI]) are associated with thrombovascular events (19)(20)(21), but it remains unclear why clinical events occur in only some individuals with aPL. Our findings demonstrate that the presence of anti-Hsp60 itself is associated with an increased risk of arterial, but not venous, vascular events.…”

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confidence: 99%

Association of autoantibodies to heat‐shock protein 60 with arterial vascular events in patients with antiphospholipid antibodies

Dieudé¹,

Correa²,

Neville³

et al. 2011

Arthritis & Rheumatism

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Objective. Anti-heat shock protein 60 autoantibodies (anti-Hsp60) are associated with cardiovascular disease and are known to affect endothelial cells in vitro, and we have recently shown that anti-Hsp60 promote thrombosis in a murine model of arterial injury. Based on those findings, we undertook the present study to investigate the hypothesis that the presence of antiHsp60, alone or in combination with other thrombogenic risk factors, is associated with an elevated risk of vascular events.Methods. The study population was derived from 3 ongoing cohort studies: 2 independent systemic lupus erythematosus (SLE) registries and 1 cohort comprising SLE patients and non-SLE patients. Data from a total of 402 participants were captured; 199 of these participants had had confirmed vascular events (arterial vascular events in 102, venous vascular events in 76, and both arterial and venous vascular events in 21). Anti-Hsp60 were detected by enzyme-linked immunoassay, and association with vascular events was assessed by regression analysis. Conclusion. We demonstrate that anti-Hsp60 are associated with an increased risk of arterial vascular events, but not venous vascular events, in aPL-positive individuals. These data suggest that anti-Hsp60 may serve as a useful biomarker to distinguish risk of arterial and venous vascular events in patients with aPL. Results. Multiple regression analysis revealed

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“…Antiphospholipid antibodies (aPL) are now recognized as representing the most frequent acquired risk factor for thrombophilia and as being a treatable cause of pregnancy loss (1). With the widespread use of tests to detect aPL, rheumatologists and obstetricians frequently have to make decisions regarding the consequences of positive test results in women who wish to conceive or are pregnant.…”

Section: Introductionmentioning

confidence: 99%