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Title:
A Review of Complement Activation in SLE | Current Rheumatology Reports
Description:
Purpose of Review Complement activation is a key event in the pathogenesis of tissue inflammation and injury in systemic lupus erythematosus (SLE). This review is aimed at comparing the usefulness of measurement of complement proteins in serum/plasma (C3, C4) to complement activation (split) products in plasma and on circulating blood cells for SLE diagnosis, disease monitoring, and prognosis. Recent Findings Complement split products, C3dg, iC3b, and C4d, are elevated in SLE, and C3dg/C3 and iC3b/C3 ratios correlate with active SLE. C4d also is higher in patients with lupus nephritis. An elevated level of the alternative pathway split product, Bb, in early lupus pregnancy is a predictor of adverse outcomes in SLE patients with antiphospholipid antibodies. Elevated levels of cell-bound complement activation products (CB-CAPs), namely, B cell-bound C4d (BC4d) and erythrocyte-bound C4d (EC4d), within a multiparameter assay panel, may predict transition to SLE more than other lupus biomarkers. EC4d better correlates with lupus disease activity than low plasma complement levels. Elevated platelet-bound C4d (PC4d) correlates with thrombosis in SLE. Both EC4d and PC4d are increased in primary and secondary anti-phospholipid syndrome, and anti-beta2glycoproteinI antibodies may directly activate the complement system. Summary Abnormal levels of plasma complement split products and CB-CAPs support complement activation as an important pathogenetic mechanism in SLE and the antiphospholipid syndromes. These tests show promise for the diagnosis of SLE and monitoring of disease activity.
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Keywords {🔍}
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augment lupus-related dysfunction article download pdf cell-bound activation products open access published ahus—microangiopathic hemolytic anemia secondary anti-phospholipid syndrome systemic lupus erythematosus systemic lupus erythematosus 1186/s13075-017-1470-2this study showed time-resolved immunofluorimetric assay normal anti-c1q levels high anti-c1q levels blood cell-bound c4d plasma/serum complement proteins mbl mannose-binding lectin elevated platelet-bound c4d complement-mediated thrombotic microangiopathy privacy choices/manage cookies complement activation—low c3 c4d/c4 ratio decreased adverse pregnancy outcomes anti-beta2glycoproteini antibodies primary antiphospholipid syndrome plasma split products technical issues related rheumatic diseases complement split products obtain permission directly full access ic3b/c3 ratios correlate low complement c3/c4 classical complement pathway platelet-derived microparticles cell-bound c4d multinational inception cohort c4d/c4 ratio correlated plasma complement levels anti-ds dna 1136/lupus-2018-000263this compilation edta plasma tubes edta plasma frozen cytokines—interferon gamma platelet-bound c4d detect complement activation multivariate regression analysis mannose-binding lectin mannose binding lectin proliferative lupus nephritis lupus nephritis analyses normal healthy subjects
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headline:A Review of Complement Activation in SLE
description:Complement activation is a key event in the pathogenesis of tissue inflammation and injury in systemic lupus erythematosus (SLE). This review is aimed at comparing the usefulness of measurement of complement proteins in serum/plasma (C3, C4) to complement activation (split) products in plasma and on circulating blood cells for SLE diagnosis, disease monitoring, and prognosis. Complement split products, C3dg, iC3b, and C4d, are elevated in SLE, and C3dg/C3 and iC3b/C3 ratios correlate with active SLE. C4d also is higher in patients with lupus nephritis. An elevated level of the alternative pathway split product, Bb, in early lupus pregnancy is a predictor of adverse outcomes in SLE patients with antiphospholipid antibodies. Elevated levels of cell-bound complement activation products (CB-CAPs), namely, B cell-bound C4d (BC4d) and erythrocyte-bound C4d (EC4d), within a multiparameter assay panel, may predict transition to SLE more than other lupus biomarkers. EC4d better correlates with lupus disease activity than low plasma complement levels. Elevated platelet-bound C4d (PC4d) correlates with thrombosis in SLE. Both EC4d and PC4d are increased in primary and secondary anti-phospholipid syndrome, and anti-beta2glycoproteinI antibodies may directly activate the complement system. Abnormal levels of plasma complement split products and CB-CAPs support complement activation as an important pathogenetic mechanism in SLE and the antiphospholipid syndromes. These tests show promise for the diagnosis of SLE and monitoring of disease activity.
datePublished:2021-02-10T00:00:00Z
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Systemic lupus erythematosus
Complement
Complement activation measurement
Disease activity
Lupus nephritis
Rheumatology
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headline:A Review of Complement Activation in SLE
description:Complement activation is a key event in the pathogenesis of tissue inflammation and injury in systemic lupus erythematosus (SLE). This review is aimed at comparing the usefulness of measurement of complement proteins in serum/plasma (C3, C4) to complement activation (split) products in plasma and on circulating blood cells for SLE diagnosis, disease monitoring, and prognosis. Complement split products, C3dg, iC3b, and C4d, are elevated in SLE, and C3dg/C3 and iC3b/C3 ratios correlate with active SLE. C4d also is higher in patients with lupus nephritis. An elevated level of the alternative pathway split product, Bb, in early lupus pregnancy is a predictor of adverse outcomes in SLE patients with antiphospholipid antibodies. Elevated levels of cell-bound complement activation products (CB-CAPs), namely, B cell-bound C4d (BC4d) and erythrocyte-bound C4d (EC4d), within a multiparameter assay panel, may predict transition to SLE more than other lupus biomarkers. EC4d better correlates with lupus disease activity than low plasma complement levels. Elevated platelet-bound C4d (PC4d) correlates with thrombosis in SLE. Both EC4d and PC4d are increased in primary and secondary anti-phospholipid syndrome, and anti-beta2glycoproteinI antibodies may directly activate the complement system. Abnormal levels of plasma complement split products and CB-CAPs support complement activation as an important pathogenetic mechanism in SLE and the antiphospholipid syndromes. These tests show promise for the diagnosis of SLE and monitoring of disease activity.
datePublished:2021-02-10T00:00:00Z
dateModified:2021-02-10T00:00:00Z
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Systemic lupus erythematosus
Complement
Complement activation measurement
Disease activity
Lupus nephritis
Rheumatology
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