ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status?

To clarify the role of ADAMTS-13 in the pathogenesis of thrombotic microangiopathy in systemic lupus erythematosus (SLE) we evaluated ADAMTS-13 profile (metalloprotease antigen levels, anti-ADAMTS-13 autoantibody levels, activity) in distinct patient groups according to disease activity, extent of c...

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Veröffentlicht in:Lupus 2013-04, Vol.22 (5), p.443-452
Hauptverfasser: Klonizakis, P, Tselios, K, Sarantopoulos, A, Gougourellas, I, Rouka, E, Onufriadou, Z, Kapali, P, Kyriakou, D, Boura, P
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container_issue 5
container_start_page 443
container_title Lupus
container_volume 22
creator Klonizakis, P
Tselios, K
Sarantopoulos, A
Gougourellas, I
Rouka, E
Onufriadou, Z
Kapali, P
Kyriakou, D
Boura, P
description To clarify the role of ADAMTS-13 in the pathogenesis of thrombotic microangiopathy in systemic lupus erythematosus (SLE) we evaluated ADAMTS-13 profile (metalloprotease antigen levels, anti-ADAMTS-13 autoantibody levels, activity) in distinct patient groups according to disease activity, extent of cumulative tissue damage and history of antiphospholipid syndrome or end-organ damage. Forty-one lupus patients were analysed. ADAMTS-13 metalloprotease antigen levels and anti-ADAMTS-13 autoantibodies were evaluated by ELISA. ADAMTS-13 activity was measured by Fluorescence resonance energy transfer (FRET) technique. ADAMTS-13 metalloprotease antigen levels were significantly decreased in patients with Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) >1 (p 
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Forty-one lupus patients were analysed. ADAMTS-13 metalloprotease antigen levels and anti-ADAMTS-13 autoantibodies were evaluated by ELISA. ADAMTS-13 activity was measured by Fluorescence resonance energy transfer (FRET) technique. ADAMTS-13 metalloprotease antigen levels were significantly decreased in patients with Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) &gt;1 (p &lt; 0.05). ADAMTS-13 metalloprotease antigen levels also exhibited a significant inverse correlation with anti-dsDNA levels (r = −0.60, p &lt; 0.05). Anti-ADAMTS-13 autoantibodies were marginally higher in patients with positive anti-dsDNA (p = 0.08). Additionally, patients with positive anti-ADAMTS-13 autoantibodies exhibited the lowest activity levels (p &lt; 0.05). To our knowledge ADAMTS-13 profile in SLE has not been studied in regard to composite structured indices. The results of this study suggest that in patients with active SLE or considerable cumulative tissue damage, ADAMTS-13 levels may be decreased and anti-ADAMTS-13 autoantibodies may partially mediate this reduction. Further evaluation of ADAMTS-13 profile may explain its role in the pathogenesis of thrombotic microangiopathy in lupus patients and reveal a potential prognostic marker of microthrombotic manifestations in SLE.</description><identifier>ISSN: 0961-2033</identifier><identifier>EISSN: 1477-0962</identifier><identifier>DOI: 10.1177/0961203313477898</identifier><identifier>PMID: 23554033</identifier><language>eng</language><publisher>London, England: SAGE Publications</publisher><subject>ADAM Proteins - antagonists &amp; inhibitors ; ADAM Proteins - blood ; ADAM Proteins - immunology ; ADAMTS13 Protein ; Adult ; Aged ; Anti-DNA antibodies ; Anticoagulants ; Antigens ; Autoantibodies - blood ; Autoantibodies - physiology ; Biomarkers - blood ; Down-Regulation - immunology ; Female ; Hematology ; Humans ; Immunology ; Internal medicine ; Lupus ; Lupus Erythematosus, Systemic - enzymology ; Lupus Erythematosus, Systemic - immunology ; Lupus Erythematosus, Systemic - pathology ; Male ; Middle Aged ; Pathogenesis ; Patients ; Plasma ; Retrospective Studies ; Young Adult</subject><ispartof>Lupus, 2013-04, Vol.22 (5), p.443-452</ispartof><rights>The Author(s), 2013. 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Forty-one lupus patients were analysed. ADAMTS-13 metalloprotease antigen levels and anti-ADAMTS-13 autoantibodies were evaluated by ELISA. ADAMTS-13 activity was measured by Fluorescence resonance energy transfer (FRET) technique. ADAMTS-13 metalloprotease antigen levels were significantly decreased in patients with Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) &gt;1 (p &lt; 0.05). ADAMTS-13 metalloprotease antigen levels also exhibited a significant inverse correlation with anti-dsDNA levels (r = −0.60, p &lt; 0.05). Anti-ADAMTS-13 autoantibodies were marginally higher in patients with positive anti-dsDNA (p = 0.08). Additionally, patients with positive anti-ADAMTS-13 autoantibodies exhibited the lowest activity levels (p &lt; 0.05). To our knowledge ADAMTS-13 profile in SLE has not been studied in regard to composite structured indices. 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Forty-one lupus patients were analysed. ADAMTS-13 metalloprotease antigen levels and anti-ADAMTS-13 autoantibodies were evaluated by ELISA. ADAMTS-13 activity was measured by Fluorescence resonance energy transfer (FRET) technique. ADAMTS-13 metalloprotease antigen levels were significantly decreased in patients with Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) &gt;1 (p &lt; 0.05). ADAMTS-13 metalloprotease antigen levels also exhibited a significant inverse correlation with anti-dsDNA levels (r = −0.60, p &lt; 0.05). Anti-ADAMTS-13 autoantibodies were marginally higher in patients with positive anti-dsDNA (p = 0.08). Additionally, patients with positive anti-ADAMTS-13 autoantibodies exhibited the lowest activity levels (p &lt; 0.05). To our knowledge ADAMTS-13 profile in SLE has not been studied in regard to composite structured indices. The results of this study suggest that in patients with active SLE or considerable cumulative tissue damage, ADAMTS-13 levels may be decreased and anti-ADAMTS-13 autoantibodies may partially mediate this reduction. Further evaluation of ADAMTS-13 profile may explain its role in the pathogenesis of thrombotic microangiopathy in lupus patients and reveal a potential prognostic marker of microthrombotic manifestations in SLE.</abstract><cop>London, England</cop><pub>SAGE Publications</pub><pmid>23554033</pmid><doi>10.1177/0961203313477898</doi><tpages>10</tpages></addata></record>
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subjects ADAM Proteins - antagonists & inhibitors
ADAM Proteins - blood
ADAM Proteins - immunology
ADAMTS13 Protein
Adult
Aged
Anti-DNA antibodies
Anticoagulants
Antigens
Autoantibodies - blood
Autoantibodies - physiology
Biomarkers - blood
Down-Regulation - immunology
Female
Hematology
Humans
Immunology
Internal medicine
Lupus
Lupus Erythematosus, Systemic - enzymology
Lupus Erythematosus, Systemic - immunology
Lupus Erythematosus, Systemic - pathology
Male
Middle Aged
Pathogenesis
Patients
Plasma
Retrospective Studies
Young Adult
title ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status?
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