Acquired von Willebrand Syndrome in Systemic Lupus Erythematodes
Acquired von Willebrand syndrome (AVWS) in systemic lupus erythematodes (SLE) is caused by autoantibodies directed against the circulating von Willebrand factor (vWF)/factor VIII (FVIII) complex. The autoantibody-vWF/ FVIII antigen complex is cleared rapidly from the circulation, leading to a modera...
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| Veröffentlicht in: | Clinical and applied thrombosis/hemostasis 2001-04, Vol.7 (2), p.106-112 |
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| creator | Michiels, Jan Jacques Schroyens, Wilfried van der Planken, Marc Bememan, Zwi |
| description | Acquired von Willebrand syndrome (AVWS) in systemic lupus erythematodes (SLE) is caused by autoantibodies directed against the circulating von Willebrand factor (vWF)/factor VIII (FVIII) complex. The autoantibody-vWF/ FVIII antigen complex is cleared rapidly from the circulation, leading to a moderate to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and normal platelet count, a prolonged activated partial thromboplastin time (APTT) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analysis of the vWF protein. Acquired von Wittebrand syndrome type II in SLE responds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FV III concentrate, but responds transiently well to high-dose gammaglobulin given intravenously. All reported cases of AvWS in SLE were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppressifln. |
| doi_str_mv | 10.1177/107602960100700205 |
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The autoantibody-vWF/ FVIII antigen complex is cleared rapidly from the circulation, leading to a moderate to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and normal platelet count, a prolonged activated partial thromboplastin time (APTT) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analysis of the vWF protein. Acquired von Wittebrand syndrome type II in SLE responds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FV III concentrate, but responds transiently well to high-dose gammaglobulin given intravenously. All reported cases of AvWS in SLE were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppressifln.</description><identifier>ISSN: 1076-0296</identifier><identifier>EISSN: 1938-2723</identifier><identifier>DOI: 10.1177/107602960100700205</identifier><identifier>PMID: 11292186</identifier><language>eng</language><publisher>Thousand Oaks, CA: SAGE Publications</publisher><subject>Adolescent ; Adult ; Aged ; Anti-Inflammatory Agents - administration & dosage ; Deamino Arginine Vasopressin - administration & dosage ; Factor VIII - administration & dosage ; Female ; Health risk assessment ; Hemorrhage ; Hemostatics - administration & dosage ; Humans ; Lupus ; Lupus Erythematosus, Systemic - blood ; Lupus Erythematosus, Systemic - complications ; Male ; Middle Aged ; Prednisone - administration & dosage ; Severity of Illness Index ; Treatment Outcome ; von Willebrand Diseases - drug therapy ; von Willebrand Diseases - etiology</subject><ispartof>Clinical and applied thrombosis/hemostasis, 2001-04, Vol.7 (2), p.106-112</ispartof><rights>Copyright SAGE PUBLICATIONS, INC. 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The autoantibody-vWF/ FVIII antigen complex is cleared rapidly from the circulation, leading to a moderate to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and normal platelet count, a prolonged activated partial thromboplastin time (APTT) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analysis of the vWF protein. Acquired von Wittebrand syndrome type II in SLE responds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FV III concentrate, but responds transiently well to high-dose gammaglobulin given intravenously. All reported cases of AvWS in SLE were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppressifln.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Anti-Inflammatory Agents - administration & dosage</subject><subject>Deamino Arginine Vasopressin - administration & dosage</subject><subject>Factor VIII - administration & dosage</subject><subject>Female</subject><subject>Health risk assessment</subject><subject>Hemorrhage</subject><subject>Hemostatics - administration & dosage</subject><subject>Humans</subject><subject>Lupus</subject><subject>Lupus Erythematosus, Systemic - blood</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Prednisone - administration & dosage</subject><subject>Severity of Illness Index</subject><subject>Treatment Outcome</subject><subject>von Willebrand Diseases - drug therapy</subject><subject>von Willebrand Diseases - etiology</subject><issn>1076-0296</issn><issn>1938-2723</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp9kEtLAzEUhYMotlb_gAsZENyNvTfJJJOdUuoDCi5UXA7JTEanzKNNZoT-e1NaKCi4OmfxnXMvh5BLhFtEKacIUgBVAhBAAlBIjsgYFUtjKik7Dj4A8ZYYkTPvlwCohBKnZIRIFcVUjMndfb4eKmeL6Ltro4-qrq1xui2i101buK6xUdUG73vbVHm0GFaDj-Zu03_ZRvddYf05OSl17e3FXifk_WH-NnuKFy-Pz7P7RZwzIfvYoE5loopEsByFYkApyzXQ3IBhaJLE8jKVnGsuSm6USjg3kIC0YIJKyybkZte7ct16sL7Pmsrntq51a7vBZ1IC45TzAF7_Apfd4NrwW0YZ56hoyjFQdEflrvPe2TJbuarRbpMhZNt1s7_rhtDVvnowjS0Okf2cAZjuAK8_7eHuP5U_tS9_rQ</recordid><startdate>20010401</startdate><enddate>20010401</enddate><creator>Michiels, Jan Jacques</creator><creator>Schroyens, Wilfried</creator><creator>van der Planken, Marc</creator><creator>Bememan, Zwi</creator><general>SAGE Publications</general><general>SAGE PUBLICATIONS, INC</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20010401</creationdate><title>Acquired von Willebrand Syndrome in Systemic Lupus Erythematodes</title><author>Michiels, Jan Jacques ; 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The autoantibody-vWF/ FVIII antigen complex is cleared rapidly from the circulation, leading to a moderate to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and normal platelet count, a prolonged activated partial thromboplastin time (APTT) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analysis of the vWF protein. Acquired von Wittebrand syndrome type II in SLE responds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FV III concentrate, but responds transiently well to high-dose gammaglobulin given intravenously. 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| identifier | ISSN: 1076-0296 |
| ispartof | Clinical and applied thrombosis/hemostasis, 2001-04, Vol.7 (2), p.106-112 |
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| language | eng |
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| source | SAGE Open Access Journals |
| subjects | Adolescent Adult Aged Anti-Inflammatory Agents - administration & dosage Deamino Arginine Vasopressin - administration & dosage Factor VIII - administration & dosage Female Health risk assessment Hemorrhage Hemostatics - administration & dosage Humans Lupus Lupus Erythematosus, Systemic - blood Lupus Erythematosus, Systemic - complications Male Middle Aged Prednisone - administration & dosage Severity of Illness Index Treatment Outcome von Willebrand Diseases - drug therapy von Willebrand Diseases - etiology |
| title | Acquired von Willebrand Syndrome in Systemic Lupus Erythematodes |
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