{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T12:16:46Z","timestamp":1763036206095},"reference-count":15,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2005,12,12]],"date-time":"2005-12-12T00:00:00Z","timestamp":1134345600000},"content-version":"vor","delay-in-days":3055,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[1997,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The catastrophic antiphospholipid syndrome (CAPS) is rare and usually fatal. In this report, we describe an unusual patient who, 31 years after experiencing an atypical preeclampsia\u2010eclampsia presentation known today as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), developed CAPS, which seemed to complicate a diagnosis of primary antiphospholipid syndrome. She responded to repeated plasmapheresis over 3 years. Anticoagulants, corticosteroids, intravenous gamma globulin, and intravenous cyclophosphamide had all failed to halt the progression of CAPS, but repeated plasmapheresis not only halted the condition, but it led to the reversal of a leukoencephalopathy. The relationship between HELLP syndrome and CAPS is discussed, and possible patho\u2010genetic mechanisms that explain the efficacy of repeated plasmapheresis in this setting are suggested. It is postulated that perhaps plasmapheresis, through removal of cytokines or other mediators, disrupts the interaction between phospholipid\u2010protein complexes and endothelial cells. Repeated plasmapheresis should be considered in the most refractory cases of CAPS when more conventional treatment regimens have failed.<\/jats:p>","DOI":"10.1002\/art.1780400823","type":"journal-article","created":{"date-parts":[[2007,2,14]],"date-time":"2007-02-14T12:40:26Z","timestamp":1171456826000},"page":"1534-1539","source":"Crossref","is-referenced-by-count":71,"title":["Catastrophic antiphospholipid syndrome. Response to repeated plasmapheresis over three years"],"prefix":"10.1002","volume":"40","author":[{"given":"C. Michael","family":"Neuwelt","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David I.","family":"Daikh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John A.","family":"Linfoot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Pfister","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robyn G.","family":"Young","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald L.","family":"Webb","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stuart S.","family":"London","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald A.","family":"Asherson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,12,12]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"Primer on the Rheumatic Diseases","author":"Harris EN","year":"1993"},{"key":"e_1_2_1_3_2","volume-title":"Phospholipid Binding Antibodies","author":"Asherson RA","year":"1991"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.151.2.231"},{"key":"e_1_2_1_5_2","first-page":"508","article-title":"The catastrophic antiphospholipid syndrome","volume":"19","author":"Asherson RA","year":"1992","journal-title":"J Rheumatol"},{"key":"e_1_2_1_6_2","first-page":"343","article-title":"Catastrophic antiphospholipid crisis","volume":"12","author":"Piette JC","year":"1994","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1159\/000168432"},{"key":"e_1_2_1_8_2","first-page":"1360","article-title":"An association between refractory HELLP syndrome and antiphospholipid antibodies during pregnancy: a report of two cases","volume":"21","author":"Ornstein MH","year":"1994","journal-title":"J Rheumatol"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9378(95)91470-6"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199504133321504"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-119-8-199310150-00008"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780390103"},{"key":"e_1_2_1_13_2","first-page":"1835","article-title":"Systemic lupus erythematosus and the antiphospholipid syndrome: reflections about the relevance of ARA criteria","volume":"19","author":"Piette J\u2010C","year":"1992","journal-title":"J Rheumatol"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602223340803"},{"key":"e_1_2_1_15_2","first-page":"1495","article-title":"Subcortical white matter lesions in osmotic demyelination syndrome","volume":"16","author":"Bourgouin PM","year":"1995","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199606273342613"}],"container-title":["Arthritis &amp; Rheumatism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fart.1780400823","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.1780400823","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T22:03:07Z","timestamp":1700776987000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/art.1780400823"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,8]]},"references-count":15,"journal-issue":{"issue":"8","published-print":{"date-parts":[[1997,8]]}},"alternative-id":["10.1002\/art.1780400823"],"URL":"https:\/\/doi.org\/10.1002\/art.1780400823","archive":["Portico"],"relation":{},"ISSN":["0004-3591","1529-0131"],"issn-type":[{"value":"0004-3591","type":"print"},{"value":"1529-0131","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,8]]}}}