{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T15:49:34Z","timestamp":1753890574061,"version":"3.41.2"},"reference-count":9,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T00:00:00Z","timestamp":1634774400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:p><jats:bold>Introduction:<\/jats:bold> Asherson's Syndrome, also defined as Catastrophic Antiphospholipid Syndrome (CAPS), represents the most severe manifestation of Antiphospholipid Antibody Syndrome. Rarely, the first CAPS diagnosis is based on macro-thrombotic event as acute limb ischemia.<\/jats:p><jats:p><jats:bold>Case Presentation:<\/jats:bold> We present a case of a 65-year-old woman admitted with an acute lower limb arterial ischemia with a complete occlusion of all the three tibial vessels. Three endovascular recanalization procedures were performed contemporary to 48 h intraarterial thrombolysis administration. The patency of tibial arteries was restored with a near-complete absence of digital arteries and microvessel perfusion of the foot. In the following days, an aggressive foot gangrene was established, leading to a major lower-limb amputation. Due to the general clinical status worsening and aggressiveness of ischemic condition, further investigations were performed leading to the diagnosis of an aggressive Asherson's Syndrome that was also complicated by a severe heparin-induced thrombocytopenia. Medical management with a high dose of intravenous steroids and nine sessions of plasma exchange led to a clinical condition stabilization.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> In our case, the presence of a \u201csine causa\u201d acute arterial occlusion of a large vessel represented the first manifestation of an aggressive form of Asherson's Syndrome that could represent a fatal disease. Due to the extreme variety of manifestations, early clinical suspicion, diagnosis, and multidisciplinary management are essential to limit the life-threatening consequences of patients.<\/jats:p>","DOI":"10.3389\/fcvm.2021.727276","type":"journal-article","created":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T04:59:09Z","timestamp":1634792349000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome"],"prefix":"10.3389","volume":"8","author":[{"given":"Edoardo","family":"Pasqui","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Camarri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianmarco","family":"de Donato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Gonnelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giancarlo","family":"Palasciano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luigi","family":"Gennari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,10,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jaut.2008.10.003","article-title":"The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: compelling evidence","volume":"32","author":"Ortega-Hernandez","year":"2009","journal-title":"J Autoimmun."},{"key":"B2","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1097\/00005792-199805000-00005","article-title":"Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients","volume":"77","author":"Asherson","year":"1998","journal-title":"Medicine (Baltimore)"},{"key":"B3","doi-asserted-by":"publisher","first-page":"820","DOI":"10.1016\/j.ejvs.2021.01.004","article-title":"Safety and efficacy of vacuum assisted thrombo-aspiration in patients with acute lower limb ischaemia: the INDIAN trial","volume":"61","author":"de Donato","year":"2021","journal-title":"Eur J Vasc Endovasc Surg."},{"key":"B4","doi-asserted-by":"publisher","first-page":"530","DOI":"10.1191\/0961203303lu394oa","article-title":"Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines","volume":"12","author":"Asherson","year":"2003","journal-title":"Lupus."},{"key":"B5","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1016\/S0002-9343(98)00061-8","article-title":"Thrombotic storm: when thrombosis begets thrombosis","volume":"104","author":"Kitchens","year":"1998","journal-title":"Am J Med"},{"key":"B6","doi-asserted-by":"publisher","first-page":"3495","DOI":"10.1182\/blood-2002-08-2349","article-title":"Role of the MyD88 transduction signaling pathway in endothelial activation by antiphospholipid antibodies","volume":"101","author":"Raschi","year":"2003","journal-title":"Blood"},{"key":"B7","doi-asserted-by":"publisher","first-page":"190","DOI":"10.1007\/s11926-007-0031-0","article-title":"Mechanisms of aPL-mediated thrombosis: effects of aPL on endothelium and platelets","volume":"9","author":"Vega-Ostertag","year":"2007","journal-title":"Curr Rheumatol Rep"},{"key":"B8","doi-asserted-by":"publisher","first-page":"740","DOI":"10.1136\/ard.2006.061671","article-title":"Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases","volume":"66","author":"G\u00f3mez-Puerta","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"B9","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/ajh.26138","article-title":"A rare case of catastrophic antiphospholipid syndrome triggered by estrogen-containing oral contraceptives in a patient with double heterozygous factor V Leiden and prothrombin G20210A mutations","author":"McRae","year":"2021","journal-title":"Am J Hematol."}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2021.727276\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T04:59:11Z","timestamp":1634792351000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2021.727276\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,21]]},"references-count":9,"alternative-id":["10.3389\/fcvm.2021.727276"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2021.727276","relation":{},"ISSN":["2297-055X"],"issn-type":[{"type":"electronic","value":"2297-055X"}],"subject":[],"published":{"date-parts":[[2021,10,21]]},"article-number":"727276"}}