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Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Introduction: 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. Case Presentation: We pre...

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Autores principales: Pasqui, Edoardo, Camarri, Silvia, de Donato, Gianmarco, Gonnelli, Stefano, Palasciano, Giancarlo, Gennari, Luigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8566535/
https://www.ncbi.nlm.nih.gov/pubmed/34746249
http://dx.doi.org/10.3389/fcvm.2021.727276
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author Pasqui, Edoardo
Camarri, Silvia
de Donato, Gianmarco
Gonnelli, Stefano
Palasciano, Giancarlo
Gennari, Luigi
author_facet Pasqui, Edoardo
Camarri, Silvia
de Donato, Gianmarco
Gonnelli, Stefano
Palasciano, Giancarlo
Gennari, Luigi
author_sort Pasqui, Edoardo
collection PubMed
description Introduction: 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. Case Presentation: 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. Conclusion: In our case, the presence of a “sine causa” 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.
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spelling pubmed-85665352021-11-05 Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome Pasqui, Edoardo Camarri, Silvia de Donato, Gianmarco Gonnelli, Stefano Palasciano, Giancarlo Gennari, Luigi Front Cardiovasc Med Cardiovascular Medicine Introduction: 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. Case Presentation: 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. Conclusion: In our case, the presence of a “sine causa” 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. Frontiers Media S.A. 2021-10-21 /pmc/articles/PMC8566535/ /pubmed/34746249 http://dx.doi.org/10.3389/fcvm.2021.727276 Text en Copyright © 2021 Pasqui, Camarri, de Donato, Gonnelli, Palasciano and Gennari. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Cardiovascular Medicine
Pasqui, Edoardo
Camarri, Silvia
de Donato, Gianmarco
Gonnelli, Stefano
Palasciano, Giancarlo
Gennari, Luigi
Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title_full Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title_fullStr Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title_full_unstemmed Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title_short Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome
title_sort case report: an unusual case of acute lower limb ischemia as precursor of the asherson's syndrome
topic Cardiovascular Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8566535/
https://www.ncbi.nlm.nih.gov/pubmed/34746249
http://dx.doi.org/10.3389/fcvm.2021.727276
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