Cargando…

A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome

Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune disea...

Descripción completa

Detalles Bibliográficos
Autores principales: Vaidya, Binit, Nakarmi, Shweta, Joshi, Rakshya, Baral, Rikesh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Journal of the Nepal Medical Association 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8827589/
https://www.ncbi.nlm.nih.gov/pubmed/31477950
http://dx.doi.org/10.31729/jnma.4226
_version_ 1784647665999413248
author Vaidya, Binit
Nakarmi, Shweta
Joshi, Rakshya
Baral, Rikesh
author_facet Vaidya, Binit
Nakarmi, Shweta
Joshi, Rakshya
Baral, Rikesh
author_sort Vaidya, Binit
collection PubMed
description Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for the diagnosis of Anti-phospholipid Antibody Syndrome. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where Anti-phospholipid Antibody Syndrome should be suspected. Presence of non-criteria manifestations like livedo reticularis, skin ulcers, nephropathy, valvular heart disease and thrombocytopenia adds to the diagnostic clue for the presence of Anti-phospholipid Antibody Syndrome. Treatment of Anti-phospholipid Antibody Syndrome has preventive and therapeutic aspects that usually focus on thrombotic and obstetric manifestations of the disease. Therapeutic anti-coagulation with heparin followed by warfarin is required for patients presenting with acute thrombosis. Those with venous thrombosis are given moderate intensity warfarin (International Normalized Ratio, 2–3), whereas those with arterial thrombosis or recurrent venous thrombosis even on warfarin are treated with high intensity warfarin (International Normalized Ratio, 3–4). Similarly, anticoagulation with heparin is advised in patients with obstetric Anti-phospholipid Antibody Syndrome throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic Anti-phospholipid Antibody Syndrome positive patients and in those with non-criteria manifestations of the disease. Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholipid syndrome characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases.
format Online
Article
Text
id pubmed-8827589
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher Journal of the Nepal Medical Association
record_format MEDLINE/PubMed
spelling pubmed-88275892022-03-01 A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome Vaidya, Binit Nakarmi, Shweta Joshi, Rakshya Baral, Rikesh JNMA J Nepal Med Assoc Review Article Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for the diagnosis of Anti-phospholipid Antibody Syndrome. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where Anti-phospholipid Antibody Syndrome should be suspected. Presence of non-criteria manifestations like livedo reticularis, skin ulcers, nephropathy, valvular heart disease and thrombocytopenia adds to the diagnostic clue for the presence of Anti-phospholipid Antibody Syndrome. Treatment of Anti-phospholipid Antibody Syndrome has preventive and therapeutic aspects that usually focus on thrombotic and obstetric manifestations of the disease. Therapeutic anti-coagulation with heparin followed by warfarin is required for patients presenting with acute thrombosis. Those with venous thrombosis are given moderate intensity warfarin (International Normalized Ratio, 2–3), whereas those with arterial thrombosis or recurrent venous thrombosis even on warfarin are treated with high intensity warfarin (International Normalized Ratio, 3–4). Similarly, anticoagulation with heparin is advised in patients with obstetric Anti-phospholipid Antibody Syndrome throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic Anti-phospholipid Antibody Syndrome positive patients and in those with non-criteria manifestations of the disease. Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholipid syndrome characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases. Journal of the Nepal Medical Association 2019-04 2019-04-30 /pmc/articles/PMC8827589/ /pubmed/31477950 http://dx.doi.org/10.31729/jnma.4226 Text en © The Author(s) 2018. https://creativecommons.org/licenses/by/4.0/This is an Open-Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Vaidya, Binit
Nakarmi, Shweta
Joshi, Rakshya
Baral, Rikesh
A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title_full A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title_fullStr A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title_full_unstemmed A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title_short A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome
title_sort simplified understanding of the black swan: anti-phospholipid antibody syndrome
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8827589/
https://www.ncbi.nlm.nih.gov/pubmed/31477950
http://dx.doi.org/10.31729/jnma.4226
work_keys_str_mv AT vaidyabinit asimplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT nakarmishweta asimplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT joshirakshya asimplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT baralrikesh asimplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT vaidyabinit simplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT nakarmishweta simplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT joshirakshya simplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome
AT baralrikesh simplifiedunderstandingoftheblackswanantiphospholipidantibodysyndrome