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Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus
This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10474971/ https://www.ncbi.nlm.nih.gov/pubmed/37667720 http://dx.doi.org/10.7759/cureus.42922 |
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author | Liang, Jessica Mahmood, Raai Benchaala, Ilyes York, Russel Sarakbi, Housam |
author_facet | Liang, Jessica Mahmood, Raai Benchaala, Ilyes York, Russel Sarakbi, Housam |
author_sort | Liang, Jessica |
collection | PubMed |
description | This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE). This is the case of a young female with lupus and end-stage renal disease secondary to lupus nephritis who presented to the emergency department for shortness of breath and bilateral leg swelling that eventually progressed to catastrophic antiphospholipid syndrome. She developed pulmonary embolisms, axillary hematoma, and bilateral lower extremity digital gangrene. The treatment course consisted of anticoagulation, steroids, intravenous immunoglobulin (IVIG), above-knee amputation, and eventually rituximab. Diagnosis and treatment of digital ischemia can be complex, especially, in the setting of lupus where the differential diagnosis is broad. A high index of suspicion for CAPS is essential for early diagnosis and treatment. |
format | Online Article Text |
id | pubmed-10474971 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-104749712023-09-04 Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus Liang, Jessica Mahmood, Raai Benchaala, Ilyes York, Russel Sarakbi, Housam Cureus Allergy/Immunology This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE). This is the case of a young female with lupus and end-stage renal disease secondary to lupus nephritis who presented to the emergency department for shortness of breath and bilateral leg swelling that eventually progressed to catastrophic antiphospholipid syndrome. She developed pulmonary embolisms, axillary hematoma, and bilateral lower extremity digital gangrene. The treatment course consisted of anticoagulation, steroids, intravenous immunoglobulin (IVIG), above-knee amputation, and eventually rituximab. Diagnosis and treatment of digital ischemia can be complex, especially, in the setting of lupus where the differential diagnosis is broad. A high index of suspicion for CAPS is essential for early diagnosis and treatment. Cureus 2023-08-03 /pmc/articles/PMC10474971/ /pubmed/37667720 http://dx.doi.org/10.7759/cureus.42922 Text en Copyright © 2023, Liang et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Allergy/Immunology Liang, Jessica Mahmood, Raai Benchaala, Ilyes York, Russel Sarakbi, Housam Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title | Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title_full | Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title_fullStr | Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title_full_unstemmed | Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title_short | Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus |
title_sort | catastrophic antiphospholipid syndrome: a complex diagnosis in the setting of lupus |
topic | Allergy/Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10474971/ https://www.ncbi.nlm.nih.gov/pubmed/37667720 http://dx.doi.org/10.7759/cureus.42922 |
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