Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review
Abstract Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare form of antiphospholipid syndrome, an autoimmune condition characterized by vascular thromboses, pregnancy loss, and antiphospholipid (aPL) antibodies. Diagnosis of CAPS relies on thrombosis of at least three different organs...
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| Format: | Article |
| Language: | English |
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Wiley
2020-07-01
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| Series: | eJHaem |
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| Online Access: | https://doi.org/10.1002/jha2.71 |
| _version_ | 1797740001895645184 |
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| author | Melody Hermel David Hermel Saif Azam Jerold Shinbane Annahita Sarcon Erika Jones Arjun Mehta Luanda Grazette Howard Liebman Ilene Weitz |
| author_facet | Melody Hermel David Hermel Saif Azam Jerold Shinbane Annahita Sarcon Erika Jones Arjun Mehta Luanda Grazette Howard Liebman Ilene Weitz |
| author_sort | Melody Hermel |
| collection | DOAJ |
| description | Abstract Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare form of antiphospholipid syndrome, an autoimmune condition characterized by vascular thromboses, pregnancy loss, and antiphospholipid (aPL) antibodies. Diagnosis of CAPS relies on thrombosis of at least three different organs systems over 1 week, histopathological evidence of small vessel occlusion, and high aPL antibody titers. In a subset of precipitating circumstances, activation or disruption of endothelial cells in the microvasculature may occur along with cardiomyopathy. We present two cases of CAPS‐associated dilated cardiomyopathy at our institution, focusing on disease management, pathophysiology, and treatment. These patients were of Southeastern Asian descent, raising the possibility of genetic polymorphisms contributing to the development of cardiomyopathy. Both met CAPS criteria and both demonstrated clinicopathologic thrombotic microangiopathy (TMA) and complement activation and developed severe dilated cardiomyopathy with shock. Complement activation plays an important role in the development of CAPS and may be important in the pathogenesis of CAPS‐associated cardiomyopathy. Clinical suspicion for TMA as a pathophysiologic mechanism of unexplained heart failure in CAPS is important and increased awareness of cardiac side effects is necessary so that early treatment can be initiated to halt further cardiac and systemic complications. |
| first_indexed | 2024-03-12T14:06:15Z |
| format | Article |
| id | doaj.art-f338693891324d91aa09b1828cdc7e79 |
| institution | Directory Open Access Journal |
| issn | 2688-6146 |
| language | English |
| last_indexed | 2024-03-12T14:06:15Z |
| publishDate | 2020-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | eJHaem |
| spelling | doaj.art-f338693891324d91aa09b1828cdc7e792023-08-21T14:10:49ZengWileyeJHaem2688-61462020-07-0111445010.1002/jha2.71Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and reviewMelody Hermel0David Hermel1Saif Azam2Jerold Shinbane3Annahita Sarcon4Erika Jones5Arjun Mehta6Luanda Grazette7Howard Liebman8Ilene Weitz9Department of Medicine Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Medicine Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Medicine Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Cardiology Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Cardiology Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Cardiology Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Pathology Keck School of Medicine University of Southern California Los Angeles CaliforniaDepartment of Cardiology Keck School of Medicine University of Southern California Los Angeles CaliforniaJane Anne Nohl Division of Hematology Keck School of Medicine University of Southern California Los Angeles CaliforniaJane Anne Nohl Division of Hematology Keck School of Medicine University of Southern California Los Angeles CaliforniaAbstract Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare form of antiphospholipid syndrome, an autoimmune condition characterized by vascular thromboses, pregnancy loss, and antiphospholipid (aPL) antibodies. Diagnosis of CAPS relies on thrombosis of at least three different organs systems over 1 week, histopathological evidence of small vessel occlusion, and high aPL antibody titers. In a subset of precipitating circumstances, activation or disruption of endothelial cells in the microvasculature may occur along with cardiomyopathy. We present two cases of CAPS‐associated dilated cardiomyopathy at our institution, focusing on disease management, pathophysiology, and treatment. These patients were of Southeastern Asian descent, raising the possibility of genetic polymorphisms contributing to the development of cardiomyopathy. Both met CAPS criteria and both demonstrated clinicopathologic thrombotic microangiopathy (TMA) and complement activation and developed severe dilated cardiomyopathy with shock. Complement activation plays an important role in the development of CAPS and may be important in the pathogenesis of CAPS‐associated cardiomyopathy. Clinical suspicion for TMA as a pathophysiologic mechanism of unexplained heart failure in CAPS is important and increased awareness of cardiac side effects is necessary so that early treatment can be initiated to halt further cardiac and systemic complications.https://doi.org/10.1002/jha2.71atypical hemolytic uremic syndromecardiomyopathycatastrophic antiphospholipid antibody syndromecomplementthrombotic microangiopathy |
| spellingShingle | Melody Hermel David Hermel Saif Azam Jerold Shinbane Annahita Sarcon Erika Jones Arjun Mehta Luanda Grazette Howard Liebman Ilene Weitz Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review eJHaem atypical hemolytic uremic syndrome cardiomyopathy catastrophic antiphospholipid antibody syndrome complement thrombotic microangiopathy |
| title | Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review |
| title_full | Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review |
| title_fullStr | Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review |
| title_full_unstemmed | Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review |
| title_short | Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review |
| title_sort | acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy a case series and review |
| topic | atypical hemolytic uremic syndrome cardiomyopathy catastrophic antiphospholipid antibody syndrome complement thrombotic microangiopathy |
| url | https://doi.org/10.1002/jha2.71 |
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