Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy

Tulai, Iulia; Penciu, Oana; Raut, Raymond; Rudinskaya, Alla

doi:10.14503/thij-17-6472

Cited by 8 publications

(8 citation statements)

References 7 publications

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“…Nevertheless, patients with C-APLAS should be given a triple therapy associating anticoagulation, high-dose corticosteroids, and either IVIG or plasma exchange ( 70 ). Rituximab has been also frequently used in combination with plasma exchange in C-APLAS with myocarditis ( 67 , 71 , 72 ). In refractory cases, the use of complement inhibitors (i.e., eculizumab) can be discussed on a case-by-case basis ( 73 ).…”

Section: Specific Subset Of Myocarditismentioning

confidence: 99%

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

et al. 2022

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The field of inflammatory disease of the heart or “cardio-immunology” is rapidly evolving due to the wider use of non-invasive diagnostic tools able to detect and monitor myocardial inflammation. In acute myocarditis, recent data on the use of immunomodulating therapies have been reported both in the setting of systemic autoimmune disorders and in the setting of isolated forms, especially in patients with specific histology (e.g., eosinophilic myocarditis) or with an arrhythmicburden. A role for immunosuppressive therapies has been also shown in severe cases of coronavirus disease 2019 (COVID-19), a condition that can be associated with cardiac injury and acute myocarditis. Furthermore, ongoing clinical trials are assessing the role of high dosage methylprednisolone in the context of acute myocarditis complicated by heart failure or fulminant presentation or the role of anakinra to treat patients with acute myocarditis excluding patients with hemodynamically unstable conditions. In addition, the explosion of immune-mediated therapies in oncology has introduced new pathophysiological entities, such as immune-checkpoint inhibitor-associated myocarditis and new basic research models to understand the interaction between the cardiac and immune systems. Here we provide a broad overview of evolving areas in cardio-immunology. We summarize the use of new imaging tools in combination with endomyocardial biopsy and laboratory parameters such as high sensitivity troponin to monitor the response to immunomodulating therapies based on recent evidence and clinical experience. Concerning pericarditis, the normal composition of pericardial fluid has been recently elucidated, allowing to assess the actual presence of inflammation; indeed, normal pericardial fluid is rich in nucleated cells, protein, albumin, LDH, at levels consistent with inflammatory exudates in other biological fluids. Importantly, recent findings showed how innate immunity plays a pivotal role in the pathogenesis of recurrent pericarditis with raised C-reactive protein, with inflammasome and IL-1 overproduction as drivers for systemic inflammatory response. In the era of tailored medicine, anti-IL-1 agents such as anakinra and rilonacept have been demonstrated highly effective in patients with recurrent pericarditis associated with an inflammatory phenotype.

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Section: Specific Subset Of Myocarditismentioning

confidence: 99%

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

et al. 2022

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“…Target organ biopsy (renal in our case) showing ischemic microvascular injury of the kidneys, myocardium, or skin, as described previously, might have provided valuable insight into the underlying diagnosis. 6 , 12 , 14 , 15 However, the risks of withholding anticoagulation for biopsy after development of a splenic infarct was deemed too great. While large vessel vasculitis was considered, given the finding of aortitis, involvement of the glomerulus made this less likely.…”

Section: Discussionmentioning

confidence: 99%

Catastrophic antiphospholipid syndrome: challenging case and importance of multidisciplinary evaluation and management

Sorathia,

Arockiam,

Haroun

et al. 2024

Clinical Case Reports

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“…Regarding the etiology of CAPS-related acute cardiac failure, a number of different causes have been described, including macrovascular ( 4 ) or microvascular coronary artery embolization ( 5 ), valve involvement ( 6 ), fulminant myocarditis ( 7 ), and severe hypoxemia. In the present case, we failed to confirm the presence of microvascular coronary artery embolization or myocarditis since a cardiac biopsy or gadolinium-enhanced cardiac MRI could not be performed due to the patient's severe thrombocytopenia and kidney dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Sudden Cardiac Arrest as the First Manifestation in a Patient with Catastrophic Antiphospholipid Syndrome

et al. 2020

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We herein report a 26-year-old woman with sudden cardiac arrest who had no remarkable medical history. While resuscitation was successfully performed with adrenalin administration and extracorporeal membrane oxygenation, the cause of cardiac arrest could not be determined for over two weeks. Given the presence of autoimmune disease along with the findings of refractory renal insufficiency and thrombocytopenia, a kidney biopsy and blood examinations, including lupus anticoagulant testing, were performed, which proved the presence of antiphospholipid syndrome. The patient was successfully treated with steroid pulse therapy. This drastic case scenario highlighted the fact that autoimmune disease can be the cause of sudden cardiac arrest.

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Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy

Cited by 8 publications

References 7 publications

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

Catastrophic antiphospholipid syndrome: challenging case and importance of multidisciplinary evaluation and management

Sudden Cardiac Arrest as the First Manifestation in a Patient with Catastrophic Antiphospholipid Syndrome

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