scholarly journals 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Marianna Strakhan ◽  
Mariana Hurtado-Sbordoni ◽  
Nahun Galeas ◽  
Kamila Bakirhan ◽  
Karenza Alexis ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Treatment Options ◽  
Immunoglobulin Therapy ◽  
High Rate ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Laboratory Findings ◽  
Intravenous Immunoglobulin Therapy ◽  
Short Period ◽  
Threatening Condition ◽  
Life Threatening

Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

Catastrophic antiphospholipid syndrome presenting with aortic barrage: case report and review of the literature

Lupus ◽  
2021 ◽  
Vol 30 (6) ◽  
pp. 1005-1009
Author(s):  
Luca Moroni ◽  
Paolo Righini ◽  
Giuseppe A Ramirez ◽  
Nicola Farina ◽  
Gaia Mancuso ◽  
...  
Keyword(s):  
Risk Factors ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Unusual Case ◽  
Thromboembolic Events ◽  
Review Of The Literature ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Short Period ◽  
Threatening Condition ◽  
Life Threatening

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition characterized by multiple thromboembolic events occurring in a short period of time, frequently accompanied by significant systemic inflammation. Aortic involvement is rare in antiphospholipid syndrome and it had been never described in the context of its catastrophic variant. Here, we report an unusual case of aortic occlusion as a debut manifestation of CAPS and discuss its clinical features with an up-to-date review of the literature to identify risk factors and clues for clinical practice.

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology

2018 ◽  
Vol 46 (4) ◽  
pp. 387-400 ◽  
Author(s):  
Alexander D. Makatsariya ◽  
Jamilya Khizroeva ◽  
Viktoriya O. Bitsadze
Keyword(s):  
Mortality Rate ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Multiorgan Failure ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
Threatening Condition ◽  
Life Threatening ◽  
Large Vessels

Abstract Background: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called “thrombotic storm” and multiple organ failure, that is, CAPS. Materials and methods: Since 2001–2016, we discovered 17 patients with CAPS development. Conclusion: CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%–40%.

scholarly journals Catastrophic Antiphospholipid Syndrome in Obstetric Practice

2012 ◽  
Vol 61 (3) ◽  
pp. 7-21 ◽  
Author(s):  
Alexander D Makatsariya ◽  
Viktoriya O Bitsadze ◽  
Dzhamilya Kh Khizroeva
Keyword(s):  
Antiphospholipid Syndrome ◽  
Molecular Mechanisms ◽  
Multiorgan Failure ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
First Case ◽  
Threatening Condition ◽  
Life Threatening ◽  
Fatal Form ◽  
High Level

Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, form of the antiphospholipid syndrome that results in a widespread coagulopathy and affects predominantly small vessels supplying organs with the development of multiorgan failure against a background of high level of antiphospholipid antibodies. Thrombotic microvasculopathy is the basis of multiorgan failure and clinically manifests with CNS disturbances, adrenal failure, and the development of acute respiratory distress syndrome. CAPS is a life-threatening condition and requires urgent measures. Optimal treatment for CAPS is not developed yet. CAPS present a multidisciplinary problem. Authors demonstrate 17 cases of CAPS in patients which were managed from 2001 to 2012 years. Molecular mechanisms of pathogenesis and different obstetric and non-obstetric manifestations of CAPS are discussed in the article. There is the description of first case of management of pregnancy and labor of patient with CAPS in her history. The methods of CAPS prevention are described

scholarly journals Catastrophic antiphospholipid syndrome in pregnancy: a life-threatening condition

2019 ◽  
Vol 12 (9) ◽  
pp. e230863 ◽  
Author(s):  
Mandy Collict ◽  
Whitney Sciberras Buhagiar ◽  
Cecilia Mercieca ◽  
John Thake
Keyword(s):  
Early Diagnosis ◽  
Antiphospholipid Syndrome ◽  
Multiorgan Failure ◽  
Fetal Outcome ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Threatening Condition ◽  
Life Threatening ◽  
Vessel Thrombosis ◽  
Clinical Emergency ◽  
In Pregnancy

Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially life-threatening variant of the antiphospholipid syndrome which is characterised by multiple small vessel thrombosis which can lead to multiorgan failure. CAPS is a clinical emergency which all clinicians need to be aware of because early diagnosis and treatment may improve maternal and fetal outcome. Here, we report a case of CAPS in pregnancy in a 31-year-old female patient who presented at 28 weeks of gestation. A literature review of CAPS in pregnancy and the puerperium is also included.

scholarly journals Intravenous immunoglobulin therapy for aspirin-heparinoid-resistant antiphospholipid syndrome

2010 ◽  
Vol 9 (4) ◽  
pp. 217-221 ◽  
Author(s):  
Shigeki Shimada ◽  
Hideto Yamada ◽  
Tatsuya Atsumi ◽  
Takashi Yamada ◽  
Noriaki Sakuragi ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Antiphospholipid Syndrome ◽  
Immunoglobulin Therapy ◽  
Intravenous Immunoglobulin Therapy

Catastrophic Antiphospholipid Syndrome Following Lower Extremity Arterial Bypass Surgery: Case Report and Evidence Review

2021 ◽  
pp. 153857442110462
Author(s):  
Ahmed A. Sorour ◽  
Levester Kirksey ◽  
Sarah Keller ◽  
Michael S. O’Connor ◽  
Sean P. Lyden
Keyword(s):  
Case Report ◽  
Lower Extremity ◽  
Antiphospholipid Syndrome ◽  
Current Literature ◽  
Bypass Surgery ◽  
Multidisciplinary Approach ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Diagnosis And Management ◽  
Life Threatening ◽  
Evidence Review

Catastrophic antiphospholipid syndrome (CAPS) is a rare life threatening presentation of antiphospholipid syndrome. Surgery has been proposed as one of the triggering factors for this life threatening entity. There are no detailed published reports in the current literature describing CAPS as a complication after surgery. We report a case of a 21 year old that developed CAPS postoperatively and discuss the multidisciplinary approach for diagnosis and management.

Bradyarrhythmias

2016 ◽  
Author(s):  
Inna Leybell ◽  
Liliya Abrukin
Keyword(s):  
Atrioventricular Block ◽  
Sinus Bradycardia ◽  
Sinus Node ◽  
Treatment Options ◽  
Presenting Symptoms ◽  
Threatening Condition ◽  
Node Disease ◽  
Life Threatening ◽  
Sinus Node Disease ◽  
Sinoatrial Block

Bradyarrhythmias can present as an incidental electrocardiographic (ECG) finding or a life-threatening condition requiring immediate intervention. They are caused by sinus node disease or atrioventricular block. This review covers pathophysiology, stabilization and assessment, diagnosis and treatment options, and disposition and outcomes for patients with bradycardia. Figures in the review demonstrate characteristic ECG tracings. Tables list classifications of sinus node dysfunction as well as of sinoatrial and atrioventricular blocks, presenting symptoms, important information to elicit on history taking, various causes of bradycardia, and specific interventions for toxicologic etiologies. Key words: atrioventricular block, atropine, bradyarrhythmia, bradycardia, first-degree atrioventricular block, second-degree atrioventricular block, sinoatrial block, sinus bradycardia, sinus node disease, tachycardia-bradycardia syndrome, third-degree atrioventricular block This review contains 9 highly rendered figures, 7 tables, and 92 references.

Antiphospholipid syndrome

2018 ◽  
pp. 375-388
Author(s):  
Gavin Clunie ◽  
Nick Wilkinson ◽  
Elena Nikiphorou ◽  
Deepak R. Jadon
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Presentation ◽  
Therapeutic Targets ◽  
Classification Criteria ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Laboratory Features ◽  
Life Threatening ◽  
Novel Therapeutic

This chapter describes the clinical and laboratory classification criteria for antiphospholipid syndrome. It highlights the complexity and diagnostic challenges of antiphospholipid syndrome and discusses potential mimics of the disease that need to be excluded. Current therapeutic targets as well as novel therapeutic avenues are explored. The chapter finishes with a section on catastrophic antiphospholipid syndrome, which constitutes a rheumatological emergency. The clinical presentation, laboratory features, treatment, and outcomes of this life-threatening presentation of antiphospholipid syndrome are also discussed.

scholarly journals Aortic Dissection in a Pregnant Patient without Other Risk Factors

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Priya V. Patel ◽  
Raanan Alter ◽  
Recia Frenn ◽  
Thaddeus P. Waters
Keyword(s):  
Risk Factors ◽  
Aortic Dissection ◽  
Aortic Rupture ◽  
Treatment Options ◽  
Pregnant Patient ◽  
Physical Exam ◽  
Vascular Events ◽  
Increased Risk ◽  
Threatening Condition ◽  
Life Threatening

Background. An aortic dissection is a life-threatening condition in which the intima of the artery tears causing separation of the intima and media. Pregnancy places women at a significantly increased risk of common vascular events including venous thromboembolism, myocardial infarction, and stroke, while also increasing the risk of rarer vascular events such as aortic dissection and aortic rupture. Case. A 30-year-old previously healthy multiparous woman presenting at 36 weeks of pregnancy with a Type A aortic dissection. She underwent a combined emergent cesarean delivery followed by repair of her aortic root. Conclusions. Aortic dissection should be high on the differential for pregnant patients presenting with the characteristic complaints and physical exam findings given the high mortality rate associated with this vascular event. Teaching Points. (1) This report reviews the characteristic presentation, risk factors, and physical exam findings in a patient with an aortic dissection. (2) The report includes treatment options for pregnant patients based on the classification of the dissection.

Dysphagia as an interdisciplinary problem. Difficulties in the diagnostic process of the vascular anomaly: a right-sided aortic arch with aberrant left subclavian artery (case report)

2018 ◽  
Vol 7 (1) ◽  
pp. 35-43
Author(s):  
Barbara Maciejewska ◽  
Karolina Kania ◽  
Piotr Kowal ◽  
Bożena Wiskirska-Woźnica
Keyword(s):  
Aortic Arch ◽  
Subclavian Artery ◽  
Left Subclavian Artery ◽  
Treatment Options ◽  
Vascular Anomaly ◽  
Differential Diagnostics ◽  
Diagnostic Process ◽  
Arteria Lusoria ◽  
Threatening Condition ◽  
Life Threatening

Dysphagia is an underestimated health problem. At the same time, it is a potentially life-threatening condition. Dysphagia in young adults is rare and thus it is rarely discussed in the literature. Vascular anomalies are much less frequent causes of dysphagia than structural pathologies of the digestive system, iatrogenic lesions or neurological causes. This paper presents a case of a 21-year-old woman with escalating dysphagia in the course of a congenital vascular anomaly in the form of a right-sided aortic arch with retroesophageal left subclavian artery (left arteria lusoria) and compression. The paper highlights delayed symptomatology of the congenital defect, reasons behind the long-lasting diagnostic process, and the role of the laryngological – phoniatric examination in order to exclude oral and pharyngeal causes. The differential diagnostics and treatment options are discussed thereinafter.

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