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.

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 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.

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%.

Thrombotic microangiopathy in the course of catastrophic antiphospholipid syndrome successfully treated with eculizumab: case report and systematic review of the literature

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 631-639 ◽  
Author(s):  
Marta Skoczynska ◽  
Mark A Crowther ◽  
Malgorzata Chowaniec ◽  
Monika Ponikowska ◽  
Shruti Chaturvedi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Single Case ◽  
Case Reports ◽  
Review Of The Literature ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Total Remission

Objective The purpose of this study was to characterize the role of eculizumab, a monoclonal antibody against the terminal complement component C5, in patients with catastrophic antiphospholipid syndrome (CAPS). Methods We present a case report of a patient with systemic lupus erythematosus (SLE) and CAPS treated with eculizumab, as well as results of a systematic review of the literature. Results Including our patient, we identified 11 case reports of patients with CAPS treated with eculizumab. All of them had partial or total remission of symptoms. Conclusion Data on eculizumab efficacy in CAPS are promising but are limited to single case reports. More studies are needed to develop evidence-based recommendations for eculizumab use in CAPS.

scholarly journals Iatrogenic Spinal Subdural Hematoma due to Apixaban: A Case Report and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Alba Colell ◽  
Adrià Arboix ◽  
Francesco Caiazzo ◽  
Elisenda Grivé
Keyword(s):  
Atrial Fibrillation ◽  
Case Report ◽  
Subdural Hematoma ◽  
Lower Risk ◽  
Unusual Case ◽  
Oral Anticoagulants ◽  
Review Of The Literature ◽  
Bleeding Events ◽  
Spinal Subdural Hematoma ◽  
Life Threatening

In the last decade, the clinical relevance for developing safer oral anticoagulants prompted the development of new classes of drugs that have shown a lower risk of life-threatening bleeding events as compared to standard warfarin. Nontraumatic spinal subdural hematoma is an uncommon urgent complication that can be associated with the use of these agents. An unusual case of spinal subdural hematoma related to apixaban treatment for nonrheumatic atrial fibrillation is reported here.

scholarly journals Spontaneous retroperitoneal hematoma in a COVID-19 patient

2021 ◽  
Vol 2 (4) ◽  
pp. 377-385
Author(s):  
Ana Sekulić ◽  
Olivera Marinković ◽  
Davor Mrda ◽  
Borislav Tošković ◽  
Marija Zdravković ◽  
...  
Keyword(s):  
Risk Factors ◽  
Case Report ◽  
Mortality Rate ◽  
Anticoagulant Therapy ◽  
Psoas Muscle ◽  
Bleeding Complications ◽  
Thromboembolic Events ◽  
Life Threatening ◽  
The Right ◽  
Radiological Examinations

Introduction: The infection caused by the SARS-CoV-2 virus is known to cause a hypercoagulable condition resulting in acute thrombotic events. Thromboembolic events occur in as many as 21.0% of cases with a mortality rate of about 74.0% in persons infected with COVID-19. Anticoagulant therapy is used in severe COVID-19 infections in order to prevent thrombosis and has been shown to reduce mortality. The use of anticoagulants is not without risks. Bleeding complications can range from mild to severe or even life-threatening, such as retroperitoneal bleeding into the psoas muscle. Case report: We present a case of a patient who developed a complication of bleeding into the retroperitoneal space during the treatment of bilateral bronchopneumonia caused by the SARS-CoV-2 virus. After the diagnosis was established, on the basis of a clinical examination, laboratory and radiological examinations, and after initial conservative treatment at the UHMC Bežanijska kosa, the patient underwent embolization of the left lumbar arteries from the right inguinal fossa, during procedural analgosedation. After the radiological procedure, the recovery was satisfactory, but due to the impossibility of resorption of an encapsulated hematoma with a zone of central necrosis, a mini left lumbotomy and evacuation of the hematoma were performed. The patient was discharged from the hospital on the 23rd day of admission in stable general condition. Conclusion: The effect of anticoagulant therapy, especially in patients with existing risk factors, early diagnosis, and prompt therapy of spontaneous retroperitoneal hematomas is imperative to reduce mortality from this severe complication, in patients with the COVID-19 infection.

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