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.

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 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.

scholarly journals Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Young In Yun ◽  
Ji Hyun Kim ◽  
Seon Hee Lim ◽  
Yo Han Ahn ◽  
Hee Gyung Kang ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Thrombotic Microangiopathy ◽  
High Titer ◽  
Multiorgan Failure ◽  
Kidney Injury ◽  
Ocular Involvement ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Life Threatening ◽  
Systemic Examination ◽  
The Right

Abstract Background Ocular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of antiphospholipid syndrome (APS) that results in multiorgan failure and a high mortality rate, has rarely been reported. Case presentation A 15-year-old girl presented with sudden vision blurring in both eyes. She had marked optic disc swelling and macular exudates in the right eye and intra-arterial white plaques, a few retinal blot hemorrhages, and a white ischemic retina in the left eye. Systemic examination revealed she had acute kidney injury with thrombotic microangiopathy (TMA), multiple cerebral infarcts, valvular dysfunction, and a high titer of triple aPL. Thus, she was diagnosed with CAPS involving the brain, eyes, heart, and kidneys. Plasma exchange and the administration of glucocorticoids, immunoglobulin, warfarin, and rituximab brought a sustained recovery of the TMA, visual symptoms, and echocardiographic findings. Conclusions Ocular involvement of both vaso-occlusive retinopathy, an APS-related thrombotic microangiopathy, and neuroretinitis, a non-thrombotic microangiopathy, can occur as an initial presentation of CAPS.

scholarly journals A Case of Exertional Heat Stroke Complicated by Hypoxic Hepatitis

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Bertram K. Woitok ◽  
Shawki Bahmad ◽  
Gregor Lindner
Keyword(s):  
Supportive Care ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Multiorgan Failure ◽  
Heat Stroke ◽  
Exertional Heat Stroke ◽  
Hypoxic Hepatitis ◽  
Threatening Condition ◽  
Life Threatening ◽  
Delayed Referral

Background.Exertional heat stroke is a life-threatening condition often complicated by multiorgan failure. We hereby present a case of a 25-year-old male presenting with syncope after a 10  km run in 28°C outside temperature who developed acute liver failure. Case Presentation. Initial temperature was found to be 41.1°C, and cooling measures were rapidly applied. He suffered from acute renal failure and rhabdomyolysis and proceeded to acute liver failure (ASAT 6100 U/l and ALAT 6561 U/l) due to hypoxic hepatitis on day 3. He did not meet criteria for emergency liver transplantation and recovered on supportive care. Conclusions. Acute liver failure due to heat stroke is a life-threatening condition with often delayed onset, which nevertheless resolves on supportive care in the majority of cases; thus, a delayed referral to transplant seems to be reasonable.

Odontogenic deep neck space infection as life-threatening condition in pregnancy

2014 ◽  
Vol 59 (3) ◽  
pp. 375-378 ◽  
Author(s):  
D Dalla Torre ◽  
D Burtscher ◽  
D Höfer ◽  
FR Kloss
Keyword(s):  
Space Infection ◽  
Threatening Condition ◽  
Life Threatening ◽  
Deep Neck Space Infection ◽  
In Pregnancy

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.

scholarly journals A role for transoesophageal echocardiography in the early diagnosis of catastrophic antiphospholipid syndrome

2008 ◽  
Vol 248 (6) ◽  
pp. 519-524
Author(s):  
E. G. Zakynthinos ◽  
T. Vassilakopoulos ◽  
D. D. Kontogianni ◽  
C. Roussos ◽  
S. G. Zakynthinos
Keyword(s):  
Early Diagnosis ◽  
Antiphospholipid Syndrome ◽  
Transoesophageal Echocardiography ◽  
Catastrophic Antiphospholipid Syndrome

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.

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