Insights into the pathogenesis of catastrophic antiphospholipid syndrome. A case report of relapsing catastrophic antiphospholipid syndrome and review of the literature on ischemic colitis

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.

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

Lupus ◽

10.1177/09612033211004728 ◽

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.

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Eculizumab in refractory catastrophic antiphospholipid syndrome: a case report and systematic review of the literature

Clinical and Experimental Medicine ◽

10.1007/s10238-019-00565-8 ◽

2019 ◽

Vol 19 (3) ◽

pp. 281-288 ◽

Cited By ~ 12

Author(s):

Maria Giulia Tinti ◽

Vincenzo Carnevale ◽

Michele Inglese ◽

Francesca Molinaro ◽

Marco Bernal ◽

...

Keyword(s):

Systematic Review ◽

Case Report ◽

Antiphospholipid Syndrome ◽

Review Of The Literature ◽

Catastrophic Antiphospholipid Syndrome

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Catastrophic antiphospholipid syndrome with bilateral adrenal hemorrhage: a case report

Endocrine Abstracts ◽

10.1530/endoabs.64.027 ◽

2019 ◽

Author(s):

De Marchi Lucrezia ◽

M K de Filette Jeroen ◽

Sol Bastiaan ◽

E Andreescu Corina ◽

Kunda Rastislav ◽

...

Keyword(s):

Case Report ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Catastrophic Antiphospholipid Syndrome ◽

Bilateral Adrenal Hemorrhage

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Recovery from catastrophic antiphospholipid syndrome by a plasma exchange procedure: report of four cases and review of the literature

Autoimmunity Reviews ◽

10.1016/j.autrev.2008.09.002 ◽

2009 ◽

Vol 8 (4) ◽

pp. 297-301 ◽

Cited By ~ 25

Author(s):

Maria Bortolati ◽

Piero Marson ◽

Fabrizio Fabris ◽

Vittorio Pengo ◽

Myriam Facchinetti ◽

...

Keyword(s):

Plasma Exchange ◽

Antiphospholipid Syndrome ◽

Review Of The Literature ◽

Catastrophic Antiphospholipid Syndrome

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210 Acute Renal Failure by Massive IVC Thromboses Treated With Mechanical Thrombectomy: A Case Report of Atypical Catastrophic Antiphospholipid Syndrome

American Journal of Kidney Diseases ◽

10.1053/j.ajkd.2021.02.215 ◽

2021 ◽

Vol 77 (4) ◽

pp. 633

Keyword(s):

Renal Failure ◽

Acute Renal Failure ◽

Case Report ◽

Antiphospholipid Syndrome ◽

Mechanical Thrombectomy ◽

Catastrophic Antiphospholipid Syndrome

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

Vascular and Endovascular Surgery ◽

10.1177/15385744211046245 ◽

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.

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