Diagnostic Dilemma: Multiorgan Failure Due to Catastrophic Antiphospholipid Syndrome in a Young Woman With Six Healthy Children

CHEST Journal ◽  
2017 ◽  
Vol 152 (4) ◽  
pp. A278
Author(s):  
An Ho ◽  
Seth Koenig
Keyword(s):  
Young Woman ◽  
Antiphospholipid Syndrome ◽  
Multiorgan Failure ◽  
Healthy Children ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Diagnostic Dilemma

scholarly journals Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

2019 ◽  
Vol 6 (1) ◽  
pp. 13
Author(s):  
Carlos Anjo ◽  
Philip Fortuna ◽  
Luís Reis ◽  
Luís Bento
Keyword(s):  
Mortality Rate ◽  
Triple Therapy ◽  
Antiphospholipid Syndrome ◽  
Rare Complication ◽  
Multiorgan Failure ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Muscular Tissue ◽  
Lethal Outcome ◽  
Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS), an infrequent autoimmune disease, manifests as a widespread thrombotic disease with multiorgan failure with a 37% mortality rate. We report 39-year-old Caucasian women, medical history of Systemic Lupus Erythematous (SLE) complicated with APS and proliferative glomerulonephritis under dialysis. Patient was admitted at the ICU with septic shock with thoracotomy dehiscence origin. During the ICU stay, extensive lower limbs ulcers with polymorphonuclear infiltrate and steatonecrosis areas in adipose and muscular tissue. Lab studies: anti-β2-glycoprotein-Iantibody was detected. As probable CAPS diagnosis was made, triple therapy with methylprednisolone, immunoglobulin and anticoagulation immediately was started. Serious vasoplegic shock occurs with failure in vasopressor support increment and the patient died. The authors report an APS with rapid progression to catastrophic stage, rare complication that occurs only in 1% of catastrophic APS. In CAPS treatment, a statistical association between triple therapy and the decrease of mortality rate of patients with CAPS was observed, when compared with other combinations or with none of the treatments included in the triple therapy. Although the case had a lethal outcome, the authors emphasize the use of triple therapy in CAPS and the challenge of treating patients with CAPS and infection where the balance between the need for anticoagulation, aggressive immunosuppression and infection control is difficult to manage.

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 Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage

2019 ◽  
Vol 12 (3) ◽  
pp. e227171 ◽  
Author(s):  
Anju Adhikari ◽  
Mohammad Muhsin Chisti ◽  
Sanjog Bastola ◽  
Ojbindra KC
Keyword(s):  
Antiphospholipid Syndrome ◽  
Intracranial Haemorrhage ◽  
Clinical Course ◽  
Multiorgan Failure ◽  
Severe Form ◽  
High Dose ◽  
Cmv Infection ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Potential Trigger ◽  
Acute Cytomegalovirus

Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe form of antiphospholipid syndrome (APS). The syndrome manifests itself as a rapidly progressive multiorgan failure that is believed to be caused by widespread micro-thrombosis. Seldom does bleeding comanifest with thrombosis. We present a patient with APS who presented with nausea, vomiting and fatigue, and rapidly progressed into multiorgan failure before being diagnosed with CAPS. The clinical course was complicated by an atraumatic intracranial haemorrhage which demanded discontinuation of anticoagulation. The patient was treated with high dose steroid, intravenous immunoglobulin, followed by weekly rituximab infusion. Although the trigger for CAPS was not obvious during her hospital stay, she was diagnosed with acute cytomegalovirus (CMV) infection soon after discharge. In this case report, we explore the differential diagnoses of CAPS, investigate the possibility of CMV infection as a potential trigger, present the therapeutic challenges of anticoagulation and discuss the emerging use of rituximab.

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 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 Presenting With Multiorgan Failure and Gangrenous Lesions of the Skin

Angiology ◽  
2008 ◽  
Vol 59 (4) ◽  
pp. 517-518 ◽  
Author(s):  
Raffaele Antonelli Incalzi ◽  
Antonella Gemma ◽  
Leo Moro ◽  
Massimo Antonelli
Keyword(s):  
Antiphospholipid Syndrome ◽  
Multiorgan Failure ◽  
Catastrophic Antiphospholipid Syndrome
Sign in / Sign up

Export Citation Format

Share Document