scholarly journals Fatal catastrophic antiphospholipid syndrome at presentation of juvenile lupus

2021 ◽  
pp. 489-490
Author(s):  
Debapoma Biswas ◽  
Saurabh Sutradhar ◽  
Argha Rajbanshi ◽  
Priyankar Pal
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Multiorgan Failure ◽  
Age Group ◽  
Multiple Site ◽  
Rare Form ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Pediatric Age Group ◽  
Pain Abdomen ◽  
Short Period

Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome, extremely uncommon inthe pediatric age group, characterized by multiple site thrombosis involving small, medium, and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We report the case of an 8-year-old girl presenting with fever, lymphadenopathy, and pain abdomen with refractory mitral regurgitation diagnosed as systemic lupus erythematosus with CAPS. All three antiphospholipid antibodies positivity in high titers further confirmed the diagnosis. In spite of the early initiation of adequate triple therapy (anticoagulation, steroids, and cyclophosphamide), our patient succumbed. The report aims to incorporate a greater awareness among clinicians for timely diagnosis and treatment of this condition and throws light on the varying ways in which lupus can present in children.

Aortitis in the setting of catastrophic antiphospholipid syndrome in a patient with systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (9) ◽  
pp. 1126-1129
Author(s):  
Andrés González-García ◽  
Mónica López-Rodríguez ◽  
Sandra Redondo ◽  
José Luis Patier ◽  
Pilar Masso ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Rare Condition ◽  
Intravenous Immunoglobulins ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Small Vessels ◽  
Short Period ◽  
Blue Toe Syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition characterized by multiple thromboses affecting mainly small vessels in a short period of time in patients with antiphospholipid antibodies. A high suspicion index is mandatory in order to initiate rapidly aggressive immunomodulatory therapy to avoid a very poor prognosis. Systemic lupus erythematosus (SLE) is often associated with antiphospholipid syndrome, with a worse outcome when the catastrophic features occur. We report the case of a 64-year-old woman with a clinical debut of SLE who presented concomitantly with CAPS with several thrombosis affecting the kidney, spleen and bilateral limbs with blue toe syndrome in both legs. Furthermore, she presented with aortitis, with a malaise and myalgias and general syndrome (asthenia, hyporexia and mild weight loss). Fortunately, she had a good response to multi-target combination therapy (anticoagulants, corticosteroids, hydroxychloroquine, intravenous immunoglobulins, plasma exchange and rituximab). Here, we discuss the association between aortitis and CAPS secondary to SLE, and review the literature regarding similar conditions.

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.

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 (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 Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus

2016 ◽  
Vol 68 (4) ◽  
pp. 1044-1044 ◽  
Author(s):  
Juan-Felipe Betancur ◽  
Erika Paola Navarro ◽  
José Humberto Bravo Bonilla ◽  
Armando Daniel Cortés ◽  
Juan Diego Vélez ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome
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