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.

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 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 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 Pharmacokinetics and Safety of Intravenous Cidofovir for Life-Threatening Viral Infections in Pediatric Hematopoietic Stem Cell Transplant Recipients

2015 ◽  
Vol 59 (7) ◽  
pp. 3718-3725 ◽  
Author(s):  
Amy E. Caruso Brown ◽  
Mindy N. Cohen ◽  
Suhong Tong ◽  
Rebecca S. Braverman ◽  
James F. Rooney ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hematopoietic Stem Cell ◽  
Half Life ◽  
Viral Infections ◽  
Multiorgan Failure ◽  
Kidney Injury ◽  
Cell Transplant ◽  
Open Label ◽  
Hematopoietic Stem ◽  
Life Threatening

ABSTRACTChildren undergoing hematopoietic stem cell transplantation (HSCT) are at risk for life-threatening viral infections. Cidofovir is often used as a first-line agent for adenovirus infections, despite the absence of randomized controlled trials with HSCT patients, and as a second-line agent for resistant herpesvirus infections. The frequency and severity of adverse effects, particularly nephrotoxicity, in pediatric HSCT recipients are unclear, and pharmacokinetics (PK) of cidofovir in children have not previously been reported. This study was an open-label, nonrandomized, single-dose pilot study to determine the safety and PK of cidofovir in pediatric HSCT recipients with symptomatic adenovirus, nucleoside-resistant cytomegalovirus (CMV) or herpes simplex virus (HSV), and/or human papovavirus infections. Subsequent dosing and frequency were determined by clinical response and side effects, as assessed by the treating physician. Blood and urine samples were obtained from patients for PK studies and assessment of toxicity and virologic response. Twelve patients were enrolled (median age, 9 years; 33.5 days posttransplantation). Four of seven patients with adenovirus infection were successfully treated and eventually cleared their infections. Four of twelve patients died of disseminated viral disease and multiorgan failure. Two of twelve patients had evidence of acute kidney injury after the first dose, and one of these patients developed chronic kidney disease; two other patients developed late nephrotoxicity. The mean drug half-life was 9.5 h. There was no correlation between nephrotoxicity and plasma maximum concentration, clearance, or half-life. PK were similar to those reported for adults, although the drug half-life was significantly longer than that for adults. Cidofovir was well tolerated in the majority of patients. However, effective therapeutic strategies are urgently needed to support patients until immune reconstitution is achieved.

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.

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.

scholarly journals Necrotising Myositis, the Deadly Impersonator

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
A. Rahman ◽  
A. K. Abou-Foul ◽  
A. Yusaf ◽  
J. Holton ◽  
L. Cogswell
Keyword(s):  
Multiorgan Failure ◽  
Pharmacological Intervention ◽  
Risk Indicator ◽  
Limb Pain ◽  
Soft Tissue Necrosis ◽  
Lower Limb Pain ◽  
Life Threatening ◽  
Group A ◽  
Extensive Debridement ◽  
The Right

We report two cases of patients with necrotising myositis who presented initially with limb pain and swelling on a background of respiratory complaints. Patient 1, a previously well 38-year-old female, underwent various investigations in the emergency department for excessive lower limb pain and a skin rash. Patient 2, a 61-year-old female with a background of rheumatoid arthritis and hypertension, presented to accident and emergency feeling generally unwell and was treated for presumed respiratory sepsis. Both deteriorated rapidly and were referred to the plastic surgery team with soft tissue necrosis, impending multiorgan failure and toxaemia. Large areas of necrotic muscle and skin were debrided, which grew group A streptococci,Streptococcus pyogenes. Patient 1 had a high above knee amputation of the left leg with extensive debridement of the right. Despite aggressive surgical intervention and microbiological input with intensive care support, patient 2 died. These two cases highlight the importance of early diagnosis and prompt surgical and pharmacological intervention in managing this life-threatening disease. Pain is the primary symptom with skin changes being a late and subtle sign in a septic patient. The Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC) may be of use if there is concern to aid diagnosis of this life-threatening disease.

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