Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome

Mustafa, Rafid

doi:10.1007/s11910-022-01228-0

Cited by 5 publications

(6 citation statements)

References 105 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Specifically, the clinical presentation of CAPS is characterized by kidney involvement in 73% of cases, with varying degrees of kidney dysfunction, and pulmonary involvement in 60% of cases, presenting as acute respiratory distress syndrome or pulmonary embolisms (26%) [ 37 ]. Up to 56% of patients exhibit cerebral manifestations, such as stroke or encephalopathy [ 10 , 38 ]. The heart is affected in half of cases, primarily as myocardial infarction or valvulopathy.…”

Section: Clinical and Biological Manifestations Of Capsmentioning

confidence: 99%

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Jacobs,

Wauters,

Lablad

et al. 2024

Antibodies

View full text Add to dashboard Cite

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes.

show abstract

Section: Clinical and Biological Manifestations Of Capsmentioning

confidence: 99%

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Jacobs,

Wauters,

Lablad

et al. 2024

Antibodies

View full text Add to dashboard Cite

show abstract

“…Antiphospholipid antibody syndrome is defined with both clinical criteria including pregnancy morbidity or early fetal loss, and venous or arterial ischemia as clinical criteria, along with laboratory criteria of anticardiolipin antibody and/or anti-β2 glycoprotein-I antibody presence on 2 sets of laboratory testing [ 154 , 155 ••]. The underlying pathophysiology is overall thought to be related to disruption of endogenous anticoagulant mechanisms resulting in a procoagulant state, and blood-brain barrier disruption resulting in antiphospholipid antibodies interacting with endothelial cells has been hypothesized to occur as well [ 156 ].…”

Section: Antiphospholipid Antibody Syndromementioning

confidence: 99%

“…Catastrophic APLS (CAPS) is defined as widespread thrombosis involving 3 different arterial and venous systems and associated with a high mortality rate of up to 50% [ 156 ]: this can be the initial clinical presentation ultimately leading to a diagnosis of APLAS. On the other end of the spectrum are those who meet diagnostic criteria for APLAS but have never suffered from arterial thrombosis.…”

Section: Antiphospholipid Antibody Syndromementioning

confidence: 99%

Advances in Management of the Stroke Etiology One-Percenters

Osteraas

Dafer

2023

Curr Neurol Neurosci Rep

View full text Add to dashboard Cite

Purpose of Review Uncommon causes of stroke merit specific attention; when clinicians have less common etiologies of stoke in mind, the diagnosis may come more easily. This is key, as optimal management will in many cases differs significantly from “standard” care. Recent Findings Randomized controlled trials (RCT) on the best medical therapy in the treatment of cervical artery dissection (CeAD) have demonstrated low rates of ischemia with both antiplatelet and vitamin K antagonism. RCT evidence supports the use of anticoagulation with vitamin K antagonism in “high-risk” patients with antiphospholipid antibody syndrome (APLAS), and there is new evidence supporting the utilization of direct oral anticoagulation in malignancy-associated thrombosis. Migraine with aura has been more conclusively linked not only with increased risk of ischemic and hemorrhagic stroke, but also with cardiovascular mortality. Recent literature has surprisingly not provided support the utilization of l -arginine in the treatment of patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); however, there is evidence at this time that support use of enzyme replacement in patients with Fabry disease. Additional triggers for reversible cerebral vasoconstriction syndrome (RCVS) have been identified, such as capsaicin. Imaging of cerebral blood vessel walls utilizing contrast-enhanced MRA is an emerging modality that may ultimately prove to be very useful in the evaluation of patients with uncommon causes of stroke. A plethora of associations between cerebrovascular disease and COVID-19 have been described. Where pertinent, authors provide additional tips and guidance. Summary Less commonly encountered conditions with updates in diagnosis, and management along with clinical tips are reviewed.

show abstract

“…Antiphospholipid syndrome is an autoimmune disorder that is associated with thrombosis and hypercoagulability in the presence of antiphospholipid antibodies (APLA) [4,5]. It is thought to predominantly affect younger individuals, especially younger women, though epidemiologic data is mixed and varies between cohorts [6,7].…”

Section: Introductionmentioning

confidence: 99%

“…The neurologic manifestations of APLS are diverse and are thought to have a relationship with recurrent ischemic stroke. However, these studies have been inconsistent, and criticisms arise from the heterogeneity of the cohorts [5,[11][12][13][14]. Specifically, APLS has not been well studied in the ESUS cohort.…”

Section: Introductionmentioning

confidence: 99%

Antiphospholipid Syndrome and Antibodies Associated With Malignancy and Older Age: A Retrospective Study

Liu,

Griffin,

Nair

et al. 2024

Cureus

View full text Add to dashboard Cite

Background: Antiphospholipid syndrome (APLS) is an established cause of thrombosis and hypercoagulability. However, the clinical characteristics of those with APLS or patients with positive antiphospholipid antibodies (APLA) in the embolic stroke of undetermined source (ESUS) have not been well studied.Methods: A retrospective analysis was conducted between January 1, 2010, and December 31, 2020, across all three Mayo Clinic sites. Patients who were included in the study were tested for APLA and had a diagnosis of ESUS. Baseline characteristics, radiographic parameters, and outcome data were collected and compared between those who tested positive for APLS or had positive APLA and those who were negative.Results: A total of 206 patients were included in the study. Eight (4%) patients were diagnosed with APLS, and 21 (10%) patients had positive APLA. On comparing those with a diagnosis of APLS and those without, patients with APLS were found to be significantly older (75 years old ± 9 vs. 58 years old ± 14, p = 0.001) and were more likely to have a history of cancer (50% vs. 13%, p = 0.012). Those with positive APLA had similar findings of being older (67 years old ±13 vs. 58 years old ± 14 p = 0.003) and more likely to have a history of cancer (29% vs. 8.4% p = 0.027). Radiographically, those with APLS had a higher white matter disease burden (Fazekas score median 2 (IQR 1.5-3) vs. median 1 (IQR 1-2), p = 0.028).Conclusion: Both APLS and positive APLA are associated with older age and a history of malignancy. These findings highlight the importance of considering a hypercoagulable evaluation even in the elderly ESUS population.

show abstract

Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome

Cited by 5 publications

References 105 publications

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Advances in Management of the Stroke Etiology One-Percenters

Antiphospholipid Syndrome and Antibodies Associated With Malignancy and Older Age: A Retrospective Study

Contact Info

Product

Resources

About