Zhang Yang: Antiphospholipid syndrome and cardiovascular disease

A young woman, who had a deep vein thrombosis three years ago and was recently admitted to hospital with acute myocardial infarction, was the cause of repeated venous thrombosis in such a young woman? A young woman, repeatedly venous embolism, this diagnosis of cardiac valve disease, pre-surgery valve replacement surgery.
, after the "correction test", the patient's active part of the clotting enzyme time (APTT) is still significantly extended, is the clotting factor defect or what other causes? In clinical, many autoimmune diseases have heart and other organs affected, cardiovascular disease is the most common clinical manifestations, for cardiovascular patients the diagnosis and exclusion of primary disease is very important;
At the 11th Straits Cardiovascular Summit Symposium (CSCSF 2020) "Test Forum" held recently, Professor Zhang Yang of the Hospital of the Chinese Academy of Medical Sciences, combined with a large number of evidence-based medical evidence and multiple cases, vividly and systematically expounded the relationship between antiphospholipid syndrome (APS) and cardiovascular disease and the clinical thinking perspective for different situations. the definition and diagnosis of
antiphospholipid syndrome (APS) refers to an autoimmune disease caused by antiphospholipid antibody (aPL), with recurrent venous thrombosis, spontaneous abortion, plate reduction and serum aPL positive as the main clinical characteristics.
aPL is a family of heterogeneous antibodies that can react with the autoprotein protosexual reaction of phospholipids, which can lead to thrombosis by interfering with the function of various clotting and anticoagulant factors that rely on phospholipids, including anti-heart phospholipid antibodies (aCL), lupus anticoagulants (LA), and anti-beta-2 glycoprotein I.
APS can not only cause venous thrombosis and pathological pregnancy, but also lead to serious cardiovascular lesions.
the 2006 Sapporo Standard Sydney Revision, the diagnostic APS is required to meet "at least 1 clinical symptom plus 1 laboratory indicator."
(1) Clinical symptoms: 1. Thrombosis: more than one movement, static or small blood vessel thrombosis in any organ or tissue; 2. Loss of pregnancy: at least one unexplained, normal-form fetal death at least 10 weeks; or three or more unexplained early spontaneous abortions (lt;10 weeks); or≤34 weeks, eclampsia, pre-eclampsia, or placental insecurity.
(2) Laboratory indicator: 12 ≥ intervals and ≥ 2 detections.
1. Plasma LA positive; 2. medium→ high tiry IgG/M class ACL (≥40 units); 3. IgG/M class anti-beta 2-GP1 (≥40 units).
Antiphospholipid syndrome and cardiovascular disease APS are divided into primary and secondary two categories, the former has no cause, the latter secondary to a variety of diseases, mainly connective tissue diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and a variety of infections, tumors, the application of certain drugs.
In recent years, APS patients with cardiovascular diseases have attracted much attention, including heart valve lesions, coronary artery lesions, pulmonary hypertension, etc., often due to complex symptoms, lack of understanding leading to misdiagnosis or missed diagnosis.
clinicians should fully understand the performance of APS cardiovascular system and understand the corresponding treatment strategies.
APS and valve disease heart valve disease are the most common heart manifestations in APS patients, including wart-like dwelling, valve thickening, fiber calcification, etc., and can be accompanied by cardiomyopathy, biceps or aortic valve reflow, may be related to the secondary inflammatory response of thrombosis deposition.
studies have shown that 15-30% of APS patients suffer from valve heart disease, known as Libman-Sacks endoarthritis or non-bacterial thrombosis endoarthritis.
Clinically, clinicians should be cautious about young patients with valve disease with repeated venous thrombosis, and if the patient's APTT is significantly prolonged and does not return to normal after correcting the test, the antiphospholipid syndrome antibody spectrum should be actively tested to identify the cause, so as to adjust the treatment management plan before, during and after surgery.
, it should be noted that for APS patients with heart valve disease, the risk of thrombosis and bleeding during surgical valve replacement surgery increased significantly.
, infectious endoarthritis (IE) is also associated with aPL as a common infectious disease in the circulatory system.
study, published in the journal HEART in 2018 to explore the relationship between aPL and thrombosis events in IE patients, showed that the risk of cerebral embolism in the aCL-lgM-positive group was significantly higher than in the aCL-lgM-negative group.
the occurrence of cerebral embolism in the beta2GP1-lgM-positive group was significantly higher than in the negative group of beta2GP1-lgM, and the regression analysis showed that beta-2GP1-lgM and aCL-lgM were two independent risk factors for cerebral embolism in IE patients.
APS and pulmonary arterial embolism, pulmonary arterial hypertension APS venous thrombosis is more common than arterial thrombosis, venous thrombosis and deep vein thrombosis (DVT) are most common.
study showed that APS increased the risk of pulmonary embolism (PE) recurrence by 2.3-8.5 times.
a 2019 study published in the journal Twitter Respir Dis to compare differences in clinical characteristics between APS-positive, APS-negative PE patients showed that APS-positive PE patients had a younger age and a higher history of pathological pregnancy and arterial embolism.
In clinical trials, for PE with no induced hemolysis, if the age is 40 years old, the pulmonary embolism severity index (PESI) low risk (risk level iii or II) and/or APTT extension (above 75 percent of the reference interval), apS may be suspected, the aPL laboratory test should be actively carried out to find the cause, especially for young PE patients.
pulmonary embolism is the leading cause of chronic embolism pulmonary hypertension (CTEPH) in APS patients.
study, published in the journal Tromb Haemost, showed that 7.7% of overall CTEPH patients were APS (23/297); In PH patients, 43% (10/23) suffered from other autoimmune diseases, the vast majority of which were systemic lupus erythematosus (8/10);
APS is an important sub-group of CTEPH, with unique clinical symptoms and hemodynamic characteristics.
for PE patients, early detection of aPL, active eynamic diagnosis, appropriate anticoagulant treatment, limit PE further progression to CTEPH.
of APS and myocardial infarction, APS is less common with acute myocardial infarction (AMI).
most scholars believe that APS occurs often due to thrombosis in coronary arteries, especially in young APS patients.
a meta-analysis of 27 studies conducted in 2017 showed that 33 of the 40 APS patients who combined AMI had primary APS and 4 had secondary APS±
A study aimed at analyzing the clinical characteristics of aCL-positive AMI patients in former hospitals showed that the aCL-positive group was younger than the aCL-negative group;
same time, a subgroup analysis of people aged 55 and older showed an increased risk of developing venous thrombosis in the antiphospholipid-positive group of low-age AMI patients.
, AMI can occur in normal APS patients, especially women who are not menotinal or who do not have a risk factor for traditional atherosclerosis.
APS should be considered when young patients without coronary heart disease risk factors have thrombosis or thrombosis coronary artery closure, and angiosis has no evidence of atherosclerosis.
high-titration aCL and aPL increase the risk of heart events in normal coronary arteries, as well as the risk of vascular closure in patients with coronary bypass transplantation (CABG) and after coronary artery interventional therapy (PCI).
a forward-looking study published in the journal CIRCULATION in 2000 to explore the risk of recurrence of antiphospholipid antibodies and cardiovascular events showed that patients with high Acl-lgG and low lgM antibodies were at the highest risk.
A 1998 study published in the journal Autoimmunity showed that the aPL-negative stenosis rate was 3/45 (15%), the aPL-positive group was 6/15 (40%), and the difference was statistically significant;
a 2012 study published in Cardiology to explore the long-term prognostication of PCI postoperative APS patients showed a significant increase in major adverse cardiovascular events (MACEs) in patients with PCI treatment for 6 months, 1 year, 2 years, and 3 years compared to patients with PCI-treated ST-stage elevated AMI.
studies have shown that the possible mechanisms for cardiovascular disease due to antiphospholipid syndrome are: aPL-mediated endotrophic nitric oxide inhibition, no production and release impairment, and endothormic dysfunction - "first blow"; Second blow"; aPL-mediated endothragm cell proliferation, endometrial hyperplusing and non-atherosclerotic vascular stenosis; APS-related atherosclerosis acceleration; aPL-mediated plateocyte activation, aggregation and thrombosis; complement system activation and thrombosis.
APS and in-heart thrombosis are rare in APS patients and are often reported on a case-by-case basis.
, however, it is important and potentially fatal.
many systemic manifestations caused by thrombosis in the heart cavity include deep vein thrombosis, pulmonary embolism, stroke, congestive heart failure, peripheral artery ischemic, multiarterial or venous embolism, and some patients may have no clinical symptoms.
clinically, aPL should be actively screened for young patients with in-cardiac thrombosis without heart-based diseases.
total heart is one of the important target organs of APS, clinically about 40% of APS patients have cardiovascular system performance, understanding the relevant conditions can improve the level of diagnosis and treatment of such diseases.
Given the close relationship between APS and cardiovascular disease, it is necessary for clinicians to screen antiphospholipid syndrome-related antibodies for some unexplained cardiovascular diseases in order to identify the cause and provide a basis for clinical diagnosis and treatment.
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