There are 5 types of APS-related cardiovascular diseases!

*For medical professionals to read and refer to the relevant knowledge of APS and cardiovascular diseases.
In the 2021 Annual Meeting of Rheumatology and Immunology Branch of the Chinese Medical Doctor Association, Professor Zhou Zhou from the National Center for Cardiovascular Diseases/Fuwai Hospital of the Chinese Academy of Medical Sciences explained to us Antiphospholipid syndrome (APS) and cardiovascular disease related knowledge, this article organizes the wonderful content of this speech, and shares the academic feast with you! Cardiovascular-related autoimmune testing In clinical work, we have found that many autoimmune diseases involve the heart and other organs.
Cardiovascular disease is one of the common clinical manifestations, such as pericarditis, myocarditis, atherosclerosis, venous thrombosis, Pulmonary hypertension, pulmonary embolism, etc.
are very important for the diagnosis and elimination of the primary disease in patients with cardiovascular disease
.

Therefore, it is necessary to carry out relevant detection of autoantibody profile (see Figure 1)
.

Figure 1.
Cardiovascular-related autoimmune detection.
Antiphospholipid antibodies (aPL) are a group of heterogeneous antibody families that react with phospholipid-bound auto-protein epitopes.
They mainly include anticardiolipin antibodies, lupus anticoagulants and anti-β2- GP1 antibody, etc.
(see Figure 2)
.

Figure 2.
Definition and scope of aPL APS and cardiovascular disease APS is an autoimmune disease caused by aPL
.

The heart is one of the important target organs of APS, and about 40% of patients have cardiovascular system manifestations
.

▍1.
Classification of APS-related cardiovascular diseases: APS and heart valve disease: neoplasms, valve thickening, fibrous calcification; APS and myocardial infarction: premature myocardial infarction without risk factors for coronary heart disease; APS and coronary artery bypass grafting Surgery (CABG), Percutaneous Coronary Intervention (PCI): Increase the risk of transplanted vessel occlusion and restenosis after PCI; APS and intracardiac thrombosis: Young patients with intracardiac thrombosis but no underlying heart disease ; APS and deep vein thrombosis: the cause of about 20% of patients with deep vein thrombosis
.

▍2.
APS and heart valve disease 15%~30% of APS patients suffer from valvular heart disease, namely Libman-Sacks endocarditis or non-bacterial thrombotic endocarditis
.

Heart valve disease is the most common cardiac manifestation in patients with APS, including verrucous neoplasms, valve thickening, fibrous calcification, etc.
, and can be accompanied by cardiomyopathy, mitral valve or aortic valve regurgitation, and may be associated with inflammation secondary to thrombus deposition The response is related (see Figure 3)
.

Figure 3.
APS and heart valve disease Professor Zhou Zhou mentioned that in clinical work, while solving the patient's valve disease, we must pay attention to the diagnosis of etiology, and briefly introduced a case (see Figure 4): Clinical problem: young patients , Repeated arterial and venous thrombosis, poor prognosis, high incidence of malignant events, need to find the cause, and adjust the treatment plan according to the cause
.

Solution: APS antibody profile detection, clear etiological diagnosis
.

In this case, the detection of aPL was obviously abnormal.
After the perfect operation, the pathology revealed non-infectious thrombotic neoplasms, and the treatment of immuno-inflammatory drugs was needed to prevent recurrence or involvement of other valves in the disease
.

Figure 4.
Heart valve disease case ▍3.
APS and chronic thromboembolic pulmonary hypertension APS-positive chronic thromboembolic pulmonary hypertension (CTEPH) patients are younger, which is related to aPL positive and a history of repeated thrombosis
.

In CTEPH, 7.
7% aPL was positive
.

Among APS, 43% have other autoimmune diseases, most of which are systemic lupus erythematosus
.

APS is an important subgroup of CTEPH, with unique clinical symptoms and hemodynamic characteristics
.

The detection of aPL in patients with pulmonary embolism (PE) and long-term anticoagulation therapy may limit their progression to CTEPH (see Figure 5)
.

Figure 5.
APS and CTEPH▍4.
Acute myocardial infarction and APS There are related literatures about arterial thrombotic events such as acute myocardial infarction and APS, but most of them are case reports
.

A retrospective analysis included 40 patients with acute myocardial infarction, including 33 primary APS, 4 secondary, and 3 catastrophic.
The average age was 41.
10±13.
61 years old.
There was no obvious gender advantage (55% male, 45 % Female), 80% of the patients’ first presentation was acute myocardial infarction
.

In addition, Professor Zhou Zhou also briefly explained a case of a patient with arteriovenous thrombosis (see Figure 6): a young woman was admitted to the hospital for diagnosis of acute extensive anterior wall myocardial infarction, and had a history of venous thrombosis.
It is necessary to actively search for the cause and confirm the etiological diagnosis.
Adjust the treatment plan for the cause
.

The test results indicate that all three types of aPL are positive, suggesting a higher risk of thrombosis
.

Figure 6.
Cases of patients with arteriovenous thrombosis▍5.
The possible mechanisms of APS leading to cardiovascular disease mainly include the following six aspects (see Figure 7): (1) aPL-mediated activation, aggregation and thrombosis of platelet cells; (2) aPL-mediated endothelial NO synthase inhibition and NO production And release damage and endothelial dysfunction (first blow); (3) activation of the complement system and thrombosis; (4) acceleration of APS-related atherosclerosis; (5) β2-GP1 on endothelial cells after inflammation injury Body upregulation (second hit); (6) aPL-mediated endothelial cell proliferation, intimal hyperplasia and non-atherosclerotic vascular stenosis
.

Figure 7.
The possible mechanism of APS leading to cardiovascular disease▍6.
APS and intracardiac thrombosis Intracardiac thrombosis is rare in patients with APS, but it is very important and has a potentially fatal risk
.

Multiple system manifestations caused by intracardiac thrombosis include deep vein thrombosis, pulmonary embolism, stroke, congestive heart failure, peripheral arterial ischemia, and multi-arterial or venous embolism (see Figure 8)
.

Figure 8.
Summary of APS and intracardiac thrombosis.
Finally, Professor Zhou Zhou also summarized the pathophysiology and treatment of APS-related cardiovascular complications (see Figure 9): 1.
In terms of pathophysiology, patients with APS are prone to endothelium Dysfunction, accelerated endothelial proliferation and intimal hyperplasia, atherosclerosis formation, platelet activation, etc.
; 2.
Cardiovascular complications include accelerated atherosclerosis, acute coronary syndrome, Libmann-Sacks endocarditis , Cardiomyopathy, arteriovenous thrombosis, etc.
; 3.
In terms of treatment, pay attention to the treatment of traditional cardiovascular risk factors, anticoagulant therapy for patients with venous thrombosis, and dual or triple antithrombotic therapy for patients with arterial thrombosis may be beneficial.
The primary prevention of thrombosis in aPL-positive patients is still controversial
.

Figure 9.
Summary