Materiały źródłowe

• #1 Antiphospholipid syndrome – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/antiphospholipid-syndrome/symptoms-causes/syc-20355831

  Antiphospholipid syndrome occurs when the immune system mistakenly produces antibodies that make blood much more likely to clot. Antiphospholipid syndrome can be caused by an underlying condition, such as an autoimmune disorder. […] You can also develop the syndrome without an underlying cause.

• #2

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/

  Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots. […] APS is an autoimmune condition. This means the immune system, which usually protects the body from infection and illness, attacks healthy tissue by mistake. […] In APS, the immune system produces abnormal antibodies called antiphospholipid antibodies. […] These target proteins attached to fat molecules (phospholipids), which makes the blood more likely to clot. […] It’s not known what causes the immune system to produce abnormal antibodies. […] As with other autoimmune conditions, genetic, hormonal and environmental factors are thought to play a part.

• #3 Blood Clotting Disorders – Antiphospholipid Syndrome (APS) | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is an autoimmune disorder that causes abnormal blood clots to form. […] The specific antibodies in APS are called antiphospholipids because they attack and damage parts of cells called phospholipids. […] Your family history and genes, other medical conditions, medicines and procedures, or lifestyle factors may raise your risk of APS. […] APS is most common in people who have lupus. In fact, 20% to 30% of people with lupus have antiphospholipid antibodies. […] Bacterial or viral infections: HIV, hepatitis C, and the bacteria that causes Lyme disease can increase your risk of making APS antibodies or trigger APS.

• #4 Antiphospholipid Syndrome: Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/21685-antiphospholipid-syndrome

  Antiphospholipid syndrome happens when your immune system accidentally starts making certain abnormal antibodies. […] Antiphospholipid syndrome gets its name from how your immune system attacks your body. If you have APS, your immune system makes antibodies that attack proteins bound to phospholipids (a type of fat cell). These attacks damage the phospholipids and make them more likely to clump together and form clots. […] The three antibodies that can cause APS include: Lupus anticoagulant, Anticardiolipin, Anti-B2 glycoprotein 1. […] Even though experts know they cause APS, they aren’t sure what triggers your immune system to start making these faulty antibodies.

• #5 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  As of yet, we dont know why people develop APS, why some patients go on to have blood clots while others dont, why some women (but not all) have pregnancy problems and why some patients are affected by symptoms more than others vital research is needed before we can answer these questions. […] However, we do know that the root cause is an over-activity of the immune system. […] In patients with APS, the body produces harmful antibodies called antiphospholipid antibodies (aPL). […] The most important of these proteins is called beta-2-glycoprotein1. […] Phospholipids contribute to blood clot formation and play an essential role in maintaining proper blood consistency. Due to the phospholipids being attacked by the antibodies, the blood becomes excessively 'sticky’, resulting in a higher risk of developing blood clots.

• #6 Antiphospholipid syndrome: Diagnosis – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-diagnosis

  Antiphospholipid syndrome (APS) is a clinical and laboratory diagnosis characterized by both persistent laboratory evidence of antiphospholipid antibodies (aPL) and related complications, which may include venous thrombosis, arterial thrombosis, adverse pregnancy outcomes, and nonthrombotic manifestations (eg, heart valve thickening, livedo reticularis/racemosa). APS occurs either as a primary condition or in the setting of an underlying disease, usually systemic lupus erythematosus (SLE). […] The diagnosis and classification criteria for APS are reviewed here. Additional topics discuss: […] Pathogenesis (see „Antiphospholipid syndrome: Pathogenesis”).

• #7 Antiphospholipid Syndrome: Symptoms, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/21685-antiphospholipid-syndrome

  Antiphospholipid syndrome happens when your immune system accidentally starts making certain abnormal antibodies. […] Antiphospholipid syndrome gets its name from how your immune system attacks your body. If you have APS, your immune system makes antibodies that attack proteins bound to phospholipids (a type of fat cell). These attacks damage the phospholipids and make them more likely to clump together and form clots. […] The three antibodies that can cause APS include: Lupus anticoagulant, Anticardiolipin, Anti-B2 glycoprotein 1. […] Even though experts know they cause APS, they aren’t sure what triggers your immune system to start making these faulty antibodies.

• #8 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #9 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  As of yet, we dont know why people develop APS, why some patients go on to have blood clots while others dont, why some women (but not all) have pregnancy problems and why some patients are affected by symptoms more than others vital research is needed before we can answer these questions. […] However, we do know that the root cause is an over-activity of the immune system. […] In patients with APS, the body produces harmful antibodies called antiphospholipid antibodies (aPL). […] The most important of these proteins is called beta-2-glycoprotein1. […] Phospholipids contribute to blood clot formation and play an essential role in maintaining proper blood consistency. Due to the phospholipids being attacked by the antibodies, the blood becomes excessively 'sticky’, resulting in a higher risk of developing blood clots.

• #10

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/

  Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots. […] APS is an autoimmune condition. This means the immune system, which usually protects the body from infection and illness, attacks healthy tissue by mistake. […] In APS, the immune system produces abnormal antibodies called antiphospholipid antibodies. […] These target proteins attached to fat molecules (phospholipids), which makes the blood more likely to clot. […] It’s not known what causes the immune system to produce abnormal antibodies. […] As with other autoimmune conditions, genetic, hormonal and environmental factors are thought to play a part.

• #11

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Antiphospholipid syndrome (APS) is caused by the body’s immune system producing abnormal antibodies called antiphospholipid antibodies. […] It’s not clear why these abnormal antibodies are produced, or why many people have antiphospholipid antibodies but don’t develop blood clots. […] A combination of genetic and environmental factors is thought to be responsible. […] Research into the genetics around APS is still at an early stage, but it seems the genes you inherit from your parents may play a role in the development of abnormal antiphospholipid antibodies. […] But having a family member with antiphospholipid antibodies increases the chance of your immune system also producing them. […] It’s thought that one or more environmental triggers may be needed to start APS in some people.

• #12 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #13 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  In addition, certain vaccines have been associated with APS. For example, vaccination with tetanus toxoid may trigger the formation of antibodies that cross-react with beta-2 glycoprotein I, due to molecular mimicry between the two molecules. […] Genetic predisposition may be involved, as follows: Relatives of persons with known APS are more likely to have aPL antibodies. One study showed a 33% frequency. An association has been found between aCL antibody and carriage of certain HLA genes, including DRw53, DR7 (mostly in people of Hispanic origin), and DR4 (mostly in Whites).

• #14 Antiphospholipid syndrome – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/antiphospholipid-syndrome/

  Antiphospholipid syndrome (APS) is an autoimmune disease associated with increased risk of thrombosis due to the presence of procoagulatory antibodies. […] Etiology: Primary: Idiopathic; Associated with genetic marker HLA-DR7. […] Secondary: Systemic lupus erythematosus (most common cause of secondary APS), Rheumatoid arthritis, Neoplasms, HIV, hepatitis A, hepatitis B, hepatitis C, Bacterial infections (e.g., syphilis, Lyme disease, tuberculosis). […] Formation of procoagulatory antiphospholipid antibodies: Antibodies form complexes with anticoagulant proteins, thereby inactivating them (e.g., protein C, protein S, antithrombin III). […] Antibodies activate platelets and vascular endothelium. […] Induction of a hypercoagulable state risk of thrombosis and embolism. […] Positive lupus anticoagulant is associated with the highest risk of thrombosis. […] A finding of positive aPL antibodies without clinical features is not sufficient to confirm a diagnosis of APS. […] CAPS is a rare but severe form of APS caused by acute and systemic small vessel thrombosis.

• #15 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  In addition, certain vaccines have been associated with APS. For example, vaccination with tetanus toxoid may trigger the formation of antibodies that cross-react with beta-2 glycoprotein I, due to molecular mimicry between the two molecules. […] Genetic predisposition may be involved, as follows: Relatives of persons with known APS are more likely to have aPL antibodies. One study showed a 33% frequency. An association has been found between aCL antibody and carriage of certain HLA genes, including DRw53, DR7 (mostly in people of Hispanic origin), and DR4 (mostly in Whites).

• #16 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  We are not sure why the immune system becomes over-active and is unable to distinguish between foreign invaders and its own body constituents. […] Some APS patients will have family members who also have the syndrome or have histories of other autoimmune diseases in their families, such as lupus or thyroid disease. […] One theory is that viral infections, such as glandular fever and shingles, stress, certain drugs, and other well-known precipitants of thrombosis, such as immobility, dehydration, surgery, the oral contraceptive pill and pregnancy, can act as the trigger for the syndrome.

• #17 Antiphospholipid syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/antiphospholipid-syndrome/

  Antiphospholipid syndrome is a disorder characterized by an increased tendency to form abnormal blood clots (thromboses) that can block blood vessels. […] The genetic cause of antiphospholipid syndrome is unknown. This condition results from the presence of three abnormal immune proteins (antibodies) in the blood. […] The production of the antiphospholipid antibodies may coincide with exposure to foreign invaders, such as viruses and bacteria, that are similar to normal human proteins. […] Certain genetic variations (polymorphisms) in a few genes have been found in people with antiphospholipid syndrome and may predispose individuals to produce the specific antibodies known to contribute to the formation of thromboses. […] Most cases of antiphospholipid syndrome are sporadic, which means they occur in people with no history of the disorder in their family.

• #18 Antiphospholipid syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/antiphospholipid-syndrome/

  Antiphospholipid syndrome is a disorder characterized by an increased tendency to form abnormal blood clots (thromboses) that can block blood vessels. […] The genetic cause of antiphospholipid syndrome is unknown. This condition results from the presence of three abnormal immune proteins (antibodies) in the blood. […] The production of the antiphospholipid antibodies may coincide with exposure to foreign invaders, such as viruses and bacteria, that are similar to normal human proteins. […] Certain genetic variations (polymorphisms) in a few genes have been found in people with antiphospholipid syndrome and may predispose individuals to produce the specific antibodies known to contribute to the formation of thromboses. […] Most cases of antiphospholipid syndrome are sporadic, which means they occur in people with no history of the disorder in their family.

• #19

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Antiphospholipid syndrome (APS) is caused by the body’s immune system producing abnormal antibodies called antiphospholipid antibodies. […] It’s not clear why these abnormal antibodies are produced, or why many people have antiphospholipid antibodies but don’t develop blood clots. […] A combination of genetic and environmental factors is thought to be responsible. […] Research into the genetics around APS is still at an early stage, but it seems the genes you inherit from your parents may play a role in the development of abnormal antiphospholipid antibodies. […] But having a family member with antiphospholipid antibodies increases the chance of your immune system also producing them. […] It’s thought that one or more environmental triggers may be needed to start APS in some people.

• #20 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  We are not sure why the immune system becomes over-active and is unable to distinguish between foreign invaders and its own body constituents. […] Some APS patients will have family members who also have the syndrome or have histories of other autoimmune diseases in their families, such as lupus or thyroid disease. […] One theory is that viral infections, such as glandular fever and shingles, stress, certain drugs, and other well-known precipitants of thrombosis, such as immobility, dehydration, surgery, the oral contraceptive pill and pregnancy, can act as the trigger for the syndrome.

• #21 Antiphospholipid syndrome

  https://dermnetnz.org/topics/antiphospholipid-syndrome

  Antiphospholipid syndrome is an acquired autoimmune disorder characterised by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests). Antiphospholipid syndrome can be primary or secondary. […] Secondary antiphospholipid syndrome is associated with an underlying disease, usually systemic lupus erythematosus, and drugs, including levamisole-adulterated cocaine. […] The mechanisms resulting in a hypercoagulable state and recurrent thrombosis are not yet defined. Complement activation is involved. The 'two-hit’ theory has associated the onset of thrombosis with increasing age, hypertension, diabetes, obesity, smoking, pregnancy, surgery, and other genetic hypercoagulable states.

• #22

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Environmental factors that may be responsible include: viral infections, such as the cytomegalovirus (CMV) or slapped cheek syndrome (parvovirus B19), bacterial infections, such as E. coli (a bacteria often associated with food poisoning) or leptospirosis (an infection usually spread by certain animals), certain medications, such as anti-epileptic medicine or the combined contraceptive pill. […] Another theory is that many people with abnormal antiphospholipid antibodies only go on to develop APS if they have a higher risk of developing blood clots.

• #23 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by thrombosis or pregnancy morbidity coupled with persistent antiphospholipid antibodies. […] The antibodies contributing to APS diagnosis are lupus anticoagulant, anticardiolipin antibodies, and antibeta-2 glycoprotein I antibodies. […] It is thought that the initiating events for induction of antibodies to phospholipid-binding proteins are infections, oxidative stress, and major physical stresses (e.g., surgery, trauma). […] The binding of phospholipids to serum proteins (e.g., B2GPI, prothrombin) leads to neoantigen formation and triggers the induction of antiphospholipids. […] Other proposed mechanisms for thrombotic events and APS-related obstetric complications include complement neutrophil activation and an imbalance between type I and III interferons.

• #24 Antiphospholipid syndrome: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/antiphospholipid-syndrome/

  Antiphospholipid syndrome is a disorder characterized by an increased tendency to form abnormal blood clots (thromboses) that can block blood vessels. […] The genetic cause of antiphospholipid syndrome is unknown. This condition results from the presence of three abnormal immune proteins (antibodies) in the blood. […] The production of the antiphospholipid antibodies may coincide with exposure to foreign invaders, such as viruses and bacteria, that are similar to normal human proteins. […] Certain genetic variations (polymorphisms) in a few genes have been found in people with antiphospholipid syndrome and may predispose individuals to produce the specific antibodies known to contribute to the formation of thromboses. […] Most cases of antiphospholipid syndrome are sporadic, which means they occur in people with no history of the disorder in their family.

• #25 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #26 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  Antiphospholipid syndrome (APS) is an acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. […] APS is an autoimmune disorder of unknown cause. The search for possible triggers has uncovered a wide array of autoimmune or rheumatic diseases, infections, and drugs that are associated with the lupus anticoagulant or anticardiolipin antibodies. These associations may ultimately provide a clue to the etiology of APS. […] A considerable percentage of persons with certain autoimmune or rheumatic diseases also have aPL antibodies. […] Infections associated with APS include the following: Syphilis, Hepatitis C, HIV infection, Human T-cell lymphotrophic virus type 1 infection, Malaria, Bacterial septicemia. […] Drugs associated with APS include the following: Cardiac – Procainamide, quinidine, propranolol, hydralazine; Neuroleptic or psychiatric – Phenytoin, chlorpromazine; Other – Interferon alfa, quinine, amoxicillin.

• #27 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  Certain genetic factors may be important, as indicated by a number of family and twin studies for SLE and the demonstration of an increased frequency of HLA-DR2, HLA-DR3, and HLA-DR4 null alleles in patients with SLE. […] PL molecules are ubiquitous in nature and are present in the inner surface of the cell (ie, on the inner or outer surface of the cell membrane or intracellular organelles) and in microorganisms. Therefore, during infectious disease processes, including viral (eg, HIV, Epstein-Barr virus [EBV], cytomegalovirus [CMV], adenoviruses), bacterial (eg, bacterial endocarditis, tuberculosis, Mycoplasma pneumonia), spirochetal (eg, syphilis, leptospirosis, Lyme disease), and parasitic (eg, malaria infection) infections, the disruption of cellular membranes may occur during cell damage. PLs are consequently released, stimulating aPL antibodies.

• #28 Blood Clotting Disorders – Antiphospholipid Syndrome (APS) | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is an autoimmune disorder that causes abnormal blood clots to form. […] The specific antibodies in APS are called antiphospholipids because they attack and damage parts of cells called phospholipids. […] Your family history and genes, other medical conditions, medicines and procedures, or lifestyle factors may raise your risk of APS. […] APS is most common in people who have lupus. In fact, 20% to 30% of people with lupus have antiphospholipid antibodies. […] Bacterial or viral infections: HIV, hepatitis C, and the bacteria that causes Lyme disease can increase your risk of making APS antibodies or trigger APS.

• #29 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #30

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Environmental factors that may be responsible include: viral infections, such as the cytomegalovirus (CMV) or slapped cheek syndrome (parvovirus B19), bacterial infections, such as E. coli (a bacteria often associated with food poisoning) or leptospirosis (an infection usually spread by certain animals), certain medications, such as anti-epileptic medicine or the combined contraceptive pill. […] Another theory is that many people with abnormal antiphospholipid antibodies only go on to develop APS if they have a higher risk of developing blood clots.

• #31 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #32 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  Antiphospholipid syndrome (APS) is an acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. […] APS is an autoimmune disorder of unknown cause. The search for possible triggers has uncovered a wide array of autoimmune or rheumatic diseases, infections, and drugs that are associated with the lupus anticoagulant or anticardiolipin antibodies. These associations may ultimately provide a clue to the etiology of APS. […] A considerable percentage of persons with certain autoimmune or rheumatic diseases also have aPL antibodies. […] Infections associated with APS include the following: Syphilis, Hepatitis C, HIV infection, Human T-cell lymphotrophic virus type 1 infection, Malaria, Bacterial septicemia. […] Drugs associated with APS include the following: Cardiac – Procainamide, quinidine, propranolol, hydralazine; Neuroleptic or psychiatric – Phenytoin, chlorpromazine; Other – Interferon alfa, quinine, amoxicillin.

• #33 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  In addition, certain vaccines have been associated with APS. For example, vaccination with tetanus toxoid may trigger the formation of antibodies that cross-react with beta-2 glycoprotein I, due to molecular mimicry between the two molecules. […] Genetic predisposition may be involved, as follows: Relatives of persons with known APS are more likely to have aPL antibodies. One study showed a 33% frequency. An association has been found between aCL antibody and carriage of certain HLA genes, including DRw53, DR7 (mostly in people of Hispanic origin), and DR4 (mostly in Whites).

• #34

  https://link.springer.com/article/10.1007/s11926-018-0773-x

  The antiphospholipid syndrome (APS) is characterized by the development of thrombotic events and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). An infectious etiology for this syndrome has been postulated. […] There is an increased risk of developing aPL in various infections, particularly in viral infections. The most frequent infection related to aPL has been hepatitis C virus. […] Accumulated evidence supports that the presence of aPL is associated with a variety of infections, including viruses, bacteria, fungi, and parasites, and the main mechanism to explain this correlation is molecular mimicry. […] Moreover, a link between vaccinations, such as the tetanus toxoid, and APS has also been described.

• #35 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  We are not sure why the immune system becomes over-active and is unable to distinguish between foreign invaders and its own body constituents. […] Some APS patients will have family members who also have the syndrome or have histories of other autoimmune diseases in their families, such as lupus or thyroid disease. […] One theory is that viral infections, such as glandular fever and shingles, stress, certain drugs, and other well-known precipitants of thrombosis, such as immobility, dehydration, surgery, the oral contraceptive pill and pregnancy, can act as the trigger for the syndrome.

• #36 Lupus anticoagulants and antiphospholipid antibodies Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/lupus-anticoagulants-and-antiphospholipid-antibodies

  Antiphospholipid syndrome (APS) is an autoimmune disorder that involves frequent blood clots (thromboses). When you have this condition, your body’s immune system makes abnormal proteins that attack the blood cells and lining of the blood vessels. The presence of these antibodies can cause problems with blood flow and lead to dangerous clots in blood vessels throughout the body. […] The exact cause of APS is not known. Both certain gene changes and other factors (such as an infection) may cause the problem to develop. […] It is often found in people with other autoimmune diseases, such as systemic lupus erythematosus (SLE). The condition is more common in women than in men. It is often found in women who have a history of repeated miscarriages. […] Some people carry the antibodies mentioned above, but do not have APS. Certain triggers may cause these people to have a blood clot, including: Smoking, Prolonged bed rest, Pregnancy, Hormone therapy or birth control pills, Cancer, Kidney disease.

• #37 Antiphospholipid Syndrome – Symptoms and Risk Factors

  https://www.uofmhealth.org/conditions-treatments/rheumatology/antiphospholipid-syndrome

  It is possible for some people to produce antibodies that attack phospholipids, but never develop blood clots. However, for people with these antibodies, a number of factors seem to be linked to an increased risk of developing blood clots, including smoking, high blood pressure, obesity, taking estrogens (birth control pills), extended periods of inactivity such as bed rest, and having an associated autoimmune disease like lupus.

• #38 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #39 Antiphospholipid syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Antiphospholipid_syndrome

  Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. […] Although the exact etiology of APS is still not clear, genetics is believed to play a key role in the development of the disease. […] Antiphospholipid syndrome can be primary or secondary. […] Primary antiphospholipid syndrome occurs in the absence of any other related disease. […] Secondary antiphospholipid syndrome occurs with other autoimmune diseases, such as systemic lupus erythematosus. […] Antiphospholipid syndrome is an autoimmune disease, in which „antiphospholipid antibodies” react against proteins that bind to anionic phospholipids on plasma membranes. […] These antibodies lead to blood clots and vascular disease in the presence (secondary APS) or absence (primary APS) of other diseases.

• #40 Antiphospholipid Syndrome: Symptoms, Diagnosis and Treatments

  https://www.webmd.com/dvt/what-is-antiphospholipid-syndrome

  Sometimes a health condition, such as lupus or another autoimmune disorder, causes you to develop APS. That’s called secondary antiphospholipid syndrome. But you can get APS even if you don’t have another condition (primary phospholipid syndrome). Scientists think genetics is at least partly to blame.

• #41 Antiphospholipid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430980/

  Antiphospholipid syndrome (APS) can be primary when there is no evidence of autoimmune disease or secondary to autoimmune processes, such as systemic lupus erythematosus (SLE), in 40% of cases. A study found positive APLAs in 6% of all pregnant patients, 13.5% of stroke patients, and 9.5% of patients with deep venous thromboses (DVTs). […] Genetic risk factors, such as coagulation factor mutations, increase the risk of antiphospholipid antibody-associated thrombosis. HLA-DR7, DR4, DRw53, DQw7, and C4 null alleles have been reported to be associated with APS. Infections, particularly viral, are associated with elevated APLA levels compared to bacterial infections. Some of these include Borrelia burgdorferi, Coxiella burnetii, Treponema, hepatitis C, HIV, COVID-19, Epstein-Barr virus (EBV), and Leptospira have been implicated in APLA formation. In fact, a large meta-analysis found that almost 50% of patients diagnosed with COVID-19 had positive APS, most commonly lupus anticoagulant. However, this analysis found no increased thrombotic risk in APLA-positive COVID-19 patients. […] Several drugs, including chlorpromazine, procainamide, quinidine, and phenytoin, can induce APLA production. Low levels of APLAs may also be normally present and may be transient, leading to the requirement of positive antibodies at least 12 weeks apart for diagnosis.

• #42 Blood Clotting Disorders – Antiphospholipid Syndrome (APS) | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is an autoimmune disorder that causes abnormal blood clots to form. […] The specific antibodies in APS are called antiphospholipids because they attack and damage parts of cells called phospholipids. […] Your family history and genes, other medical conditions, medicines and procedures, or lifestyle factors may raise your risk of APS. […] APS is most common in people who have lupus. In fact, 20% to 30% of people with lupus have antiphospholipid antibodies. […] Bacterial or viral infections: HIV, hepatitis C, and the bacteria that causes Lyme disease can increase your risk of making APS antibodies or trigger APS.

• #43 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics/print

  APS is more common in people with other autoimmune or rheumatic diseases, particularly systemic lupus erythematosus (SLE). APS is referred to as „primary” when it occurs alone. […] ANTIPHOSPHOLIPID SYNDROME SYMPTOMS […] The symptoms of antiphospholipid syndrome (APS) are related primarily to abnormal blood clotting, miscarriage, or stillbirth. Rarely, patients with APS can also develop symptoms that are unrelated to clotting, such as low red blood cell counts (anemia), low platelet counts (thrombocytopenia), skin rashes and/or ulcers, memory problems, and/or heart valve disease. […] […] Thrombosis (blood clot) — The presence of antiphospholipid antibodies (aPL) can increase the risk of developing a thrombosis (blood clot) in a vein or artery, especially if the person also has other risk factors for blood clots. If they don’t get treatment for the first blood clot, people often experience repeated clots. […]

• #44 Antiphospholipid Syndrome: Antibodies, Clots, Risks

  https://www.verywellhealth.com/antiphospholipid-syndrome-7565240

  While not a complete list, other autoimmune diseases associated with secondary APS include rheumatoid arthritis, Sjgren’s disease, autoimmune thyroid disease, and systemic sclerosis. […] Antiphospholipid syndrome, or APS, is an autoimmune disease (when your immune system attacks its own tissue) associated with one of three APS antibodies, blood clots, and/or pregnancy-related complications, like recurrent miscarriages. […] APS may occur on its own or with another autoimmune disease, namely lupus.

• #45 About Antiphospholipid Syndrome

  https://www.genome.gov/Genetic-Disorders/Antiphospholipid-Syndrome

  APS is classified within the category of autoimmune disorders (conditions caused by an immune response against the body’s own tissues). Individuals with aPL antibodies sometimes have an additional autoimmune disorder, most commonly systemic lupus erythematosus (SLE). About 30-40 percent of individuals with SLE have elevated aPL antibodies. APS has also been associated with a number of other autoimmune disorders, including myasthenia gravis, Graves’ disease, autoimmune hemolytic anemia, and Evan’s syndrome. […] Although APS has been reported to occur in multiple members of the same family, no clear inheritance pattern has been identified and no gene has been found to be the sole cause of this condition. […] Treatment for APS must be individualized according to the person’s current health status and the types of problems that person has experienced due to their APS.

• #46 Antiphospholipid Syndrome Specialist – Denver, CO: Jill R. Schofield, MD: Autoimmune Disease and Dysautonomia Practice: Center for Multisystem Disease

  https://www.centerformultisystemdisease.com/services/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is an autoimmune blood clotting disorder that may cause recurrent miscarriage or stillbirth as well as other serious problems like strokes, heart attacks and blood clots in the lungs (pulmonary embolus) and/or legs (deep vein thrombosis). […] APS, which is most often diagnosed in your 20s to 40s, develops when your immune system produces antiphospholipid antibodies. These autoantibodies affect the normal clotting processes, leading to sticky blood and an increased tendency to form blood clots. […] Primary APS occurs without the presence of another autoimmune disorder. However, APS may also co-exist with other autoimmune disorders, most commonly lupus, but also Sjogrens syndrome, autoimmune thyroid disease, and celiac disease. […] Antiphospholipid antibodies are especially dangerous in pregnancy. In fact, APS is recognized as one of the most common causes of recurrent miscarriage, especially late miscarriage. […] Blood clots caused by APS can affect any artery, vein, and organ in your body, even leading to organ damage. […] In some patients, APS leads to thrombocytopenia, or low blood platelets.

• #47 Antiphospholipid Syndrome: Symptoms, Diagnosis and Treatments

  https://www.webmd.com/dvt/what-is-antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a rare immune system disorder that can cause clots to form in your blood vessels. This raises your risk for several types of health problems, including heart attack, stroke, and miscarriage. […] Scientists aren’t sure exactly what causes APS, which is most common in young women (and those assigned female at birth). There’s no cure, but treatment can help prevent blood clots from forming. […] About 1%-5% of people in the U.S. have such abnormal antibodies in their blood that can lead to antiphospholipid syndrome. But some people with these antibodies never show any signs of the disease. To be diagnosed with antiphospholipid syndrome, you must have both the antibodies and APS symptoms. […] As with other autoimmune disorders, it’s not clear what causes these antibodies to develop. Scientists think both genes and things in your environment are involved.

• #48 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics/print

  ANTIPHOSPHOLIPID SYNDROME OVERVIEW […] Phospholipids are molecules that are present in the membranes that form the surface of cells, including blood cells and the cells that line blood vessels. In some people, the immune system develops abnormal antibodies (proteins) called „antiphospholipid antibodies” (aPL). These antibodies can affect blood flow and increase the risk of developing blood clots in the veins or arteries, as well as the risk of miscarriage or stillbirth among pregnant people. However, some people with aPL do not develop clots or other complications. […] […] People who have aPL and develop blood clots or pregnancy-related complications are said to have a syndrome called the antiphospholipid syndrome (APS). APS is an autoimmune disorder, meaning that it occurs when the body’s immune system mistakenly attacks healthy tissues and organs. […]

• #49 Antiphospholipid Syndrome – Symptoms and Risk Factors

  https://www.uofmhealth.org/conditions-treatments/rheumatology/antiphospholipid-syndrome

  It is possible for some people to produce antibodies that attack phospholipids, but never develop blood clots. However, for people with these antibodies, a number of factors seem to be linked to an increased risk of developing blood clots, including smoking, high blood pressure, obesity, taking estrogens (birth control pills), extended periods of inactivity such as bed rest, and having an associated autoimmune disease like lupus.

• #50 Antiphospholipid syndrome

  https://dermnetnz.org/topics/antiphospholipid-syndrome

  Antiphospholipid syndrome is an acquired autoimmune disorder characterised by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests). Antiphospholipid syndrome can be primary or secondary. […] Secondary antiphospholipid syndrome is associated with an underlying disease, usually systemic lupus erythematosus, and drugs, including levamisole-adulterated cocaine. […] The mechanisms resulting in a hypercoagulable state and recurrent thrombosis are not yet defined. Complement activation is involved. The 'two-hit’ theory has associated the onset of thrombosis with increasing age, hypertension, diabetes, obesity, smoking, pregnancy, surgery, and other genetic hypercoagulable states.

• #51 Antiphospholipid Syndrome

  https://rheumatology.org/patients/antiphospholipid-syndrome

  Antiphospholipid antibody syndrome (APS) is an autoimmune disease mostly affecting women between ages 30 and 40. In APS, abnormal proteins (aPL) can cause the formation of blood clots in veins and arteries. […] Risk factors for developing blood clots include high blood pressure, obesity, smoking, atherosclerosis, birth control pills, or having an associated autoimmune disease like SLE.

• #52

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Environmental factors that may be responsible include: viral infections, such as the cytomegalovirus (CMV) or slapped cheek syndrome (parvovirus B19), bacterial infections, such as E. coli (a bacteria often associated with food poisoning) or leptospirosis (an infection usually spread by certain animals), certain medications, such as anti-epileptic medicine or the combined contraceptive pill. […] Another theory is that many people with abnormal antiphospholipid antibodies only go on to develop APS if they have a higher risk of developing blood clots.

• #53 Antiphospholipid Syndrome and Stroke – European Stroke Organisation

  https://eso-stroke.org/antiphospholipid-syndrome-and-stroke/

  The first description of antiphospholipid syndrome (APS) is dated to 1983 following the discovery of lupus anticoagulant immunoglobulin and its relation to autoimmune disorders. APS represents an autoimmune condition characterized by a wide range of clinical manifestations. […] APS is accompanied by the presence of antiphospholipid antibodies (aPL), primarily lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-beta(2)-glycoprotein I (aB2GPI). The circulating antibodies induce endothelial dysfunction and interfere with the coagulation pathways by competing with coagulation factors. This leads to a procoagulant state, clot formation, and recurrent thromboembolic events. The pathophysiology is probably based on a two-hit hypothesis where the first hit represents the asymptomatic presence of aPL antibodies occurring in approx. 1-5% of the population. The second hit represents a stress condition (pregnancy, infection, etc.) triggering the pathologic state itself. […] In conclusion, a considerable part of young stroke cases is due to APS. This fact makes the APS an important etiology to consider in the diagnostic work-up, mainly in patients under 45 years of age.

• #54 Antiphospholipid Syndrome: Symptoms, Diagnosis and Treatments

  https://www.webmd.com/dvt/what-is-antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a rare immune system disorder that can cause clots to form in your blood vessels. This raises your risk for several types of health problems, including heart attack, stroke, and miscarriage. […] Scientists aren’t sure exactly what causes APS, which is most common in young women (and those assigned female at birth). There’s no cure, but treatment can help prevent blood clots from forming. […] About 1%-5% of people in the U.S. have such abnormal antibodies in their blood that can lead to antiphospholipid syndrome. But some people with these antibodies never show any signs of the disease. To be diagnosed with antiphospholipid syndrome, you must have both the antibodies and APS symptoms. […] As with other autoimmune disorders, it’s not clear what causes these antibodies to develop. Scientists think both genes and things in your environment are involved.

• #55 Antiphospholipid syndrome | STROKE MANUAL

  https://www.stroke-manual.com/antiphospholipid-syndrome/

  antiphospholipid syndrome (APS) is an acquired autoimmune disease characterized by a hypercoagulable state. […] the diagnosis is based on the presence of clinical symptoms (thrombosis or pregnancy morbidity) along with persistently elevated titers of antiphospholipid antibodies (APLA or aPL). […] APS most commonly affects individuals aged 20-50 years and is more prevalent in women, with a female-to-male ratio of approximately 3:1 to 5:1. […] paraneoplastic syndromes associated with malignancies can increase the production of APLA. […] the presence (in 2 occasions at least 12 weeks apart) of lupus anticoagulant or of double (any combination of lupus anticoagulant, anticardiolipin antibodies, or anti-2-glycoprotein 1 antibodies) or triple (all 3 subtypes) aPL positivity OR the presence of persistently high aPL titers. […] in antiphospholipid syndrome, antibodies should be high and repeatedly present at high titers (a minimal interval of 3 months between the 2 samples) to confirm the diagnosis. […] Diagnosis of APS requires the presence of at least one clinical and one laboratory criterion.

• #56 Antiphospholipid Syndrome (APS Syndrome): Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/antiphospholipid-syndrome/guide/

  Antiphospholipid syndrome is a disease in which the body’s immune system mistakenly produces antibodies that attack certain parts of cells called phospholipids. […] When these phospholipids become damaged, blood clots can develop in the veins or arteries, leading to a stroke or heart attack. […] Its not known exactly what causes antiphospholipid syndrome, although certain gene variants have been linked to the condition. […] These variants may then prompt certain people to produce abnormal antibodies specifically, the three antibodies lupus anticoagulant, anticardiolpin, and anti-B2 glycoprotein 1 that then cause antiphospholipid syndrome. […] APS is thought to be caused by a combination of genetic factors and environmental exposure, such as to a virus. […] Antiphospholipid syndrome is more common in women than men. […] People with autoimmune and rheumatic diseases, such as lupus, are also more likely to have the condition.

• #57

  https://111.wales.nhs.uk/antiphospholipidsyndrome(aps)hughessyndrome/

  Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots. […] APS is an autoimmune condition. This means the immune system, which usually protects the body from infection and illness, attacks healthy tissue by mistake. […] In APS, the immune system produces abnormal antibodies called antiphospholipid antibodies. […] These target proteins attached to fat molecules (phospholipids), which makes the blood more likely to clot. […] It’s not known what causes the immune system to produce abnormal antibodies. […] As with other autoimmune conditions, genetic, hormonal and environmental factors are thought to play a part. […] Antiphospholipid syndrome (APS) is caused by the body’s immune system producing abnormal antibodies called antiphospholipid antibodies.

• #58 Antiphospholipid Syndrome (APS) – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/thrombotic-disorders/antiphospholipid-syndrome-aps

  Antiphospholipid syndrome is an autoimmune disorder characterized by venous and arterial thrombosis or pregnancy complications (eg, recurrent miscarriage) and persistent autoantibodies to phospholipid-bound proteins. […] The antiphospholipid syndrome (APS) is an autoimmune disorder that is characterized by arterial, venous, or microvascular thrombosis or recurrent pregnancy loss caused by antibodies directed against one or more phospholipid-bound proteins (eg, beta-2 glycoprotein 1, prothrombin, annexin A5). […] The pathogenesis of thrombosis in APS is unclear. […] Each of these derangements may contribute to the hypercoagulable state associated with antiphospholipid syndrome.

• #59 Antiphospholipid Syndrome: A Comprehensive Clinical Review

  https://www.mdpi.com/2077-0383/14/3/733

  Antiphospholipid syndrome (APS) is a rare systemic autoimmune disease characterized by persistent antiphospholipid antibodies (aPL) in combination with recurrent thrombosis in the veins and/or arteries, obstetric morbidity, and various non-thrombotic associated complications. […] APS pathogenesis involves complex interactions between aPL, phospholipid-binding proteins, and the coagulation cascade. […] APS constitutes a systemic autoimmune disease characterized by thrombotic events, obstetric morbidity (obstetric APS), and/or non-thrombotic complications, associated with the presence of persistently positive circulating antiphospholipid antibodies (aPL antibodies): anticardiolipin antibodies, anti-β2-glycoprotein 1 antibodies, and lupus anticoagulant. […] The pathophysiology of APS involves complex interactions among immune cells, endothelial cells, and the coagulation cascade. Antiphospholipid antibodies (aPLs) are produced by B-cells.

• #60 About Antiphospholipid Syndrome

  https://www.genome.gov/Genetic-Disorders/Antiphospholipid-Syndrome

  Antiphospholipid syndrome (APS) is a disorder characterized by elevated levels of multiple different antibodies that are associated with both arterial and venous thrombosis (clots in the arteries and veins). […] There are two primary classes of antiphospholipid (aPL) antibodies, the antibodies associated with APS. These are called anticardiolipin antibodies and the lupus anticoagulant, and are directed against specific molecules. […] Up until the 1980s, it was thought that aPL antibodies were directed against a type of molecule known as anionoic phospholipids. However, in the early 1990s, several different groups discovered that this was not the case. Anticardiolipin antibodies were found to act against 2GPI, while the lupus anticoagulant was first found to act against 2GPI and, more recently, prothrombin.

• #61 Antiphospholipid syndrome: Diagnosis – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-diagnosis

  Antiphospholipid syndrome (APS) is a clinical and laboratory diagnosis characterized by both persistent laboratory evidence of antiphospholipid antibodies (aPL) and related complications, which may include venous thrombosis, arterial thrombosis, adverse pregnancy outcomes, and nonthrombotic manifestations (eg, heart valve thickening, livedo reticularis/racemosa). APS occurs either as a primary condition or in the setting of an underlying disease, usually systemic lupus erythematosus (SLE). […] The diagnosis and classification criteria for APS are reviewed here. Additional topics discuss: […] Pathogenesis (see „Antiphospholipid syndrome: Pathogenesis”).

• #62 Antiphospholipid Syndrome (APS) – An Update on Clinical Features and Treatment Options

  https://openurologyandnephrologyjournal.com/VOLUME/8/PAGE/27/FULLTEXT/

  Antiphospholipid syndrome (APS) is an autoantibody disorder characterized by the presence of antiphospholipid (APL) antibodies and heterogeneous clinical manifestations. […] APS was first described by Hughes, Harris and Gharavi. These antibodies are directed against anionic phospholipids (PL) or protein-phospholipid complexes. It is one of the most common causes of acquired thrombophilia. […] Antiphospholipid syndrome has been divided into primary and secondary APS. Primary APS is characterized by the presence of antiphospholipid antibody (aPL) and its clinical/ lab sequelae without evidence of an associated rheumatic disorder. Secondary antiphospholipid syndrome is seen with a coexisting connective tissue disease such as systemic lupus erythematosus or rheumatoid arthritis. […] The clinical features of APS occur as a consequence of large, medium and small vessel thrombosis and in some instances, due to direct antibody interaction with cells such as neurons.

• #63 Risk Factors of Antiphospholipid Antibody Syndrome: its Pathogenesis, Signs and Symptoms

  https://www.jcmedu.org/jcmedu-articles/risk-factors-of-antiphospholipid-antibody-syndrome-its-pathogenesis-signs-and-symptoms-97906.html

  Antiphospholipid Syndrome (APS) is a disorder where the immune system unintentionally produces antibodies that assault bodily tissues. Blood clots may develop in arteries and veins as a result of these antibodies. Although the precise cause of APS is still unknown, genetics is thought to be a major factor in the diseases progression. […] Primary or secondary antiphospholipid syndrome are both possible. Primary antiphospholipid syndrome is a condition that doesnt have any concomitant diseases. Systemic lupus erythematosus and other autoimmune disorders are associated with secondary antiphospholipid syndrome. […] A subset of anti-cardiolipin antibodies and anti- apolipoprotein H (ApoH) bind to ApoH. Protein C, a glycoprotein with a crucial coagulation regulation role (inactivating Factor Va and Factor VIIIa), is inhibited by apoH. Prothrombin is more easily broken down into thrombin, which is the active form, when lupus anticoagulant antibodies bind to it. Antibodies that bind to protein S, a co-factor of protein C, are also present in APS. Anti-protein S antibodies thus reduce the effectiveness of protein C.

• #64 Risk Factors of Antiphospholipid Antibody Syndrome: its Pathogenesis, Signs and Symptoms

  https://www.jcmedu.org/jcmedu-articles/risk-factors-of-antiphospholipid-antibody-syndrome-its-pathogenesis-signs-and-symptoms-97906.html

  Antiphospholipid Syndrome (APS) is a disorder where the immune system unintentionally produces antibodies that assault bodily tissues. Blood clots may develop in arteries and veins as a result of these antibodies. Although the precise cause of APS is still unknown, genetics is thought to be a major factor in the diseases progression. […] Primary or secondary antiphospholipid syndrome are both possible. Primary antiphospholipid syndrome is a condition that doesnt have any concomitant diseases. Systemic lupus erythematosus and other autoimmune disorders are associated with secondary antiphospholipid syndrome. […] A subset of anti-cardiolipin antibodies and anti- apolipoprotein H (ApoH) bind to ApoH. Protein C, a glycoprotein with a crucial coagulation regulation role (inactivating Factor Va and Factor VIIIa), is inhibited by apoH. Prothrombin is more easily broken down into thrombin, which is the active form, when lupus anticoagulant antibodies bind to it. Antibodies that bind to protein S, a co-factor of protein C, are also present in APS. Anti-protein S antibodies thus reduce the effectiveness of protein C.

• #65 Antiphospholipid syndrome and haemorrhagic complications: A narrative review of the literature | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-antiphospholipid-syndrome-haemorrhagic-complications-a-S2444440523000523

  Antiphospholipid syndrome is an acquired autoimmune thrombophilia characterized by a series of phenomena consisting of thrombosis (venous and / or arterial or microvascular) and/or loss of pregnancy or complications in association with persistent positive antiphospholipid antibodies. […] There are multiple causes that lead to bleeding complications in this context. […] The causes of bleeding in APS include acquired thrombocytopathies, thrombocytopenia, and inhibitors against specific coagulation factors. […] Lupus anticoagulant-hypoprothrombinemia syndrome is characterized by the association of acquired deficiency of factor II (prothrombin) in the presence of LA. […] Thrombocytopenia is frequent in patients with APS, and approximately 20%-40% of patients with APS have different degrees of thrombocytopenia.

• #66 Antiphospholipid Syndrome and Stroke – European Stroke Organisation

  https://eso-stroke.org/antiphospholipid-syndrome-and-stroke/

  The first description of antiphospholipid syndrome (APS) is dated to 1983 following the discovery of lupus anticoagulant immunoglobulin and its relation to autoimmune disorders. APS represents an autoimmune condition characterized by a wide range of clinical manifestations. […] APS is accompanied by the presence of antiphospholipid antibodies (aPL), primarily lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-beta(2)-glycoprotein I (aB2GPI). The circulating antibodies induce endothelial dysfunction and interfere with the coagulation pathways by competing with coagulation factors. This leads to a procoagulant state, clot formation, and recurrent thromboembolic events. The pathophysiology is probably based on a two-hit hypothesis where the first hit represents the asymptomatic presence of aPL antibodies occurring in approx. 1-5% of the population. The second hit represents a stress condition (pregnancy, infection, etc.) triggering the pathologic state itself. […] In conclusion, a considerable part of young stroke cases is due to APS. This fact makes the APS an important etiology to consider in the diagnostic work-up, mainly in patients under 45 years of age.

• #67 Antiphospholipid Syndrome: A Comprehensive Clinical Review

  https://www.mdpi.com/2077-0383/14/3/733

  The “two-hit hypothesis” in antiphospholipid syndrome (APS) proposes that the development of APS requires two independent events: the first hit represents the persistently presence of aPL inducing a thrombophilic state whereas the second hit represents a stress condition, such as trauma, surgery, or infection, which pushes the hemostatic balance in favor of thrombosis. […] The presence of antiphospholipid antibodies is linked to obstetric morbidity such as intrauterine growth restriction, preeclampsia, chorea gravidarum, and recurrent spontaneous fetal loss. […] The presence of aPL in the appropriate clinical setting (venous thrombosis, arterial thrombosis, or pregnancy morbidity) is the hallmark laboratory test result that establishes the diagnosis of APS.

• #68 Antiphospholipid Syndrome: Antibodies, Clots, Risks

  https://www.verywellhealth.com/antiphospholipid-syndrome-7565240

  While not a complete list, other autoimmune diseases associated with secondary APS include rheumatoid arthritis, Sjgren’s disease, autoimmune thyroid disease, and systemic sclerosis. […] Antiphospholipid syndrome, or APS, is an autoimmune disease (when your immune system attacks its own tissue) associated with one of three APS antibodies, blood clots, and/or pregnancy-related complications, like recurrent miscarriages. […] APS may occur on its own or with another autoimmune disease, namely lupus.

• #69 The well-defined antiphospholipid syndrome induced by COVID-19: a rare case report and review of the literature | Thrombosis Journal | Full Text

  https://thrombosisjournal.biomedcentral.com/articles/10.1186/s12959-024-00669-6

  The association between antiphospholipid antibody positivity and COVID-19 remains insufficiently established, posing challenges for the accurate diagnosis of antiphospholipid syndrome. […] Therefore, we propose that SARS-CoV-2 infection may induce antiphospholipid syndrome characterized by sustained elevation in antiphospholipid antibody levels. […] Similar to other autoimmune diseases, the aetiology of antiphospholipid syndrome (APS) remains unclear. […] Study findings have indicated the presence of antiphospholipid antibodies in patients with thrombosis following varicella, hepatitis C, and cytomegalovirus infections. […] Interestingly, since the COVID-19 pandemic, antiphospholipid antibody positivity after SARS-CoV-2 infection has been consistently observed in numerous studies. […] However, the pathogenicity of these antibodies and their relevance to COVID-19 remain uncertain.

• #70

  https://link.springer.com/article/10.1007/s11926-018-0773-x

  The antiphospholipid syndrome (APS) is characterized by the development of thrombotic events and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). An infectious etiology for this syndrome has been postulated. […] There is an increased risk of developing aPL in various infections, particularly in viral infections. The most frequent infection related to aPL has been hepatitis C virus. […] Accumulated evidence supports that the presence of aPL is associated with a variety of infections, including viruses, bacteria, fungi, and parasites, and the main mechanism to explain this correlation is molecular mimicry. […] Moreover, a link between vaccinations, such as the tetanus toxoid, and APS has also been described.

• #71 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  Certain genetic factors may be important, as indicated by a number of family and twin studies for SLE and the demonstration of an increased frequency of HLA-DR2, HLA-DR3, and HLA-DR4 null alleles in patients with SLE. […] PL molecules are ubiquitous in nature and are present in the inner surface of the cell (ie, on the inner or outer surface of the cell membrane or intracellular organelles) and in microorganisms. Therefore, during infectious disease processes, including viral (eg, HIV, Epstein-Barr virus [EBV], cytomegalovirus [CMV], adenoviruses), bacterial (eg, bacterial endocarditis, tuberculosis, Mycoplasma pneumonia), spirochetal (eg, syphilis, leptospirosis, Lyme disease), and parasitic (eg, malaria infection) infections, the disruption of cellular membranes may occur during cell damage. PLs are consequently released, stimulating aPL antibodies.

• #72 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  The sequence TL-RVYK also shows homology to 8 different bacteria, including Haemophilus influenzae, Neisseria gonorrhoeae, and Shigella dysenteriae, and to viruses such as EBV and HIV. Therefore, data may support the theory predicting that epitope mimicry is involved in the propagation of the autoimmune status.

• #73 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  Certain genetic factors may be important, as indicated by a number of family and twin studies for SLE and the demonstration of an increased frequency of HLA-DR2, HLA-DR3, and HLA-DR4 null alleles in patients with SLE. […] PL molecules are ubiquitous in nature and are present in the inner surface of the cell (ie, on the inner or outer surface of the cell membrane or intracellular organelles) and in microorganisms. Therefore, during infectious disease processes, including viral (eg, HIV, Epstein-Barr virus [EBV], cytomegalovirus [CMV], adenoviruses), bacterial (eg, bacterial endocarditis, tuberculosis, Mycoplasma pneumonia), spirochetal (eg, syphilis, leptospirosis, Lyme disease), and parasitic (eg, malaria infection) infections, the disruption of cellular membranes may occur during cell damage. PLs are consequently released, stimulating aPL antibodies.

• #74 APS Support UK | APS – antiphospholipid syndrome

  https://aps-support.org.uk/about-aps/what-causes-it

  As of yet, we dont know why people develop APS, why some patients go on to have blood clots while others dont, why some women (but not all) have pregnancy problems and why some patients are affected by symptoms more than others vital research is needed before we can answer these questions. […] However, we do know that the root cause is an over-activity of the immune system. […] In patients with APS, the body produces harmful antibodies called antiphospholipid antibodies (aPL). […] The most important of these proteins is called beta-2-glycoprotein1. […] Phospholipids contribute to blood clot formation and play an essential role in maintaining proper blood consistency. Due to the phospholipids being attacked by the antibodies, the blood becomes excessively 'sticky’, resulting in a higher risk of developing blood clots.

• #75 Antiphospholipid syndrome: Symptoms, treatment, and diagnosis

  https://www.medicalnewstoday.com/articles/181700

  Antiphospholipid syndrome is an immune disorder in which abnormal antibodies are linked to abnormal blood clots in veins and arteries. […] APS is an autoimmune disorder. An autoimmune disorder causes antibodies to attack good cells by mistake. […] In APS, the persons immune system produces antibodies called antiphospholipid antibodies. […] These abnormal antibodies attack proteins and fats in the blood, and specifically phospholipids. […] Phospholipids are cell membrane substances that play a role in the blood-clotting process. […] If the antibodies attack the phospholipids, this can increase the risk of developing blood clots. […] The fats and proteins that are attacked are thought to be important in maintaining blood consistency. […] The blood becomes sticky, significantly increasing the risk of developing blood clots.

• #76 Antiphospholipid syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Antiphospholipid_syndrome

  Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. […] Although the exact etiology of APS is still not clear, genetics is believed to play a key role in the development of the disease. […] Antiphospholipid syndrome can be primary or secondary. […] Primary antiphospholipid syndrome occurs in the absence of any other related disease. […] Secondary antiphospholipid syndrome occurs with other autoimmune diseases, such as systemic lupus erythematosus. […] Antiphospholipid syndrome is an autoimmune disease, in which „antiphospholipid antibodies” react against proteins that bind to anionic phospholipids on plasma membranes. […] These antibodies lead to blood clots and vascular disease in the presence (secondary APS) or absence (primary APS) of other diseases.

• #77 Antiphospholipid syndrome: Symptoms, treatment, and diagnosis

  https://www.medicalnewstoday.com/articles/181700

  People with antiphospholipid syndrome produce antibodies that attack either the phospholipids or the blood proteins that bind to the phospholipids. […] There are two main types of APS: Primary antiphospholipid syndrome: It is not linked to any other disease or condition but develops in isolation […] Secondary antiphospholipid syndrome: It develops along with another autoimmune disorder, such as lupus. […] Why autoimmune disorders happen is unclear, and it is also unclear why some people with abnormal antibodies never develop symptoms. […] A combination of genetic and environmental factors appears to be involved.

• #78 Antiphospholipid syndrome (APS) | healthdirect

  https://www.healthdirect.gov.au/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a blood disorder cause by abnormal antibodies in the blood. […] APS happens when antibodies, proteins the body produces to fight infections, start attacking your body instead this is called an autoimmune reaction. […] There are a few different types of APS: Primary APS when you have the disorder with no underlying autoimmune disorder. Secondary APS when the disorder is caused by or occurs alongside another conditions, including: autoimmune conditions such as lupus, infections such as HIV/AIDS and Q Fever, medicines phenothiazines and procainamide. […] Catastrophic APS (CAPS) a rare but life-threatening type of APS. It is when there is widespread clotting (thrombosis) in your small blood vessels that causes failure of 3 or more organs in less than one week.

• #79 Antiphospholipid syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Antiphospholipid_syndrome

  Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. […] Although the exact etiology of APS is still not clear, genetics is believed to play a key role in the development of the disease. […] Antiphospholipid syndrome can be primary or secondary. […] Primary antiphospholipid syndrome occurs in the absence of any other related disease. […] Secondary antiphospholipid syndrome occurs with other autoimmune diseases, such as systemic lupus erythematosus. […] Antiphospholipid syndrome is an autoimmune disease, in which „antiphospholipid antibodies” react against proteins that bind to anionic phospholipids on plasma membranes. […] These antibodies lead to blood clots and vascular disease in the presence (secondary APS) or absence (primary APS) of other diseases.

• #80 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  Antiphospholipid syndrome (APS) is an autoimmune disorder that is associated with pregnancy complications, including preeclampsia, thrombosis, autoimmune thrombocytopenia, fetal growth restriction, and fetal loss. […] APS is classified as primary or secondary, depending on its association with other autoimmune disorders. Primary APS is diagnosed in patients demonstrating the clinical and laboratory criteria for the disease without other recognized autoimmune disease. Secondary APS is diagnosed in patients with other autoimmune disorders, such as systemic lupus erythematosus (SLE). […] Like other autoimmune disorders, APS does not have a known etiology, although it is known that the passive transfer of maternal antibodies mediates autoimmune disorders in the fetus and newborn. The mechanism of excess autoantibody production and immune complex formation is not well understood.

• #81 Antiphospholipid syndrome (APS) | healthdirect

  https://www.healthdirect.gov.au/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a blood disorder cause by abnormal antibodies in the blood. […] APS happens when antibodies, proteins the body produces to fight infections, start attacking your body instead this is called an autoimmune reaction. […] There are a few different types of APS: Primary APS when you have the disorder with no underlying autoimmune disorder. Secondary APS when the disorder is caused by or occurs alongside another conditions, including: autoimmune conditions such as lupus, infections such as HIV/AIDS and Q Fever, medicines phenothiazines and procainamide. […] Catastrophic APS (CAPS) a rare but life-threatening type of APS. It is when there is widespread clotting (thrombosis) in your small blood vessels that causes failure of 3 or more organs in less than one week.

• #82 Antiphospholipid Syndrome and Pregnancy: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/261691-overview

  Antiphospholipid syndrome (APS) is an autoimmune disorder that is associated with pregnancy complications, including preeclampsia, thrombosis, autoimmune thrombocytopenia, fetal growth restriction, and fetal loss. […] APS is classified as primary or secondary, depending on its association with other autoimmune disorders. Primary APS is diagnosed in patients demonstrating the clinical and laboratory criteria for the disease without other recognized autoimmune disease. Secondary APS is diagnosed in patients with other autoimmune disorders, such as systemic lupus erythematosus (SLE). […] Like other autoimmune disorders, APS does not have a known etiology, although it is known that the passive transfer of maternal antibodies mediates autoimmune disorders in the fetus and newborn. The mechanism of excess autoantibody production and immune complex formation is not well understood.

• #83 Antiphospholipid syndrome

  https://dermnetnz.org/topics/antiphospholipid-syndrome

  Antiphospholipid syndrome is an acquired autoimmune disorder characterised by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests). Antiphospholipid syndrome can be primary or secondary. […] Secondary antiphospholipid syndrome is associated with an underlying disease, usually systemic lupus erythematosus, and drugs, including levamisole-adulterated cocaine. […] The mechanisms resulting in a hypercoagulable state and recurrent thrombosis are not yet defined. Complement activation is involved. The 'two-hit’ theory has associated the onset of thrombosis with increasing age, hypertension, diabetes, obesity, smoking, pregnancy, surgery, and other genetic hypercoagulable states.

• #84 Antiphospholipid syndrome (APS) | healthdirect

  https://www.healthdirect.gov.au/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a blood disorder cause by abnormal antibodies in the blood. […] APS happens when antibodies, proteins the body produces to fight infections, start attacking your body instead this is called an autoimmune reaction. […] There are a few different types of APS: Primary APS when you have the disorder with no underlying autoimmune disorder. Secondary APS when the disorder is caused by or occurs alongside another conditions, including: autoimmune conditions such as lupus, infections such as HIV/AIDS and Q Fever, medicines phenothiazines and procainamide. […] Catastrophic APS (CAPS) a rare but life-threatening type of APS. It is when there is widespread clotting (thrombosis) in your small blood vessels that causes failure of 3 or more organs in less than one week.

• #85 Antiphospholipid Syndrome (APS) – An Update on Clinical Features and Treatment Options

  https://openurologyandnephrologyjournal.com/VOLUME/8/PAGE/27/FULLTEXT/

  Significant evidence exists that aPL antibodies are directly related to increased atherogenesis. […] Catastrophic antiphospholipid syndrome (CAPS) is a rare (less than 1%) manifestation with a very high mortality. CAPS causes thrombosis in three or more organs within one week with the histological demonstration of thrombi in the small vessels of the involved organs and presence of aPL antibodies in high titers. […] Infections, sudden withdrawal of anticoagulation, oral contraceptives, surgical procedures, and obstetric complications have been implicated in triggering these episodes.

• #86 Antiphospholipid syndrome (APS) | healthdirect

  https://www.healthdirect.gov.au/antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a blood disorder cause by abnormal antibodies in the blood. […] APS happens when antibodies, proteins the body produces to fight infections, start attacking your body instead this is called an autoimmune reaction. […] There are a few different types of APS: Primary APS when you have the disorder with no underlying autoimmune disorder. Secondary APS when the disorder is caused by or occurs alongside another conditions, including: autoimmune conditions such as lupus, infections such as HIV/AIDS and Q Fever, medicines phenothiazines and procainamide. […] Catastrophic APS (CAPS) a rare but life-threatening type of APS. It is when there is widespread clotting (thrombosis) in your small blood vessels that causes failure of 3 or more organs in less than one week.

• #87 Guidelines on the investigation and management of antiphospholipid syndrome

  https://b-s-h.org.uk/guidelines/guidelines/guidelines-on-the-investigation-and-management-of-antiphospholipid-syndrome

  APS may also present with unusual site thrombosis such as portal, renal, mesenteric and cerebral venous sinus thrombosis. […] Microvascular thrombosis in APS is uncommon but may manifest as the potentially lethal catastrophic antiphospholipid syndrome (CAPS), which develops in 1% of patients with APS and where there is evidence of multiorgan failure commonly affecting the heart, lungs, brain and/or kidneys.

• #88 Antiphospholipid Syndrome (APS) – An Update on Clinical Features and Treatment Options

  https://openurologyandnephrologyjournal.com/VOLUME/8/PAGE/27/FULLTEXT/

  Significant evidence exists that aPL antibodies are directly related to increased atherogenesis. […] Catastrophic antiphospholipid syndrome (CAPS) is a rare (less than 1%) manifestation with a very high mortality. CAPS causes thrombosis in three or more organs within one week with the histological demonstration of thrombi in the small vessels of the involved organs and presence of aPL antibodies in high titers. […] Infections, sudden withdrawal of anticoagulation, oral contraceptives, surgical procedures, and obstetric complications have been implicated in triggering these episodes.

• #89 Antiphospholipid Syndrome – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.4.

  Antiphospholipid syndrome (APS) is a disease proposed to be caused by autoantibodies directed against protein-phospholipid complexes. The etiology is unknown. Complications are attributed to procoagulant effects of antiphospholipid antibodies (APLAs): lupus anticoagulant (LA), anticardiolipin (aCL) antibodies, and antibeta2-glycoprotein (GP) I antibodies. APS may be either primary (not related to any other disorder) or secondary (associated with another autoimmune disease, which in 30%-50% of cases is systemic lupus erythematosus [SLE]). […] Seronegative APS with clinical features of APS but no detectable serum antibodies has also been described. […] CAPS may be due to a combination of autoimmune APS in a patient with an underlying, usually genetic, complement regulation disorder. It may be the first clinical presentation of APS.

• #90 Antiphospholipid Syndrome – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.4.

  Antiphospholipid syndrome (APS) is a disease proposed to be caused by autoantibodies directed against protein-phospholipid complexes. The etiology is unknown. Complications are attributed to procoagulant effects of antiphospholipid antibodies (APLAs): lupus anticoagulant (LA), anticardiolipin (aCL) antibodies, and antibeta2-glycoprotein (GP) I antibodies. APS may be either primary (not related to any other disorder) or secondary (associated with another autoimmune disease, which in 30%-50% of cases is systemic lupus erythematosus [SLE]). […] Seronegative APS with clinical features of APS but no detectable serum antibodies has also been described. […] CAPS may be due to a combination of autoimmune APS in a patient with an underlying, usually genetic, complement regulation disorder. It may be the first clinical presentation of APS.

• #91 Antiphospholipid Syndrome – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.4.

  Antiphospholipid syndrome (APS) is a disease proposed to be caused by autoantibodies directed against protein-phospholipid complexes. The etiology is unknown. Complications are attributed to procoagulant effects of antiphospholipid antibodies (APLAs): lupus anticoagulant (LA), anticardiolipin (aCL) antibodies, and antibeta2-glycoprotein (GP) I antibodies. APS may be either primary (not related to any other disorder) or secondary (associated with another autoimmune disease, which in 30%-50% of cases is systemic lupus erythematosus [SLE]). […] Seronegative APS with clinical features of APS but no detectable serum antibodies has also been described. […] CAPS may be due to a combination of autoimmune APS in a patient with an underlying, usually genetic, complement regulation disorder. It may be the first clinical presentation of APS.

• #92 APS: What Rheumatologists Should Know about Hughes Syndrome – The Rheumatologist

  https://www.the-rheumatologist.org/article/aps-what-rheumatologists-know-about-hughes-syndrome/?singlepage=1

  Like migraine, stroke is one of the recognized complications of APS/Hughes syndrome. […] However, as with migraine, recognition of aPL in the etiology of stroke is, in general, still poor. […] In 1985, we observed that in lupus patients, seizures were more common in those patients with positive aPL tests. […] The early clinical studies of APS were carried out in lupus clinics, and could well have included lupus features. […] The passage of time has also revealed one positive finding: Its very unusual for patients with primary APS to develop lupus in later years. […] I believe that one of the benefits of a trainee clinicians time spent in the lab is the recognition that many laboratory tests are open to variation. […] Three possible explanations for seronegative APS are: 1) The diagnosis is incorrect (unlikely in all cases); 2) the previously positive tests have become negative over time (uncommon in my experience); or 3) new tests are needed. […] The recognition of the many non-thrombotic manifestations of APS has added to the importance of separating diagnosis from classification.

• #93 APS: What Rheumatologists Should Know about Hughes Syndrome – The Rheumatologist

  https://www.the-rheumatologist.org/article/aps-what-rheumatologists-know-about-hughes-syndrome/?singlepage=1

  Like migraine, stroke is one of the recognized complications of APS/Hughes syndrome. […] However, as with migraine, recognition of aPL in the etiology of stroke is, in general, still poor. […] In 1985, we observed that in lupus patients, seizures were more common in those patients with positive aPL tests. […] The early clinical studies of APS were carried out in lupus clinics, and could well have included lupus features. […] The passage of time has also revealed one positive finding: Its very unusual for patients with primary APS to develop lupus in later years. […] I believe that one of the benefits of a trainee clinicians time spent in the lab is the recognition that many laboratory tests are open to variation. […] Three possible explanations for seronegative APS are: 1) The diagnosis is incorrect (unlikely in all cases); 2) the previously positive tests have become negative over time (uncommon in my experience); or 3) new tests are needed. […] The recognition of the many non-thrombotic manifestations of APS has added to the importance of separating diagnosis from classification.

• #94

  https://111.wales.nhs.uk/antiphospholipidsyndrome(aps)hughessyndrome/

  Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots. […] APS is an autoimmune condition. This means the immune system, which usually protects the body from infection and illness, attacks healthy tissue by mistake. […] In APS, the immune system produces abnormal antibodies called antiphospholipid antibodies. […] These target proteins attached to fat molecules (phospholipids), which makes the blood more likely to clot. […] It’s not known what causes the immune system to produce abnormal antibodies. […] As with other autoimmune conditions, genetic, hormonal and environmental factors are thought to play a part. […] Antiphospholipid syndrome (APS) is caused by the body’s immune system producing abnormal antibodies called antiphospholipid antibodies.

• #95

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/causes/

  Antiphospholipid syndrome (APS) is caused by the body’s immune system producing abnormal antibodies called antiphospholipid antibodies. […] It’s not clear why these abnormal antibodies are produced, or why many people have antiphospholipid antibodies but don’t develop blood clots. […] A combination of genetic and environmental factors is thought to be responsible. […] Research into the genetics around APS is still at an early stage, but it seems the genes you inherit from your parents may play a role in the development of abnormal antiphospholipid antibodies. […] But having a family member with antiphospholipid antibodies increases the chance of your immune system also producing them. […] It’s thought that one or more environmental triggers may be needed to start APS in some people.

• #96 Antiphospholipid Syndrome: Symptoms, Diagnosis and Treatments

  https://www.webmd.com/dvt/what-is-antiphospholipid-syndrome

  Antiphospholipid syndrome (APS) is a rare immune system disorder that can cause clots to form in your blood vessels. This raises your risk for several types of health problems, including heart attack, stroke, and miscarriage. […] Scientists aren’t sure exactly what causes APS, which is most common in young women (and those assigned female at birth). There’s no cure, but treatment can help prevent blood clots from forming. […] About 1%-5% of people in the U.S. have such abnormal antibodies in their blood that can lead to antiphospholipid syndrome. But some people with these antibodies never show any signs of the disease. To be diagnosed with antiphospholipid syndrome, you must have both the antibodies and APS symptoms. […] As with other autoimmune disorders, it’s not clear what causes these antibodies to develop. Scientists think both genes and things in your environment are involved.

• #97 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by thrombosis or pregnancy morbidity coupled with persistent antiphospholipid antibodies. […] The antibodies contributing to APS diagnosis are lupus anticoagulant, anticardiolipin antibodies, and antibeta-2 glycoprotein I antibodies. […] It is thought that the initiating events for induction of antibodies to phospholipid-binding proteins are infections, oxidative stress, and major physical stresses (e.g., surgery, trauma). […] The binding of phospholipids to serum proteins (e.g., B2GPI, prothrombin) leads to neoantigen formation and triggers the induction of antiphospholipids. […] Other proposed mechanisms for thrombotic events and APS-related obstetric complications include complement neutrophil activation and an imbalance between type I and III interferons.

• #98 Antiphospholipid Syndrome – ERN ReCONNET | European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases

  https://reconnet.ern-net.eu/disease-aps/

  Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial, venous, or small vessel thromboembolic events and/or obstetric morbidity in the presence of persistent circulating antiphospholipid (aPL) antibodies detected by means of three tests: lupus anticoagulant, anticardiolipin, and beta-2 glycoprotein I antibodies. […] The cause of APS is not entirely understood. Like other autoimmune diseases it is thought to be multifactorial and that genetic, hormonal and environmental factors play a significant role in developing the disease. APS occurs when the immune system mistakenly produces antibodies that make blood much more likely to clot. […] The severity of APS varies from person to person, so the prognosis is also variable. APS can cause life-threatening or life-limiting illnesses depending on the location and the extent of the thrombosis. In rare instances, a catastrophic APS supervenes. A higher risk of recurrent thrombosis is associated with the presence of lupus anticoagulant and/ triple antiphospholipid antibodies positivity (i.e. positivity for lupus anticoagulant, anti-cardiolipin and anti-2glycoprotein I antibodies), as opposed to a positive result of only one test.

• #99 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics/print

  ANTIPHOSPHOLIPID SYNDROME OVERVIEW […] Phospholipids are molecules that are present in the membranes that form the surface of cells, including blood cells and the cells that line blood vessels. In some people, the immune system develops abnormal antibodies (proteins) called „antiphospholipid antibodies” (aPL). These antibodies can affect blood flow and increase the risk of developing blood clots in the veins or arteries, as well as the risk of miscarriage or stillbirth among pregnant people. However, some people with aPL do not develop clots or other complications. […] […] People who have aPL and develop blood clots or pregnancy-related complications are said to have a syndrome called the antiphospholipid syndrome (APS). APS is an autoimmune disorder, meaning that it occurs when the body’s immune system mistakenly attacks healthy tissues and organs. […]

• #100 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics/print

  APS is more common in people with other autoimmune or rheumatic diseases, particularly systemic lupus erythematosus (SLE). APS is referred to as „primary” when it occurs alone. […] ANTIPHOSPHOLIPID SYNDROME SYMPTOMS […] The symptoms of antiphospholipid syndrome (APS) are related primarily to abnormal blood clotting, miscarriage, or stillbirth. Rarely, patients with APS can also develop symptoms that are unrelated to clotting, such as low red blood cell counts (anemia), low platelet counts (thrombocytopenia), skin rashes and/or ulcers, memory problems, and/or heart valve disease. […] […] Thrombosis (blood clot) — The presence of antiphospholipid antibodies (aPL) can increase the risk of developing a thrombosis (blood clot) in a vein or artery, especially if the person also has other risk factors for blood clots. If they don’t get treatment for the first blood clot, people often experience repeated clots. […]

• #101 Antiphospholipid Syndrome – Research at Michigan Medicine

  https://www.uofmhealth.org/conditions-treatments/rheumatology/research-antiphospholipid-syndrome

  Ongoing research at Michigan Medicine seeks to understand why certain people develop Antiphospholipid syndrome (APS), to find new and better ways to treat the disease, and ultimately, to find a cure. […] Patients who wish to are given the opportunity to participate in research at every clinic visit, by donating blood for studies into the genetic and cellular causes of APS. […] These studies would not be possible without samples of blood from people who have antiphospholipid antibodies.

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