Antiphospholipid Syndrome is autoimmune disorder producing antiphospholipid antibodies. It has been seen to be common in Down syndrome, IBS, and PCOS patients.

Antiphospholipid Syndrome

Humza Khizer

Overview

<p>The disorder &lsquo;antiphospholipid syndrome&rsquo; occurs when the immune system produces antibodies against the body&#39;s normal tissues. As a result of these antibodies, blood clots may develop in arteries and veins, lungs, legs, and other organs like the kidneys and spleen can also be affected. These clots can also result in <a href="https://www.merisehat.pk/disease/stroke" target="_blank">strokes</a>, <a href="https://www.merisehat.pk/disease/myocardial-infarction" target="_blank">heart attacks</a>, and other illnesses. Antiphospholipid syndrome during pregnancy can potentially lead to stillbirth and miscarriage. However, some patients may show no symptoms at all.<br />
&nbsp;</p>

<p>Antiphospholipid syndrome or APLS, is a multisystemic autoimmune condition, also referred to as Hughes syndrome. 50-70% of systemic lupus erythematosus (SLE) patients with positive antiphospholipid antibodies (APLA) have a high chance of developing APLS<sup><a href="#accordion">[1]</a></sup>.&nbsp;In a study of 197 couples with recurrent miscarriages, 20% were found to have APLA<sup><a href="#accordion">[2]</a></sup>.</p>

<p>Approximately 35,000 new cases of APS-related venous thrombosis are reported in the United States each year<sup><a href="#accordion">[3]</a></sup>. These people are significantly younger than the patients generally affected by thrombosis. Antiphospholipid syndrome is also known as:</p>

<ul>
	<li>Lupus anticoagulant syndrome</li>
	<li>Antiphospholipid antibody syndrome</li>
	<li>APLS</li>
	<li>APS</li>
	<li>Hughes syndrome<br />
	&nbsp;</li>
</ul>

<ol>
	<li>Primary antiphospholipid syndrome</li>
	<li>Secondary antiphospholipid syndrome</li>
	<li>Catastrophic antiphospholipid syndrome</li>
</ol>

<p>&nbsp;</p>

<h5><strong>Primary And Secondary APS&nbsp;</strong></h5>

<ul>
	<li>
	<p>Primary Antiphospholipid Syndrome (APS) can occur alone without any prominent indications. However, when APS occurs in conjunction with another autoimmune condition, such as systemic lupus erythematosus, it is known as secondary antiphospholipid syndrome.&nbsp;</p>
	</li>
	<li>
	<p>In both primary and secondary APS, the rates of pregnancy loss and arterial and venous thrombosis are identical.</p>
	</li>
	<li>Although the symptoms of primary APS and secondary APS with SLE are similar (making it more challenging to distinguish between the two), secondary is more prevalent.&nbsp;</li>
</ul>

<p>&nbsp;</p>

<h5><strong>Catastrophic Anti-Phospholipid Syndrome (CAPS)</strong></h5>

<p>Less than 1% of APLS patients will experience CAPS, an uncommon but potentially fatal condition<sup><a href="#accordion">[1]</a></sup>. In individuals with SLE and those who have cardiac, pulmonary, renal, and splenic involvement, mortality is particularly significant (48%)<sup><a href="#accordion">[1]</a></sup>. It is characterized by thrombosis that occurs quickly (a few days), in several organs. Small and medium-sized arteries are usually affected.</p>

<p>&nbsp;</p>

<p>According to the organ affected, the clinical presentation might vary and include:&nbsp;</p>

<ul>
	<li>Peripheral thrombosis (deep vein, femoral artery, or radial artery)</li>
	<li>Renal involvement (renal failure, thrombotic microangiopathy)</li>
	<li>Pulmonary involvement (pulmonary embolism, <a href="https://www.merisehat.pk/disease/severe-acute-respiratory-infection-sari" target="_blank">acute respiratory distress syndrome</a>, pulmonary hemorrhage)</li>
	<li>Cutaneous involvement (livedo reticularis, gangrene, digital ischemia, skin ulcerations)</li>
	<li>Cardiac involvement (myocardial infarction, valve lesions, heart failure)</li>
	<li>Cerebral involvement (<a href="https://www.merisehat.pk/disease/neonatal-encephalopathy-75874" target="_blank">encephalopathy</a>, ischemic stroke)</li>
	<li>Gastrointestinal involvement (bowel infarction, irritable bowel syndrome)</li>
	<li>Haematological involvement (thrombocytopenia)</li>
</ul>

<p>&nbsp;</p>

<p>In 2003, preliminary standards for CAPS classification were defined<sup><a href="#accordion">[1]</a></sup>. The four requirements are:</p>

<ol>
	<li>Involvement of 3 or more systems, tissues, or organs</li>
	<li>Manifestations appearing at the same time or within less than a week</li>
	<li>At least one organ or tissue shows tiny vessel blockage on histopathology.</li>
	<li>Confirmation of APLA in laboratory results</li>
</ol>

<p>Definite CAPS can be classified by the presence of all four criteria. In comparison, probable CAPS can be classified if three criteria are present.<br />
&nbsp;</p>

Signs And Symptoms

<ol>
	<li>High blood pressure</li>
	<li><a href="https://www.merisehat.pk/disease/myocardial-infarction" target="_blank">Heart attacks</a></li>
	<li>A <a href="https://www.merisehat.pk/disease/stroke" target="_blank">stroke </a>or a transient ischaemic attack (TIA) (&lsquo;mini-stroke&rsquo;)</li>
	<li><a href="https://www.merisehat.pk/disease/pulmonary-hypertension" target="_blank">Pulmonary embolism</a> (a blockage in one of the blood vessels in the lungs)</li>
	<li><a href="https://merisehat.pk/disease/deep-vein-thrombosis">Deep vein thrombosis</a></li>
</ol>

<p>&nbsp;</p>

<p>Any of the below symptoms can also be present in APS patients:</p>

<ol>
	<li>Mobility and balance issues</li>
	<li>Double vision&nbsp;</li>
	<li>Issues with memory and speech</li>
	<li>An itching or tingling sensation in your legs or arms.</li>
	<li>Extreme fatigue&nbsp;</li>
	<li>Persistent headaches or migraines<br />
	&nbsp;</li>
</ol>

<h5><strong>Pregnancy Problems</strong></h5>

<p>Pregnancy difficulties are far more likely to occur in women with APS, especially if the illness is left untreated. Potential issues include:</p>

<ul>
	<li>Repeated early miscarriages (3 or more times), typically in the first 10 weeks of pregnancy</li>
	<li>After the 10th week of pregnancy, typically 1 or more subsequent miscarriages</li>
	<li>Pre-eclampsia causing premature birth, which typically occurs at or before the 34th week of pregnancy due to high blood pressure<br />
	&nbsp;</li>
</ul>

<h5><strong>Livedo Reticularis</strong></h5>

<p>A skin condition called livedo reticularis is caused by blood clots developing inside the skin&#39;s blood vessels. Livedo reticularis can produce dark or brownish blotches on black and brown skin, and red or blue marks on white skin. Additionally, some people get nodules with sores, called ulcers (bumps).</p>

<p>Colder temperatures frequently worsen these symptoms. Additionally, exposure to cold might make the affected skin tingle or the affected skin might feel numb.<br />
&nbsp;</p>

<h5><strong>Superficial Thrombophlebitis</strong></h5>

<p>Inflammation of the veins just beneath the skin, typically in the leg, is known as superficial thrombophlebitis. Although they are not as severe, the symptoms are equivalent to DVT.</p>

<p>&nbsp;</p>

<p>The following are signs of superficial thrombophlebitis:</p>

<ul>
	<li>Edema</li>
	<li>Redness and discomfort along the afflicted vein</li>
	<li>A temperature of 38&deg;C or higher (although this occurs rarely)</li>
</ul>

<p>Usually, the symptoms go away in two to six weeks.<br />
&nbsp;</p>

Causes

<p>Antiphospholipid syndrome (APS) is brought on by the body&#39;s immune system creating faulty antibodies known as antiphospholipid antibodies. This raises the possibility of blood clots forming in the arteries, which can result in severe health issues, like:</p>

<ul>
	<li><a href="https://merisehat.pk/disease/deep-vein-thrombosis">Deep vein thrombosis</a>&nbsp;</li>
	<li><a href="https://www.merisehat.pk/disease/myocardial-infarction" target="_blank">Heart attack</a></li>
	<li><a href="https://www.merisehat.pk/disease/stroke" target="_blank">Stroke</a></li>
</ul>

<p>&nbsp;</p>

<p>It is unclear why these faulty antibodies are created or why many people have antiphospholipid antibodies, yet blood clots are not formed. The root cause is believed to be a result of both hereditary and environmental influences.</p>

<p>&nbsp;</p>

<h5><strong>Antiphospholipid Antibodies</strong></h5>

<p>Proteins called antibodies are created by the immune system to aid in the defense against &lsquo;external invaders&rsquo; like viruses and bacteria. In order to kill these bacteria and viruses, and stop the spread of infection, antibodies tell the immune system to release specific chemicals. However, in APS, the immune system creates faulty antibodies that wrongly target proteins present on the exterior of blood and blood vessel cells instead of targeting germs and viruses.</p>

<p>It is unknown what causes the blood to clot more quickly in this situation. But most medical professionals agree that maintaining the ideal blood consistency&mdash;not too fluid and not too sticky&mdash;requires a careful balancing act between various proteins and fats. In APS patients, faulty antibodies upset this balance.</p>

<p>&nbsp;</p>

<h5><strong>Hereditary Factors</strong></h5>

<p>It appears that the genes you inherit from your parents may be involved in the development of faulty antiphospholipid antibodies. However, research into the genetics surrounding APS is still in its infancy. Unlike other disorders, such as hemophilia and sickle cell anemia, APS is not directly passed from parents to children.</p>

<p>However, the likelihood that your immune system may produce antiphospholipid antibodies is increased if a family member has them. Studies have revealed that some APS patients have a defective gene that contributes to other autoimmune diseases, including lupus.</p>

<p>This may help to explain why APS sometimes co-occurs with another immune system disorder in some persons.</p>

<p>&nbsp;</p>

<h5><strong>Environmental Factors</strong></h5>

<p>Possible contributing environmental triggers are as follows:</p>

<ul>
	<li>Infections caused by viruses such the cytomegalovirus (CMV) or parvovirus B19 (slapped cheek syndrome)</li>
	<li>Infections caused by bacteria, such as E. coli which are frequently linked to food poisoning, and leptospirosis (zoonotic infection))</li>
	<li>Certain drugs, like anti-epileptic drugs or oral contraceptive pills</li>
</ul>

<p>&nbsp;</p>

<p>According to a different hypothesis, many people with abnormal antiphospholipid antibodies only go on to develop APS if they are at a higher risk of blood clots. For instance, if they:</p>

<ul>
	<li>Consume a poor diet, which raises blood cholesterol levels&nbsp;</li>
	<li>Don&#39;t exercise&nbsp;</li>
	<li>Smoke</li>
	<li>Are overweight or <a href="https://www.merisehat.pk/disease/obesity" target="_blank">obese&nbsp;</a></li>
	<li>Use hormone replacement therapy (HRT), or take contraceptives</li>
</ul>

<p>But this still doesn&#39;t explain why some kids and adults with none of these risk factors have APS.<br />
&nbsp;</p>

<p>Due to the emergence of neurological symptoms similar to those of multiple sclerosis (MS), some APS patients may be incorrectly diagnosed with MS. Multiple sclerosis is a chronic condition affecting the brain and spinal cord. Tiny lesions called plaques, which can appear randomly across the brain and spinal cord, are a hallmark of MS. These patches stop the neurological system&#39;s signals from being transmitted properly, which causes a number of symptoms, such as deformities and impairment.</p>

<p>The expression of lupus anticoagulant, moderate-high titers of IgG or IgM anticardiolipin or anti-beta-2-glycoprotein I antibodies, or both, is necessary for diagnosing APLS in combination with clinical criteria. In order to exclude clinically insignificant or transient antibodies, the standards further demand that a subsequent APLA test must be positive and conducted 12 weeks after the first positive test.&nbsp;</p>

<p>The diagnosis of APLS is controversial if that period is shorter than 12 weeks or if more than 5 years pass between two distinct clinical symptoms and positive laboratory testing.&nbsp;<br />
&nbsp;</p>

<h5><strong>Lupus Anticoagulant Test</strong></h5>

<p>The biggest predictor of unfavorable pregnancy-related outcomes is the lupus anticoagulant test. When predicting thrombosis, it is less sensitive but more specific than the anticardiolipin antibodies test. 20% of patients with anticardiolipin antibodies also have a positive lupus anticoagulant test, and 80% of patients with a positive lupus anticoagulant test also have anticardiolipin antibodies<sup><a href="#accordion">[1]</a></sup>.&nbsp;</p>

<p>The presence of lupus anticoagulants indicates a coagulation inhibitor of phospholipid-dependent coagulation events. It does not directly interact with coagulation factors and is not linked to problems with bleeding. Patients who are taking heparin or warfarin may get false-positive and false-negative results.<br />
&nbsp;</p>

<h5><strong>Anticardiolipin And Anti-Beta-2-Glycoprotein I Antibodies</strong></h5>

<p>A typical method is the enzyme-linked immunosorbent assay (ELISA), which is used to measure anticardiolipin antibodies and anti-beta-2-glycoprotein I antibodies. Compared to IgM or IgA antibodies, IgG antibodies correlate more strongly with clinical symptoms. Lesser titers have a less well-established connection with thrombotic events than titers greater than 40 GPL units(One GPL unit =1 microgram of IgG antibody).<br />
&nbsp;</p>

<h5><strong>Other Laboratory Findings</strong></h5>

<p>In APLS, anemia or thrombocytopenia are frequently observed. Renal dysfunction with thrombotic microangiopathy may be indicated by renal failure and proteinuria. The acute thrombotic incident may cause a high erythrocyte sedimentation rate (ESR). The rest of the time, inflammation-related indicators are often normal.</p>

<div>&nbsp;</div>

Diagnosis

<p>The diagnosis of the condition is based on:</p>

<p>&nbsp;</p>

<h5><strong>Classification Criteria</strong></h5>

<p>The Sapporo criteria, the first and primary classification standards, were established in 1999 and were then modified in 2006. At least one clinical and one laboratory requirement must be satisfied in order to meet the updated Sapporo categorization criteria for APLS<sup><a href="#accordion">[1]</a></sup>.</p>

<p>&nbsp;</p>

<h5><strong>Clinical Criteria</strong></h5>

<p>To diagnose antiphospholipid antibody syndrome, one of the subsequent clinical symptoms should be confirmed.</p>

<p>&nbsp;</p>

<h5><strong>1. Vascular Thrombosis</strong></h5>

<p>One or more episodes of organ thrombosis in any of the arterial, venous, or smaller vessels. Using the proper imaging or histology, thrombosis must be conclusively proven. Thrombosis must be there for histopathology without considerable vessel wall inflammation.</p>

<ul>
	<li>As long as adequate diagnostic tools correctly proved it and there was no other reason for thrombosis, a prior thrombotic episode may be considered a factor.</li>
	<li>It is forbidden to include superficial venous thrombosis as a factor.</li>
</ul>

<h5>&nbsp;</h5>

<h5><strong>2. Pregnancy Morbidity&nbsp;</strong></h5>

<ul>
	<li>
	<p>As determined by ultrasound or physical examination, one or more unexplained fetal deaths of morphologically healthy fetuses occur at or after 10 weeks of gestation.</p>
	</li>
	<li>
	<p>Premature birth of one or more morphologically healthy newborns before the 34th week of pregnancy. Either severe preeclampsia, eclampsia, or placental insufficiency must have preceded the premature birth.</p>
	</li>
	<li>After eliminating any anatomical or hormonal abnormalities in the mother as well as parental genetic reasons, there have been three or more consecutive sudden abortions well before the 10th week of gestation.</li>
</ul>

<p>&nbsp;</p>

<h5><strong>Laboratory Criteria</strong></h5>

<p>The antiphospholipid antibody syndrome should be identified based on one of the following laboratory results.</p>

<ul>
	<li>Presence of lupus anticoagulant in plasma on blood tests done at least 12 weeks apart (blood tests should be repeated for confirmation</li>
	<li>Detection of moderate to high titers of IgG or IgM anticardiolipin antibodies in serum or plasma tests, as determined by conventional ELISA. The tests need to be repeated two or more times, spaced at least 12 weeks apart from each other for confirmation.</li>
	<li>Detection of moderate to high titers of IgG or IgM anti-beta-2-glycoprotein I antibodies in serum or plasma, as determined by conventional ELISA, on two or more occasions, at least 12 weeks apart.</li>
</ul>

<p>&nbsp;</p>

<p>Blood clots can have detrimental effects. Thus, it&#39;s crucial to make an accurate antiphospholipid syndrome (APS) diagnosis. The results of particular blood tests and a medical evaluation are used to make the diagnosis of APS.</p>

<p>You will often be sent to the hospital to see one of the following if APS is suspected:</p>

<ul>
	<li>A specialist in conditions affecting the blood, known as a hematologist.&nbsp;</li>
	<li>A specialist in conditions affecting the immune system, known as a rheumatologist</li>
</ul>

<p>&nbsp;</p>

<h5><strong>Special Blood Tests</strong></h5>

<p>Blood must be examined for the antiphospholipid antibodies that raise the risk of blood clots in order to diagnose APS. A blood test made particularly to detect these antibodies is necessary for this.</p>

<p>Only after two abnormal blood test findings with a minimum 12-week interval between them can APS be diagnosed. This is due to the fact that inadvertent antiphospholipid antibodies can occasionally form in the body for brief periods of time.</p>

<p>Typically, this is a side effect of a drug, like an antibiotic, or the outcome of an infection.</p>

<p>In the event that antiphospholipid antibodies are found during the initial blood test, a subsequent test will be required to determine whether the faulty antibodies are still present.<br />
&nbsp;</p>

<h5><strong>Medical Evaluation</strong></h5>

<p>If blood tests reveal you have APS, your medical history will be carefully reviewed to see if you have ever had any symptoms that could have been brought on by APS.</p>

<p>&nbsp;</p>

<p>If you have experienced one or more blood clots that have been verified and, if applicable:</p>

<ul>
	<li>One or more late miscarriages occurred after week 10 of your pregnancy, or without apparent cause.&nbsp;</li>
	<li>At least one premature delivery occurs at or before week 34 of your pregnancy.</li>
	<li>Three or more unexplained, early miscarriages before the 10th week of pregnancy.&nbsp;</li>
</ul>

<div>&nbsp;</div>

Treatment

<p>Minimizing other thrombophilic risk factors in APS patients with a high level of APL is the first line of therapy. Regular exercise, weight loss, and cholesterol-lowering drugs should be promoted along with quitting smoking. The foundation of treatment plans for APS include reducing thrombosis, fetal loss, and non-thrombotic neurologic symptoms.</p>

<p>&nbsp;</p>

<h5><strong>Your Treatment Plan</strong></h5>

<p>Most APS patients must take daily anticoagulant or antiplatelet medicine for the rest of their lives. Low-dose aspirin tablets are typically advised if blood testing reveals abnormal antiphospholipid antibodies but no history of blood clots. If you cannot take aspirin or clopidogrel, an alternate antiplatelet pill may be recommended.</p>

<p>If you suffer from APS and have a tendency to have blood clots (past history of a stroke or DVT), warfarin tablets are frequently advised. However, this needs to be modified if you become pregnant or consider becoming pregnant. If so, let your physician know.</p>

<p>Heparin injections may be required if you form a blood clot or if your symptoms worsen unexpectedly. You may be instructed to administer these injections yourself or receive them in a hospital setting.</p>

<p>&nbsp;</p>

<h5><strong>Treatment Following Thrombotic Episodes</strong></h5>

<p>Fibrinolytic therapy with streptokinase and anticoagulants is used to treat acute thrombotic episodes in APS when necessary. Unlike venous thromboembolism which accounts for less than 1% of fatalities, arterial thrombosis carries a substantially higher risk of morbidity and death<sup><a href="#accordion">[3]</a></sup>. Cerebral ischemia may require more severe and protracted anticoagulant therapy. A long course of anticoagulants is typically advised because of the possibility of recurrence.</p>

<p>&nbsp;</p>

<h5><strong>Management Of Pregnancy In APS</strong></h5>

<p>Treatment is most beneficial in improving pregnancy outcomes when started as soon as possible, preferably following conception. The management of pregnancy in APS requires antiplatelet or anticoagulant therapy. This lessens the risk of systemic thrombosis and enhances favorable obstetric and fetal outcomes.&nbsp;</p>

<p>Presently, the preferred course of treatment is heparin and low-dose aspirin. In low-risk individuals, low-dose aspirin is a safe and effective prenatal prophylactic, as indicated by the increase in live birth rates to 75% in one trial<sup><a href="#accordion">[3]</a></sup>. It is typical to begin aspirin or heparin therapy from the beginning of pregnancy and to sustain it for up to six weeks after birth.</p>

<p>Warfarin is not recommended during pregnancy since it carries a small risk of birth defects but is advised postpartum to reduce the risks of heparin-induced osteoporosis, fractures, and thrombocytopenia.</p>

<p>Prednisone and prednisolone are two corticosteroids that may be used in more complicated cases of recurrent miscarriage.</p>

<p>&nbsp;</p>

<h5><strong>Complications Associated With Treatment</strong></h5>

<p>The most frequent side effect of anticoagulant treatment is bleeding. Reducing the dosage of the anticoagulant helps stop bleeding. These drugs occasionally cause moderate side effects, like indigestion or feeling unwell (nausea). However, there is a chance that the impairment of blood clotting will result in severe bleeding (a hemorrhage).</p>

<p>&nbsp;</p>

<p>Excessive bleeding can cause the following symptoms:</p>

<ul>
	<li>Blood in the urine or feces</li>
	<li>Black feces</li>
	<li>Severe bruising</li>
	<li>Persistent nosebleeds (lasting longer than 10 minutes)</li>
	<li>Blood in vomit</li>
	<li>Spitting blood during cough (hemoptysis)</li>
</ul>

<p><strong>Note: </strong>If you experience any of these symptoms while receiving an anticoagulant, speak with your doctor immediately.</p>

<p>&nbsp;</p>

<h5><strong>Catastrophic Anti-Phospholipid Syndrome (CAPS) Management</strong></h5>

<p>The significant mortality rate linked with CAPS necessitates early diagnosis in order to manage the condition effectively. For CAPS management IVIG (Intravenous immunoglobulin), plasmapheresis, cyclophosphamide, rituximab, or eculizumab are used with anticoagulation and high-dose corticosteroids.</p>

<div>&nbsp;</div>

<p>Catastrophic antiphospholipid syndrome (CAPS) is an uncommon and potentially fatal consequence that requires immediate medical attention. It happens in less than 1% of patients with APS<sup><a href="#accordion">[1]</a></sup>.</p>

<p>Blood clots abruptly grow all over the body in people with CAPS, leading to the failure of several organs. One in five cases occurs after an illness, trauma, or surgery, while the precise cause is unknown<sup><a href="#accordion">[6]</a></sup>. Depending on the organs affected, early symptoms can vary greatly.</p>

<p>&nbsp;</p>

<p>Some signs could be:</p>

<ul>
	<li>Reduction of blood flow to the tips of your fingers or toes, causing them to turn dark blue or black (people with black or brown skin may find it difficult to notice this)</li>
	<li>Enlarged hands, feet, or ankles</li>
	<li>Difficulty breathing</li>
	<li>Abdominal or tummy&nbsp;pain</li>
	<li>Blood in urine</li>
	<li>Confusion</li>
	<li>Seizures&nbsp;</li>
	<li>Convulsions&nbsp;</li>
	<li>Coma</li>
</ul>

<p>The symptoms typically appear suddenly and worsen quickly. If you or a loved one has APS and it suddenly worsens, call for an ambulance immediately. Individuals with CAPS must be admitted immediately to an intensive care unit (ICU) to support the body&#39;s processes.</p>

<p>Anticoagulants used at high doses prevent blood clots from growing while being slowly distributed in the body. However, even with the greatest available care, 1 in 2 persons can pass away from the illness<sup><a href="#accordion">[6]</a></sup>.</p>

<p>&nbsp;</p>

<p>Complications of the antiphospholipid syndrome can include:</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Kidney Failure</strong></h5>
	</li>
</ul>

<p>Reduced blood supply to your kidneys due to the tendency of clot formation.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Central Nervous System</strong></h5>
	</li>
</ul>

<p>A stroke can be brought on by reduced blood supply to a portion of the brain, leading to long-term neurological impairment such as partial paralysis and speech loss.&nbsp;</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Dermatology</strong></h5>
	</li>
</ul>

<p>Livedo reticularis, a lace-like, purple cutaneous lesion most frequently encountered on the extremities due to superficial venous thrombosis and thrombo-phlebitis is the distinguishing feature of skin symptoms of APS. Skin nodules, recurrent leg ulcers, and peripheral gangrene can also develop.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Cardiovascular Problems</strong></h5>
	</li>
</ul>

<p>The valves in the veins, which keep blood flowing to your heart, can be harmed by a blood clot in your leg. Your lower legs may become permanently discolored and swollen as a result. The possibility of cardiac damage is another risk. The most often reported cardiac abnormalities in APS patients include mitral regurgitation, thickness, stenosis, and vegetation growth.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Lung Problems</strong></h5>
	</li>
</ul>

<p>These include pulmonary embolism and elevated blood pressure in the lungs.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Obstetrical Complications</strong></h5>
	</li>
</ul>

<p>These can include pregnancy-related high blood pressure (preeclampsia), delayed fetal growth, preterm birth, stillbirth, and miscarriages. Pregnancy loss in APS typically happens during the first or second trimester. Untreated patients have a fetal loss rate of 25&ndash;61%<sup><a href="#accordion">[3]</a></sup>. However, one study put that number as high as 90%<sup><a href="#accordion">[3]</a></sup>. In severe cases, the antiphospholipid syndrome can quickly cause damage to many organs, however, this is a rare occurrence.</p>

<div>&nbsp;</div>

<p>These include:</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Being Female</strong></h5>
	</li>
</ul>

<p>Antiphospholipid syndrome is more common in women, as almost 70% of patients with antiphospholipid syndrome are women<sup><a href="#accordion">[5]</a></sup>.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong><a href="https://www.merisehat.pk/disease/polycystic-ovary-syndrome-pcos" target="_blank">Polycystic Ovary Syndrome (PCOS)</a></strong></h5>
	</li>
</ul>

<p>PCOS can be classified as an autoimmune disorder because of the low levels of progesterone that overstimulate the immune system and cause the creation of autoantibodies. However, there is a link between PCOS and autoimmune conditions such as antinuclear antibodies (ANA) and thyroid peroxidase antibodies (anti-TPO), which have been linked to Hashimoto thyroiditis and systemic lupus erythematosus, respectively.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Having Other Autoimmune Diseases</strong></h5>
	</li>
</ul>

<p>Antiphospholipid syndrome is a condition that affects 30 to 40% of persons with the autoimmune disease systemic lupus erythematosus(SLE)<sup><a href="#accordion">[5]</a></sup>.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Having A&nbsp;Rheumatic Condition</strong></h5>
	</li>
</ul>

<p>Your joints, bones, and muscles are all affected by rheumatic diseases. Antiphospholipid syndrome is more common in those with rheumatic diseases.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Having Antiphospholipid Syndrome Run In The Family</strong></h5>
	</li>
</ul>

<p>In some instances, the syndrome runs in families.</p>

<div>&nbsp;</div>

<p>Preventing blood clots is the primary goal of treating antiphospholipid syndrome. Blood thinners can help prevent blood clots in the majority of cases. However, several other illnesses can also increase your chances of blood clots.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong><a href="https://www.merisehat.pk/disease/diabetes-type-1-and-2" target="_blank">Diabetes</a></strong></h5>
	</li>
</ul>

<p>Diabetes raises your risk of developing artery plaque accumulation, which can result in blood clots. If you have antiphospholipid syndrome, it&#39;s critical to control your diabetes appropriately.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>High Blood Pressure (<a href="https://www.merisehat.pk/disease/hypertension-57">Hypertension</a>)</strong></h5>
	</li>
</ul>

<p>In the arteries that supply blood to your brain, high blood pressure can lead to the formation of blood clots and result in a stroke. If you have antiphospholipid syndrome, controlling your blood pressure is crucial.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Elevated <a href="https://www.merisehat.pk/disease/cholesterol#causes-5532" target="_blank">Cholesterol</a></strong></h5>
	</li>
</ul>

<p>Blood clots in your legs may develop as a result of high cholesterol. If you have antiphospholipid syndrome, controlling your cholesterol is crucial.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong><a href="https://www.merisehat.pk/disease/obesity" target="_blank">Obesity</a></strong></h5>
	</li>
</ul>

<p>Obesity encourages an inflammatory state in the body that might help blood clot formation. If you have antiphospholipid syndrome, striving to keep a healthy weight is crucial.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Smoking</strong></h5>
	</li>
</ul>

<p>Smoking can harm blood arteries and raise your chance of blood clots. It is imperative to give up smoking if you have antiphospholipid syndrome.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Birth Control Or&nbsp;Oestrogen Treatment For Menopause (Contraception)</strong></h5>
	</li>
</ul>

<p>Your chance of getting a blood clot rises when estrogen levels rise.&nbsp;Please discuss the best birth control method or menopausal treatment for you with your healthcare professional.</p>

<p>&nbsp;</p>

<ul>
	<li>
	<h5><strong>Rheumatological Conditions Or Additional Autoimmune Diseases</strong></h5>
	</li>
</ul>

<p>To stay healthy and avoid blood clots, you must address any autoimmune diseases or rheumatic conditions you may have.</p>

<div>&nbsp;</div>

<h5><strong>Neurological And Neuropsychiatric Manifestations Of Antiphospholipid-Antibody Syndrome (APS)<sup><a href="#accordion">[7]</a></sup></strong></h5>

<p>The purpose of this study was to present the numerous neurological and neuropsychiatric signs of APS, their etiology, and their treatment. Rarely apparent at presentation, these non-thrombotic, non-criteria neuropsychiatric signs e.g. cognitive decline, memory loss, abrupt psychosis, behavioral abnormalities, etc. are frequently misunderstood or recognized later when thrombotic events occur. Hence it is advisable to consider a differential of APS if these symptoms are present in a patient.</p>

<p><strong>Note:</strong> APS patients&#39; course of treatment is determined on a case-by-case basis based on the risk-benefit balance of the chosen therapy.</p>

<div>&nbsp;</div>

Key Points

<ul>
	<li>An immune system illness called antiphospholipid syndrome (APS) also referred to as Hughes syndrome, raises the risk of blood clots</li>
	<li>There are three types of APS - primary, secondary, and catastrophic</li>
	<li>Antiphospholipid Syndrome symptoms and signs depend upon the severity of the disease. Patients most commonly present with deep vein thrombosis (DVT), heart attacks, and pregnancy complications&nbsp;</li>
	<li>The treatment option is based on the type of APS and the organ(s) affected due to APS.</li>
</ul>

<div>&nbsp;</div>

[1] Bustamante JG, Goyal A, Singhal M. Antiphospholipid syndrome. In: StatPearls [Internet]. StatPearls Publishing; 2021.

[2] Stephenson MD. Frequency of factors associated with habitual abortion in 197 couples. Fertil Steril. 1996 Jul;66(1):24–9.

[3] Durrani OM, Gordon C, Murray PI. Primary anti-phospholipid antibody syndrome (APS): current concepts. Surv Ophthalmol. 2002;47(3):215–38.

[4] Cervera R. Antiphospholipid syndrome. Thromb Res. 2017;151:S43--S47.

[5] Antiphospholipid syndrome: Symptoms, diagnosis & treatment. Cleveland Clinic.

[6] Antiphospholipid Syndrome (APS). NHS choices.

[7] Asif S, Bali A, Dang AK, Gonzalez DA, Kumar R. Neurological and Neuropsychiatric Manifestations of Antiphospholipid-Antibody  Syndrome (APS). Cureus. 2022 Jun;14(6):e26022.