Secondary Conditions

Posted in Lupus Info on 5/25/2010

Although lupus can be well controlled in many people, serious medical conditions caused by or associated with the disease can still occur. It is important that you know about these conditions and how they may make you feel so that you can call your doctor right away. The sooner a problem is detected and evaluated, the sooner it can be treated to prevent or reduce damage to your body’s organs.

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  • Lupus Nephritis (Kidney Disease)

Many people with lupus develop some form of mild kidney disease. Others, however, develop kidney disease serious enough to lead to kidney failure.

Warning signs can include swelling around your ankles, hands, and eyes; increased fatigue or tiredness, and increased need to urinate at night.

Lupus nephritis may cause weight gain, high blood pressure, dark urine, or swelling around the eyes, legs, ankles, or fingers. However, some people with SLE have no overt symptoms of kidney disease, which must be diagnosed by blood and urine tests. Treatment depends on the symptoms and test results. Medicines called corticosteroids can decrease swelling and inflammation by suppressing the immune system. Additional immunosuppressive drugs related to cancer and drugs used to prevent rejection of organ transplants may also be used. In severe cases, your doctor may prescribe cyclophosphamide (Cytoxan, Neosar) or mycophenolate (CellCept). Newer experimental treatments include a drug called rituximab (Rituxan). You may need one or more medicines to control your blood pressure as well as limiting protein, sodium and potassium in your diet.

  • Pericarditis

An inflammation of the thin sac that surrounds the heart.

Warning signs include: chest pain, shortness of breath and new or higher than usual fever.

  • Myocarditis

An inflammation of the heart muscle.

Warning signs include: chest pain, shortness of breath, and new or higher-than-usual fever.

  • Atherosclerosis

This is a condition in which fatty deposits build up on the inside of arteries. These deposits can reduce or block blood flow. A blockage or reduced blood flow through an artery that supplies the heart can cause a heart attack to occur.

Warning signs include: burning, choking, squeezing, or pressing chest pain felt in the center of the chest that may radiate to the left shoulder and arm (anginal pain.) This can last up to 5 minutes and will become much less intense or go away completely if you rest; crushing, prolonged chest pain that is not relieved by rest; shortness of breath; unrelieved indigestion; and a weak or faint feeling.

High blood cholesterol may promote the formation of artherosclerotic plaque in the arteries of the heart as well as in other arteries. This may lead to cardiovascular disease and increase ones risk of a heart attack or stroke. Everyone of us needs to contend with this health issue, but patients with who have had the disease for at least 5 to 10 years appear to be at increased risk for cardiovascular disease, particularly for myocardial infarction. This could be due to inflammation, steroids or kidney disease. Scientists are working to understand why lupus patients are more prone to develop vascular disease.

The first study ever on children with lupus is still being conducted at this time, known as the APPLE study which stands for Atherosclerosis Prevention in Pediatric Lupus Erythematosus. This study hopes to find the answers to these exact questions. To see information on the study and its results please click here

  • Pleuritis

An inflammation of the lining of the lung. Warning signs include: shortness of breath, and chest pain, especially when taking a deep breath.

  • Central Nervous System (CNS)

CNS disease covers a variety of problems that may or may not be related to lupus. Problems can include seizures, memory loss, headache, confusion, hearing and visual changes, muscle weakness, depression, and emotional disturbances. Because many of these problems can be related to use of medications or indicate other conditions, it is often difficult to make a definite diagnosis of CNS disease.

Warning signs include: severe or chronic headaches; seizures; periods of forgetfulness, restlessness, or confusion; new or increased hearing and vision problems; bizarre or erratic changes in behavior; mood swings; and signs of a stroke, including weakness or numbness in the arms, legs, face, or down one side of the body; a change in speech; confusion; or severe headaches.

  • Depression

With depression, people may feel helpless, hopeless, or overwhelmed. They may find it difficult to get through the day. Depression can occur as a result of lupus or be caused by the drugs used to treat it, especially cortico-steroids.

Warning signs include: depressed mood; significant weight loss or gain; trouble sleeping or sleeping too much; extreme tiredness and lack of energy; decreased concentration or an inability to make a decision; feelings of being overwhelmed and unable to carry out simple tasks, such as personal hygiene, housework, or childcare; feelings of hopelessness about various aspects of life; unusual anger or irritability; and recurrent thoughts of death and suicide.

  • Osteonecrosis

This is a condition that usually affects the hip joint, but may occur in other joints such as the knees, ankles, or shoulders. Blood supply to the hip is reduced and, over time, leads to severe degenerative arthritis. Osteonecrosis is considered to be a side effect of corticosteroid therapy and not a manifestation of SLE itself.

Warning signs include: sharp pain in the groin or buttocks that may radiate down the back of the leg, decreased exercise tolerance, stiffness of the hips, and increased pain and difficulty in walking after exercise.

  • Pancreatitis

In pancreatitis, the pancreas (an organ involved in digestion and in producing hormones that regulate blood sugar levels) becomes inflamed. It is a very serious problem that must be treated immediately.

Warning signs include: sharp, intense pain at the level of the belly button that radiates around to the back; nausea and vomiting; and new or higher-than-usual fever.

  • Acute Abdomen

This is a condition that describes the sudden onset of abdominal pain. A variety of serious problems can cause this condition. You should see your doctor immediately if you develop acute abdomen.

Warning signs include: abdominal pain that may be severe; nausea, vomiting, or loss of appetite; change in usual bowel movements; and vomiting blood or blood in the stool.

  • Vision Problems

Changes in vision can be a result of lupus or because of the corticosteroids and antimalarials used to treat lupus. Problems can include inflammation of the eye, glaucoma, cataracts, general changes in vision, and blocked tear ducts. On very rare occasions, blindness can result. In many cases the antimalarials are stopped for a period of time.

Warning signs include: development of a rash over the eyelids; mucus discharge from the eye; blurred vision; sensitivity to light; headaches; a sore, red eye; lack of tears, and eyes that hurt and are dry; and episodes of flashing lights and partial blindness.

  • Scleroderma

Derived from the Greek words “sklerosis,” meaning hardness, and “derma,” meaning skin, scleroderma literally means hard skin. Although it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is believed to be an autoimmune disease. In scleroderma, the immune system is thought to stimulate cells called fibroblasts so they produce too much collagen. The collagen forms thick connective tissue that builds up within the skin and internal organs and can interfere with their functioning. Blood vessels and joints can also be affected.

Although scleroderma is more common in women, (7 to 12 times more often than men) the disease also occurs in men and children. It affects people of all races and ethnic groups. For some people, scleroderma (particularly the localized forms) is fairly mild and resolves with time. But for others, living with the disease and its effects day to day has a significant impact on their quality of life.

  • Sjogren`s Syndrome

Sjogren`s (SHOW-grens), Is another autoimmune disease in which disease-fighting cells attack various organs, most notably the glands that produce tears and saliva (the lacrimal and salivary glands). Damage to these glands causes a reduction in both the quantity and quality of their secretions. This results in symptoms that include dry eyes and dry mouth. In technical terms, the form of eye dryness associated with Sjögren’s syndrome is called keratoconjunctivitis sicca, or KCS, and the symptoms of dry mouth are called xerostomia. Your doctor may use these terms when talking to you about Sjögren’s syndrome.

Sjögren’s syndrome can cause many symptoms. The main ones are: Dry eyes – Eyes affected by Sjögren’s syndrome may burn or itch. Some people say it feels like they have sand in their eyes. Others have trouble with blurry vision, or are bothered by bright light, especially fluorescent lighting. Dry mouth – Dry mouth may feel chalky or like your mouth is full of cotton. It may be difficult to swallow, speak, or taste. Because you lack the protective effects of saliva, you may develop more dental decay (cavities) and mouth infections. As noted above, both primary and secondary Sjögren’s syndrome can also affect other parts of the body, causing symptoms such as: multiple sites of joint and muscle pain, prolonged dry skin, skin rashes on the extremities, chronic dry cough, vaginal dryness, numbness or tingling in the extremities and prolonged fatigue that interferes with daily life.

  • Avascular Necrosis

also known as Osteonecrosis is a disease resulting from the temporary or permanent loss of blood supply to the bones. Without blood, the bone tissue dies, and ultimately the bone may collapse. If the process involves the bones near a joint, it often leads to collapse of the joint surface. Other names are avascular necrosis, aseptic necrosis, and ischemic necrosis. Although it can happen in any bone, osteonecrosis most commonly affects the ends (epiphysis) of the femur, the bone extending from the knee joint to the hip joint. Other common sites include the upper arm bone, knees, shoulders, and ankles. The disease may affect just one bone, more than one bone at the same time, or more than one bone at different times. The amount of disability that results from osteonecrosis depends on what part of the bone is affected, how large an area is involved, and how effectively the bone rebuilds itself. Normally, bone continuously breaks down and rebuilds - old bone is replaced with new bone. This process - which takes place after an injury as well as during normal growth - keeps the skeleton strong and helps it to maintain a balance of minerals. In the course of osteonecrosis, however, the healing process is usually ineffective and the bone tissues break down faster than the body can repair them. If left untreated, the disease progresses, the bone collapses, and the joint surface breaks down, leading to pain and arthritis. In the early stages of osteonecrosis, people may not have any symptoms. As the disease progresses, however, most experience joint pain. At first, the pain occurs only when putting weight on the affected joint. Later, it occurs even when resting. Pain usually develops gradually, and may be mild or severe. Pain may be severe enough to limit range of motion in the affected joint. In some cases, particularly those involving the hip, disabling osteoarthritis may develop. Severe cases involve joint replacement.

  • Fibromyalgia

Fibromyalgia syndrome is a common and chronic disorder characterized by widespread pain, diffuse tenderness, and a number of other symptoms. Although fibromyalgia is often considered an arthritis-related condition, it is not truly a form of arthritis because it does not cause inflammation or damage to the joints, muscles, or other tissues. Like arthritis, however, fibromyalgia can cause significant pain and fatigue, and it can interfere with a person’s ability to carry on daily activities. Fibromyalgia is considered a rheumatic condition.

In addition to pain and fatigue, people who have fibromyalgia may experience a variety of other symptoms including: cognitive and memory problems (sometimes referred to as “fibro fog”), sleep disturbances, morning stiffness, headaches, irritable bowel syndrome, painful menstrual periods, numbness or tingling of the extremities, restless legs syndrome, temperature sensitivity, sensitivity to loud noises or bright lights.

The causes of fibromyalgia are unknown, but there are probably a number of factors involved. Many people associate the development of fibromyalgia with a physically or emotionally stressful or traumatic event, such as an automobile accident. Some connect it to repetitive injuries. Others link it to an illness. For others, fibromyalgia seems to occur spontaneously. Many researchers are examining other causes, including problems with how the central nervous system processes pain.

Some scientists speculate that a person’s genes may regulate the way his or her body processes painful stimuli. According to this theory, people with fibromyalgia may have a gene or genes that cause them to react strongly to stimuli that most people would not perceive as painful. There have already been several genes identified that occur more commonly in fibromyalgia patients, and NIAMS-supported researchers are currently looking at other possibilities.

Scientists estimate that fibromyalgia affects 5 million Americans age 18 or older. For unknown reasons, between 80 and 90 percent of those diagnosed with fibromyalgia are women; however, men and children also can be affected. Most people are diagnosed during middle age, although the symptoms often become present earlier in life.

People with certain rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus (lupus), or ankylosing spondylitis (spinal arthritis) may be more likely to have fibromyalgia, too.Several studies indicate that women who have a family member with fibromyalgia are more likely to have fibromyalgia themselves, but the exact reason for this—whether it be heredity, shared environmental factors, or both—is unknown.

Fibromyalgia can be difficult to treat. Not all doctors are familiar with fibromyalgia and its treatment, so it is important to find a doctor who is. Many family physicians, general internists, or rheumatologists (doctors who specialize in arthritis and other conditions that affect the joints or soft tissues) can treat fibromyalgia. Fibromyalgia treatment often requires a team approach, with your doctor, a physical therapist, possibly other health professionals, and most importantly, yourself, all playing an active role. It can be hard to assemble this team, and you may struggle to find the right professionals to treat you. When you do, however, the combined expertise of these various professionals can help you improve your quality of life.

Only three medications, duloxetine (Cymbalta1), milnacipran (Savella), and pregabalin (Lyrica) are approved by the U.S. Food and Drug Administration (FDA) for the treatment of fibromyalgia. Cymbalta was originally developed for and is still used to treat depression. Savella is similar to a drug used to treat depression, but is FDA approved only for fibromyalgia. Lyrica is a medication developed to treat neuropathic pain (chronic pain caused by damage to the nervous system).

Other drugs that are used are analgesics (painkillers) which range from over-the-counter acetaminophen (Tylenol) to prescription medicines, and even stronger narcotic preparations. However, there is no solid evidence showing that for most people narcotics actually work to treat the chronic pain of fibromyalgia.

Another option is nonsteroidal anti-inflammatory drugs, including aspirin, ibuprofen (Advil, Motrin), and naproxen sodium (Anaprox, Aleve). Although inflammation is not a symptom of fibromyalgia, NSAIDs also relieve pain. These medications, some of which are available without a prescription, may help ease the muscle aches of fibromyalgia. They may also relieve menstrual cramps and the headaches often associated with fibromyalgia.

Perhaps the most useful medications for fibromyalgia are several in the antidepressant class. These drugs work equally well in fibromyalgia patients with and without depression, because antidepressants elevate the levels of certain chemicals in the brain (including serotonin and norepinephrine) that are associated not only with depression, but also with pain and fatigue. Increasing the levels of these chemicals can reduce pain in people who have fibromyalgia. Doctors prescribe several types of antidepressants for people with fibromyalgia.

Benzodiazepines can sometimes help people with fibromyalgia by relaxing tense, painful muscles and stabilizing the erratic brain waves that can interfere with deep sleep. Benzodiazepines also can relieve the symptoms of restless legs syndrome, a neurological disorder that is more common among people with fibromyalgia. The disorder is characterized by unpleasant sensations in the legs and an uncontrollable urge to move the legs, particularly when at rest, in an effort to relieve these feelings. Doctors usually prescribe benzodiazepines only for people who have not responded to other therapies because of the potential for addiction. Benzodiazepines include Klonopin and Valium.

  • Raynaud`s

Raynaud’s phenomenon is a condition that affects the blood vessels in the extremities—generally, the fingers and toes. It is characterized by episodic attacks, called vasospastic attacks, in which the blood vessels in the digits (fingers and toes) constrict (narrow), usually in response to cold temperatures and/or emotional stress. When this condition occurs on its own, it is called primary Raynaud’s phenomenon. When it occurs with another condition such as scleroderma or lupus, it is called secondary Raynaud’s phenomenon.

Attacks of Raynaud’s phenomenon are caused by an intensification of the body’s natural response to cold. When a person is exposed to cold, the body’s normal response is to slow the loss of heat and preserve its core temperature. Blood vessels in the surface of the skin are called thermoregulatory vessels because they react to changes in the ambient temperature. To maintain normal core temperature, these specialized blood vessels in the skin surface constrict and move blood from arteries near the surface to veins deeper in the body. But for people who have Raynaud’s phenomenon, the thermoregulatory vessels overreact to cold exposure with sudden and intense spasmodic contractions of these small blood vessels that supply blood to the skin of the fingers, toes, ears, face, and other body areas.

Once an attack begins, a person may experience three phases (though not all people have all three) of skin color changes—typically from white to blue to red—in the fingers or toes. Whiteness (called pallor) may occur in response to spasms of the arterioles (small branches of an artery) and the resulting collapse of the arteries supplying the fingers and toes. Blueness (cyanosis) may appear because the fingers or toes are not getting enough oxygen-rich blood. Finally, as the arterioles dilate (relax) and blood returns to the digits, redness (rubor) may occur. During the attack, the fingers or toes may feel cold and numb as blood flow to them is interrupted. As the attack ends and blood flow returns, fingers or toes may throb and tingle. Typically, the blood flow to the skin will remain low until the skin is rewarmed. After warming, it usually takes 15 minutes to recover normal blood flow to the skin.

The aim of treatment are to reduce the number and severity of attacks and to prevent tissue damage and loss of tissue in the fingers and toes. Most doctors are conservative in treating patients with primary Raynaud’s phenomenon because they do not get tissue damage. For these patients, doctors tend to recommend nondrug treatments before moving onto medications. For patients with secondary Raynaud’s phenomenon, medications are more often prescribed, because severe attacks with ulcers or tissue damage are more likely.

In the most severe cases, Raynaud’s causes ulcers and serious tissue damage that does not respond to medications. Doctors may use a surgical procedure called a digital sympathectomy with adventitial stripping (which involves removing the tissue and nerves around the blood vessels supplying the affected digits). Although this procedure may result in reducing symptoms and healing tissue, it only helps temporarily and therefore is reserved for difficult cases.

The most common treatments and self-help measures are Nondrug Treatments and Self-Help Measures such as:

    • Take action during an attack. You can decrease both its length and severity by a few simple actions. The first and most important one is avoid the cold. Warming the body and the hands or feet is also helpful. If you’re outside and the weather is cold, go indoors. Run warm water over your fingers or toes or soak them in a bowl of warm water to warm them. If a stressful situation triggers the attack, get out of the stressful situation, if possible, and relax. Although biofeedback and similar nondrug methods are used, formal studies have suggested they are not helpful.
    •Keep warm. It is important not only to keep the extremities warm, but also to avoid chilling any part of the body. Remember, a drop in the body’s core temperature triggers the attack. Shifting temperature (for example, rapidly moving from 90 degrees outside to a 70-degree air-conditioned room) and damp rainy weather are to be avoided. In cold weather, pay particular attention to dressing. Several layers of loose clothing, socks, hats, and gloves or mittens are recommended. A hat is important because a great deal of body heat is lost through the scalp. Keep feet warm and dry. Some people find it helpful to wear mittens and socks to bed during the winter. Chemical warmers, such as small heating pouches that can be placed in pockets, mittens, boots, or shoes, can give added protection during long periods outdoors. People who have secondary Raynaud’s phenomenon should talk to their doctors before exercising outdoors in cold weather.

In warm weather, be aware that air conditioning also can trigger attacks. Setting the thermostat for a higher temperature or wearing a sweater indoors can help prevent an attack. Some people find it helpful to use insulated drinking glasses and to put on gloves before handling frozen or refrigerated foods.

    • Do not smoke. The nicotine in cigarettes causes the skin temperature to drop, which may lead to an attack.
    • Avoid aggravating medications such as vasconstrictors, which cause the blood vessels to narrow. Vasoconstrictors include beta-blockers, many cold preparations, caffeine, narcotics, some migraine headache medications, some chemotherapeutic drugs, and clonidine, a blood pressure medication. Some studies also associate the use of estrogen with Raynaud’s phenomenon.
    • Control stress. Because stress and emotional upsets may trigger an attack, particularly for people who have primary Raynaud’s phenomenon, learning to recognize and avoid stressful situations may help control the number of attacks. Many people have found that relaxation can help decrease the number and severity of attacks. Local hospitals and other community organizations, such as schools, often offer programs in stress management.
    • Exercise regularly. Many doctors encourage patients who have Raynaud’s phenomenon—particularly the primary form—to exercise regularly. Most people find that exercise promotes overall well-being, increases energy level, helps control weight, and promotes cardiovascular fitness and restful sleep. Patients with Raynaud’s phenomenon should talk to their doctors before starting an exercise program.
    • See a doctor. People with Raynaud’s phenomenon should see their doctors if they are worried or frightened about attacks or if they have questions about caring for themselves. They should always see their doctors if episodes occur only on one side of the body (one hand or one foot) and any time one results in sores or ulcers on the fingers or toes.
People with secondary Raynaud’s phenomenon are more likely than those with the primary form to be treated with medications. Many doctors believe that the most effective and safest drugs for Raynaud’s phenomenon are calcium channel blockers such as nifedipine (Procardia) or amlodipine (Norvasc. Some patients have found relief with alpha receptor blockers, which are high blood pressure medications such as prazosin (Minipres) or doxazosin (Cardura). Effects are reported to be modest and side effects are associated with long-term use. To help heal skin ulcers, some doctors prescribe a nonspecific vasodilator (drug that relaxes blood vessels) such as nitroglycerine paste, which is applied to the fingers. Many new agents are being used in cases that do not respond to vasodilator drugs. These include the antidepressant fluoxetine (Prozac); phosphodiesterase inhibitors such as cilostazol (Pletal), pentoxifylline (Trental), and sildenafil (Viagra); and an angiotensin II receptor antagonist (used for blood pressure control), losartan (Cozaar). Patients should keep in mind that the treatment for Raynaud’s phenomenon is not always successful. Often, patients with the secondary form will not respond as well to treatment as those with the primary form of the disorder. In cases of critical digital ischemia (where the blood flow will not return and finger loss may result), intravenous vasodilator therapy is used with prostaglandins such as epoprostenol (Flolan). Patients may find that one drug works better than another. Some people may experience side effects that require stopping the medication. For other people, a drug may become less effective over time. Women of childbearing age should know that the medications used to treat Raynaud’s phenomenon may affect the growing fetus. Therefore, women who are pregnant or who might become pregnant should avoid taking these medications if possible. Interestingly, Raynaud’s phenomenon gets better or goes away during pregnancy.

  • Vasculitis

Vasculitis refers to inflammation of blood vessels; either arteries or veins may be involved. The culprit in lupus vasculitis is an overactive immune system, which generates antibodies and white blood cells that attack the blood vessel. An inflammatory reaction occurs within the blood vessel wall, and this process results in a damaged artery or vein. The clinical consequence of the damage depends on the type of vessel that is involved. If a very small vessel in the skin is damaged, the only manifestation may be a tiny red spot (purpura) in the skin. If vasculitis affects a large thick-walled artery, the outcome relates to an interruption of blood flow as a thrombus may develop at the site of inflammation and occlude the artery. If blood can no longer flow to the area of the body supplied by the inflamed artery, one could develop an infarction. Infarction refers to a state when tissues die because they are deprived of oxygen. Infarctions may occur anywhere in the body, e.g. heart (myocardial infarction), brain (stroke), intestines (bowel infarction), fingers and toes.

Diagnosing vasculitis can sometimes present a challenge to the physician. Manifestations of vasculitis are quite variable. They can include rash (purpura or ulcerations), persistent discoloration of a finger or toe, numbness or tingling, chest pain, stomach pain, or visual disturbances. Most of these symptoms, however, are not specific to vasculitis! Therefore, you should consult your physician, and let your doctor determine how to handle the situation. When vasculitis is present in the skin, rheumatologists and dermatologists can rapidly identify the process. On occasion, a relatively painless skin biopsy might be performed. When vasculitis affects internal blood vessels and organs, the diagnosis is more difficult to make. Depending on the area of suspected involvement, biopsies (nerve biopsy) are sometimes performed, and in other situations, imaging tests of the blood vessels (angiograms) are performed. Because of the urgency of the situation and the need to acutely treat a patient, doctors often make a clinical diagnosis without invasive testing.

Vasculitis can vary tremendously in its severity. Some patients with mild skin vasculitis, consisting of a few purpura, may not require any treatment, whereas others with more severe skin involvement, consisting of ulcerations, may require aggressive therapy with steroids or immunosuppressives. When vasculitis involves internal organs, aggressive therapy is a must. This may take the form of high dose steroids and often an immunosuppressive therapy such as cyclophosphamide. Vasculitis is fortunately a rare complication of SLE. Nevertheless, it can be very serious, and patients need to contact their doctors if they are not feeling well.

Vasculitis represents a family of 15 rare diseases including: Behcet's disease, Buerger's disease, Central Nervous System, Churg Strauss syndrome, Cryoglobulinemia, Giant Cell Arteritis, Henoch-Schönlein purpura, Hypersensitivity vasculitis, Kawasaki disease, Microscopic Polyangiitis, Polyarteritis nodosa, Polymyalgia rheumatica, Rheumatoid vasculitis, Takayasu's arteritis, and Wegener's Granulomatosis. Vasculitis is treated with prescription medications with the goal of stopping the inflammation and relieving the symptoms. Corticosteroid medication and cytotoxic medicines are the drugs that are commonly prescribed. There is no known cure for vasculitis and its course varies from person to person. Some people go into remission. For others, the disease remains chronic with recurring relapses. In rare cases, vasculitis causes severe disability or death.


  • Antiphospholipid Antibody Syndrome

APLS, or the antiphospholipid syndrome, is an immune disorder which leads to problems in the regulation of blood clotting. This leads to serious complications in some people, including strokes, heart attacks, blood clots in large veins which can break off and lodge in the lungs (pulmonary embolus), miscarriages, and other complications of pregnancy such as pre-eclampsia (a condition involving high blood pressure and spilling of protein from the kidneys which can be dangerous to both the baby and the mother). In fact APLS can affect almost any organ or any kind of blood vessel in the body, ranging from tiny blood clots that disrupt the kidneys or the skin to larger clots that affect the eyes or other vital organs.

There are some other problems than can occur in patients with APLS as well, including mottling of the skin, Raynaud's phenomenon and low platelet counts. All of these can also be found in patients with lupus and other autoimmune diseases. APLS is often found in patients with Systemic Lupus (SLE) but may also occur in people who do not have lupus (some of whom may or may not have other autoimmune disorders).

A series of tests have been developed to detect the antibodies found in APLS the most common is called the anticardiolipin test. There are still several lupus anticoagulant tests available in clinical laboratories and they may be very useful in diagnosing APLS if the simpler tests are negative in some patients with this syndrome.

Antibodies are usually made to target foreign invaders, and serve to protect the body against infections and cancers. In a well organized, healthy body, it is important to understand that some autoimmunity is appropriate. Autoimmune diseases such as lupus and APLS develop when autoantibodies (antibodies which target the body itself) get out of hand and begin to cause damage to the body by initiating and perpetuating inflammation around the parts of the body they target, and interfering with the functioning of their target( s). In the case of APLS, there seems to be a twopronged problem, involving direct interference by autoantibodies with blood clotting regulation and possibly increased inflammation in blood vessels as well, especially a certain kind of inflammation process known as complement activation.

Because much remains to be learned about APLS, current treatments are less than optimal. Most people are not known to have antiphospholipid antibodies before a serious blood clot occurs. In patients with lupus, the antibodies are sometimes found before problems occur, but most patients found to have these antibodies will not be in immediate danger. Unfortunately, it is not yet possible to predict, in people with antiphospholipid antibodies, which are likely to suffer from serious problems. The current treatments are also problematic, with a need for careful monitoring and potentially serious side effects. For all of these reasons, most people with this disorder do not receive aggressive treatment until after a life threatening blood clot has occurred. Once treatment is begun, there is no way to know if and when it would be safe to stop it.

It is now well known that the antibodies can come and go and return again in an unpredictable manner, so measuring antibodies is not a very useful way to monitor the need for therapy. In general, most people who have had a serious event are left on anticoagulation indefinitely. Research is going on in many laboratories to try to improve the diagnosis of this disorder and to develop safer long term treatments or, even better, a way to eliminate the risks.

Currently, the most common therapies used are Coumadin (coumarin) and different types of heparin. Coumadin requires close monitoring with blood tests. It is a treatment which lowers the levels of some proteins that regulate blood clotting and its effects are highly dependent on the way that it interacts with other medications and with foods that a person eats. If a person eats a diet that is low in Vitamin K the effects of Coumadin are enhanced. Changes in diet or medications that a person is taking can actually increase the risk that a person might develop a blood clot and/or bleed when they are taking Coumadin for APLS. Blood levels of Comadin's effects on clotting need to be carefully monitored for this reason. An excellent website for patients on Coumadin is provided by Bristol Myers Squibb.

Various formulations of heparin are used for patients with APLS who are pregnant (Coumadin should be avoided in pregnancy, especially in the early months) and/or cannot take Coumadin for any other reason. Other treatments that have been used in APLS include IVIG (intravenous immunoglobulin) and immune suppression (various strategies from steroids such as prednisone or methylprednisolone to Cyclophosphamide have been reported to be successful in some patients).

Plasmapheresis is another procedure that is sometimes used when other treatments are not working in severe cases. It is unfortunate that treatments for APLS are not as well studied as treatments for more common disorders. In fact there are no drugs actually approved by the FDA for this syndrome, and doctors have had to rely on word of mouth or anecdotal reports to a large extent in learning how best to approach treatment of these patients.

APLS: The statistics are:

    • 1-5% of the general population is believed to have APLS.
    • 15-20% of all cases of blood clots in large veins (deep vein thrombosis), including blood clots that go to the lungs (pulmonary embolism) are due to APLS.
    • 10-25% of women with recurrent miscarriages have APLS.
    • 1/3 of strokes occurring in younger people under the age of 50 are due to APLS.
    • APS is a major women’s health issue: 75-90% of those affected by APLS are women.
    • 40-50% of patients with lupus also have APLS.

Clinical Features of APS - People with antiphospholipid antibodies have an increased risk of developing one or more of the following problems:

    • Blood clots in veins, particularly deep vein thrombosis (DVT)
    • Blood clots that go to the lungs (pulmonary embolism)
    • Blood clots in arteries
    • Miscarriages – these can occur at any stage of pregnancy but are most common in the late first trimester or early second trimester
    • Pre-eclampsia, eclampsia, fetal growth retardation, premature delivery
    • Heart attacks, angina
    • Strokes
    • Brief stroke-like episodes called transient ischemic attacks (TIAs), for example, loss of vision
    • Decreased levels of platelets (small blood cells involved in blood clotting)
    • Heart valve problems, sometimes requiring valve surgery or valve replacement
    • Persistent or transient blotchy, lacy bluish rash (called livedo reticularis)
    • Skin ulcers, most commonly on the legs or fee
    • “Catastrophic” APLS – a very rare, life-threatening syndrome in which clots form in small blood vessels of multiple organs (such as heart, lungs, brain, kidneys)

Other features that might be associated with antiphospholipid antibodies include:

    • Problems with thinking clearly (loss of concentration, difficulty with reading comprehension and performing calculations, memory loss)
    • Neurological problems similar to multiple sclerosis.
    • Migraine headaches, sometimes with visual disturbances
    • Other neurological symptoms including episodes of partial or total vision loss, dizziness, vertigo, loss of balance, seizures, and other abnormal movements

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