Lupus: The Disease With A Thousand Faces
Chapter 1: Symptoms of SLE
What are the symptoms of SLE?
SLE is a disease with a thousand faces. Symptoms can vary widely from person to person so that each person’s profile or list of symptoms is different. All of the symptoms described can occur either at the onset (beginning) of SLE or at any time during the course of the disease. Nowadays, it is very unusual to see the sudden and severe onset of SLE. Rather, the disease usually begins slowly with new symptoms appearing over a period of several weeks, months or, at times, years. In general, as one symptom appears it tends to stay while, in turn, new symptoms develop. Because the symptoms may be hard to see or describe and tend to come and go suddenly, it may take time before the person becomes aware that something is wrong. There are two different types of symptoms—symptoms that can occur in other illnesses and symptoms that are specific (particular) to lupus.
The word non-specific is used by physicians to describe symptoms that can occur in many illnesses other than SLE. They include fatigue, weight changes, fever and swollen glands. Often, these symptoms can be connected to SLE only after some of the specific symptoms begin to develop.
Fatigue is perhaps the most frequent symptom that affects patients with SLE. The degree of tiredness seems to be far greater than the activities of the patient would indicate. Lupus fatigue may occur early in the afternoon, leaving the person drained and without energy to continue his or her day. Other patients come home from work so exhausted that all they can think about is going to sleep. It is important not to be alarmed by the normal fatigue associated with the strains of modern life. With experience, most patients can learn the difference between normal tiredness and lupus fatigue and this is very helpful to their physician in determining how active their lupus has become.
Unexplained and continuing weight loss of more than 5 pounds can be caused by SLE. Weight loss may occur slowly (20 pounds over a one year period) or very quickly (20 pounds in two months).
Weight gain that occurs, even though the diet remains the same, may be caused by swelling, particularly in the feet and legs. This swelling may be related to kidney, heart, blood vessel or other organ involvement in SLE. If the swelling continues, it requires prompt medical attention. The mild swelling noticed by many people at the end of the day or by women around the time of their menstrual period is not serious and should not be mistaken for a lupus symptom.
Fever is another non-specific symptom that may occur in many illnesses such as the common cold or the flu. However, low-grade fever that lasts a long time or sudden, high fevers may indicate that SLE is becoming active or that a serious infection may be present. Fever is therefore always taken seriously by physicians who treat lupus patients.
Slightly swollen glands (lymph nodes) are not uncommon in normal young adults. However, if many swollen glands suddenly appear this may be a sign of SLE, particularly if they occur along with
Symptoms specific to lupus
Specific symptoms are caused by involvement of one or several organs of the body. SLE can affect the organs in any combination imaginable and a complete list of all SLE symptoms would fill many booklets such as this one. To help distinguish SLE from other diseases, physicians of the American Rheumatism Association have established a list of 11 abnormalities which, when combined, point to SLE. These abnormalities include symptoms (what only you can feel, such as pain), signs (what your physician can see during an examination) and changes in laboratory tests. Note that although the list that follows is quite long, few lupus patients ever develop all 11 abnormalities.
To make a diagnosis of SLE, the patient must have had at least 4 of these 11 abnormalities at any time since the beginning of the disease. Some of the criteria (items or conditions) in this list contain as many as 4 possible abnormalities but only 1 of them needs to be present for a particular condition to be met. As well, even if 2 or more abnormalities of a particular condition are present, these are counted as a single condition.
To make a diagnosis of SLE at least 4 of these 11 criteria must be present:
- The Butterfly rash is a red rash that occurs over the cheeks and often over the bridge of the nose. This rash eventually happens to 50% of all SLE patients. It may be flat or raised and should not be confused with simple blushing or the redness that comes with fever. Some people notice a feeling of warmth in the area of the rash while others do not. The rash may be so faint that only the physician will notice it or it may be obvious to the point of being commented on by relatives or friends.
- Photosensitivity is an excessive skin reaction to sunlight (causing rash) that occurs in at least 50% of patients. Usually, only the exposed skin is involved while skin that is covered by clothing is spared. Your physician will decide that photosensitivity is present only if there is a sudden change in your usual reaction to sunlight. Some SLE patients experience photosensitivity indoors, especially when exposed to fluorescent lights. In Canada, SLE patients usually have the most trouble with photosensitivity during the spring and summer months. Except for those who travel south in the winter, the Canadian climate has a distinct advantage for sunsensitive lupus patients!
- Discoid lupus is a scaly, raised rash that appears on the face, scalp, ears, chest or arms in 25% of SLE patients. The rash, if untreated, may leave permanent scars. Involvement of the hair roots on the scalp may also cause permanent damage in the form of bald spots but widespread or total baldness is rare. The rash is photosensitive (it will get worse after being in the sun) but this can be avoided if the proper sunscreens are used. In Canada, patients typically experience the reappearance or worsening of discoid lesions during spring and summer. The use of a sunscreen for protection and appropriate treatments can usually reduce or control this problem. It is important to be aware that approximately 10% of discoid lupus patients will eventually develop a usually mild form of SLE. As a result, discoid lupus patients are tested for SLE from time to time.
- Mucosal ulcers are small sores that occur on the mucous lining of the mouth or nose in as many as 40% of SLE patients. Mouth ulcers most often occur on the roof of the mouth and, because these are usually painless, the patient may not notice them except, perhaps, when eating spicy foods. Painful canker sores happen to lupus patients too but are different from lupus ulcers. Nose ulcers are usually painless but they may cause nosebleeds which, if frequent, should be brought to the physician’s attention.
- Arthritis is very common in SLE. In fact, it is unusual for patients with SLE not to have arthritis sooner or later. It causes pain in the joints of the hands, wrists, elbows, knees or other joints in the arms and legs. (Lupus arthritis does not cause back pain although patients may experience back pain from other causes.) The pain may be migratory, meaning that it goes from one joint to another, and it may occur in the same joint on both sides of the body. Signs of inflammation (swelling, redness, heat) may not show up but, when SLE is very active, the inflammation and pain may be severe.Both the patient and the physician can become very frustrated by the arthritis of SLE. Because the pains move from joint to joint and can appear and disappear quite quickly, the patient may not have any signs of arthritis at the time of their appointment. As a result, diagnosing arthritis in the patient can take quite a long time.Lupus arthritis is usually not crippling and patients should not fear becoming wheelchair bound (although the use of a wheelchair is sometimes necessary in SLE due to causes other than arthritis). Changes (deformities) in the fingers do sometimes happen as a result of arthritis but this does not usually cause a major loss of function.
- Pleuritis is inflammation of the pleura, the lining of the lungs, and pericarditis is inflammation of the pericardium, the lining of the heart. These problems may occur alone or together and affect at least 50% of all SLE patients. Pleuritis, and sometimes pericarditis, cause a particular type of chest pain that gets worse when breathing in and gets better or goes away when breathing out. Patients may also have less pain when sitting or standing and more pain when lying down. If the pain is severe, the person may be limited to shallow breathing only. Lupus patients who experience a second bout of pleuritis or pericarditis later in the course of their disease often readily point out that “…the pain is like it was the first time…”.While there are many benign (not serious) causes of chest pain, it is wise to inform your doctor immediately if you experience such a pain. By examining you, and performing tests such as a chest x-ray, heart ultrasound and electrocardiogram (ECG), your physician will be able to make a diagnosis and, if necessary, start treatment.
- Kidney involvement ranges from very mild to very serious. It is believed that most, if not all, patients with SLE have at least some mild degree of kidney (or renal) involvement. When mild, this involvement is usually not a problem. However, in 50% of patients, kidney damage may occur and this is of concern because it may lead to a loss of the kidney’s ability to clean the blood. An upsetting feature of SLE kidney involvement is that it tends to remain silent (the patient will not have any symptoms) until damage has already occurred. Then the patient will complain of weight gain and swelling, particularly in the feet and legs. Doctors call this fluid retention or oedema and it is the most common symptom of major kidney involvement.It is very important to detect kidney involvement early because some of the deaths caused by SLE are related to severe kidney involvement. For this reason, regular tests of kidney function (simple urine and blood tests) will be ordered by your physician. Urine analysis and tests of kidney function are best performed from time to time, even when lupus seems to be quiet. Regular testing of the kidneys is absolutely necessary whenever lupus is active. Urine analysis can show various signs of inflammation in the kidney such as clumps of red blood cells (called cylinders or casts) or the presence of an excessive amount of protein. To determine how much involvement is present, doctors measure the amount of protein and the performance of the kidneys on all your urine collected over a 24 hour period. In this way, early involvement of the kidneys can be discovered and, if necessary, treatment can be started.Although most patients with SLE never develop kidney involvement that requires the use of artificial blood filtration (dialysis), some patients clearly will. Because kidney involvement can be so serious, affected patients should be evaluated by a nephrologist, a physician who specializes in treating diseases of the kidney.
- Seizures (epilepsy) and psychosis are serious problems caused by central nervous system (CNS) involvement in SLE, and occur in 15-25% of patients. These problems can be caused by many disorders other than SLE so doctors must take the time to rule out other conditions before deciding that SLE is the true culprit. It is important to be aware that, with CNS involvement, other types of problems may occur which are not as serious as seizures or psychosis.Seizures most commonly involve loss of consciousness and involuntary body movements. The person does not usually recall what happened and descriptions by relatives or friends who witnessed the seizure are most helpful. Seizures can usually be confirmed by performing an electroencephalogram (EEG) which is a reading of the electrical activity of the brain. Fortunately, several drugs are available to control seizures in SLE patients.When seizures are the first symptom experienced by the patient, SLE may not be suspected until other lupus problems appear. This can cause confusion, as physicians may wonder if the other symptoms were triggered by the anti-epileptic drugs. In other words, is this drug-induced lupus? In most cases the answer is no and the seizures will begin again if the anti-epileptic drug is stopped.Psychosis is a serious mental condition where thinking and behavior are disturbed, often including hallucinations (seeing or hearing things that aren’t there) and delusions (false notions or ideas, for instance the belief that one is being poisoned). Relatives may say that the person has partly or completely lost contact with reality. Other symptoms may be present such as confusion (the person does not know where he/she is, what the time and date are and may not recognize family members or close friends). Many doctors think that SLE psychosis is best treated with prednisone or a steroid drug.
- Disorders of the blood cells are caused by autoantibodies that attack one of the blood cells or particles, usually the red or white cells or platelets. It is important to understand that these problems may have causes other than SLE. In all cases, doctors must rule out the other possible causes before treating these problems as related to lupus.Red blood cells may be attacked, resulting in a large number of cells being destroyed and removed from the body in the spleen, a process called hemolytic anemia. This destruction may be slow and relatively mild or may be very quick and cause an emergency. Prednisone is effective in treating this anemia in most people but sometimes the spleen must be removed to bring the anemia under control.SLE commonly causes a decrease in the white blood cells called leukopenia. In most instances this is not dangerous and by itself does not require treatment.Platelets are cell particles that control the clotting of blood. Autoantibodies to platelets may cause the platelet count to drop, a situation called thrombocytopenia. Some SLE patients continue to have, over time, lower than normal platelet counts which do not require treatment but do need to be checked regularly. In others, a severe decrease in the platelet count can cause bleeding in various parts of the body such as the digestive tract, the urinary tract, the uterus or the brain. This is a serious situation that requires treatment with prednisone and, sometimes, removal of the spleen. Easy bruising of the skin may be a sign of a decreased platelet count but it also occurs in normal individuals or can be caused by prednisone. In my experience, these explanations account for far more cases of bruising than does a low platelet count.
- Immunologic disorders refer to four autoantibodies found in the blood which, when taken with other symptoms, point to SLE.The lupus erythematosus cell preparation (doctors say LE prep) test is positive when a particular cell (the LE cell) is found in the blood of patients with active SLE. However, the LE cell is sometimes found in disorders other than SLE and most doctors have stopped using this test simply because better tests are now available.Anti-native DNA autoantibodies are common in SLE and it would be unlikely that they would be seen in other diseases. This test is repeated often because the amount of anti-native DNA autoantibodies seems to increase when lupus is active and the test can help the physician measure the degree of disease activity.Anti-Sm autoantibodies refer to the name of the first patient in whose blood they were found (her name was Smith). These antibodies point to SLE.A false-positive test for syphilis, which happens in approximately 20% of SLE patients, suggests that patients have the venereal disease called syphilis when, in fact, they don’t. This is another of SLE’s sneaky thousand faces and one that has caused a lot of fright and upset. When a diagnosis of SLE is suspected, doctors simply request this blood test for syphilis (V.D.R.L.) and, if it is positive, further tests are done that can show whether or not syphilis is actually present.
- Antinuclear antibodies, or ANA, are found in the blood in almost every patient with SLE. This test has made the diagnosis of SLE more certain, as it is very accurate, but three cautions must be kept in mind. The first is that ANA may be present as a reaction to various drugs, may be present in diseases other than SLE and may be present in healthy individuals, particularly the elderly. Therefore, a positive ANA must always be looked at in light of other symptoms of SLE. The second caution is that the improved methods of testing for ANA have found small amounts of ANA in the blood of many apparently healthy people. Therefore a positive ANA does not necessarily mean a diagnosis of SLE. The third caution is that a positive ANA test should be repeated to be absolutely sure.In rare cases, a diagnosis of SLE will be made even when the ANA test is not positive. Some patients with several specific symptoms of lupus will not develop a positive ANA until later on in their disease. Other patients may have a particular autoantibody in their bloodstream called anti-Ro (from Robert who was the first person identified with this antibody) which is poorly detected by standard ANA tests. In these cases, there is a specific test for anti-Ro that is available at University Hospitals.
Are there other symptoms of SLE?
Yes. Describing the thousand faces of SLE is not possible within the covers of one small booklet. However, there are some additional symptoms and signs that are commonly experienced by patients.
- A persistent increase in hair loss involving the whole scalp may be due to SLE. Patients become aware of a much greater than usual amount of hair on their brush or comb, on their pillow or when they shampoo. The normal cycle of hair growth and loss should not be mistaken as a sign of SLE. Unless hair loss is associated with discoid lupus lesions (damage) of the scalp, baldness due to SLE is very uncommon and hair usually grows again as the disease is brought under control.
- Subacute cutaneous lupus erythematosus (SCLE) is a rash that occurs most often on the arms and upper body. In 50% of patients this rash occurs along with SLE and, when this happens, SLE is usually quite mild. The rash is usually sun sensitive and therefore sun protection is required and quite effective. This rash is associated with the anti-Ro autoantibody.
- Raynaud’s phenomenon occurs in at least 20% of people with SLE. Patients may notice that their fingers or toes turn white on exposure to cold temperatures. This blanching is due to spasm of the blood vessels, where the flow of blood is reduced, and may be followed by a bluish and/or reddish phase. In Canada, Raynaud’s tends to show up during the fall and winter months and then go away during the warmer weather in spring and summer. Patients may notice these colour changes when they go out in the cold to work, while some may experience it just by going downstairs to the basement or walking into a cooler room. It is important to be aware that Raynaud’s occurs in at least 4% of the normal population so it is not necessarily a sign of SLE. However, the onset of Raynaud’s after SLE is diagnosed should be brought to the doctor’s attention as it may be a sign of more serious blood vessel problems and/or mark the beginning of a flare.
- Another problem may be vasculitis which means inflammation of the blood vessels and which may appear as hives (doctors call this “urticaria”). Hives are raised, often itchy patches of skin which may occur on any part of the body and which tend to come and go. Hives caused by vasculitis usually last at least 24 hours, unlike hives caused by allergy in which individual lesions tend to fade more quickly. SLE patients may also experience another type of vasculitis which results in raised, bruise-like lesions which occur first on the skin of the feet and legs.
- Headaches are common in SLE and, as in the general population, the most common cause is probably muscular tension related to stress. In my opinion, migraine headaches seem to be much more common among lupus patients than in other illnesses in the rheaumatic disease family. Lupus patients often mention visual problems such as zig-zagging lights. The new onset of headache, of a different type of headache or of an unusually severe headache should be brought to the doctor’s attention as it may indicate that SLE is getting worse.
- Finally, persistent high blood pressure (hypertension) in any person is always abnormal but it frequently is present in SLE when kidney involvement is severe. As well, it may occur with higher doses of prednisone. Persistent hypertension may lead to damage to the blood vessels which, in turn, may eventually lead to strokes or heart failure. Because the high blood pressure in SLE usually does not cause symptoms, blood pressure should be checked regularly by your doctor. Nowadays, there are extremely effective treatments for hypertension.
It is clear from this long list of symptoms that lupus is a complex disease and one in which almost every system of the body can (not will) be affected. It is important to remember that only four specific symptoms, signs or test results are needed to make a diagnosis. Very few patients will go on to develop all of the symptoms listed in this chapter and, if they do, this will occur over a period of many years. Lupus seems to be a different disease for each person which is why I call it the “disease with a thousand faces.” Physicians must carefully look at each “face” before a diagnosis of lupus can be made.