15 Questions - Understanding Lupus

(May 2012) Lupus is an autoimmune disease that can affect any part of the body, including internal organs, the skin, and joints. It also has many different symptoms, and, because of that, affects each person differently. Because of its unpredictable nature, people with lupus often have many questions about the disease. For May, Dr. Jinoos Yazdany answered your questions about any aspect of lupus.

1. What is the simplest way, in layman's talk, to explain what lupus is and is not? Binghamton, NY

Lupus is a disease in which the immune system does not function properly and attacks normal, healthy tissues. This can result in inflammation and symptoms throughout the body, including the skin, joints, blood, kidneys, and heart.

Lupus is not contagious and it is not a form of cancer. It is an autoimmune disease, just like diabetes type 1, psoriasis, thyroiditis and rheumatoid arthritis.

2. Is lupus truly a woman's disease and if so why? Some men do have lupus, me for one, but I wonder if it's because men don't go to the doctor as often as women and have less of a chance to be diagnosed correctly. Why do some men get this disease and others don't? Groveport, O

This is one of the most common misconceptions about lupus. While lupus is much more common in women, men also develop lupus. In adults, about nine of every 10 cases of lupus occur in women. But that means that 10% of cases of lupus occur in men. The difference we see between men and women in the prevalence of lupus is unlikely to be from men not seeking medical attention. In fact, even in children, we see that girls are more likely to develop lupus than boys.

We do not yet fully understand what causes lupus, although we suspect that genes play an important role. However, genes are not the entire story, and recent evidence shows that environmental factors, for example smoking, may increase the risk of lupus. Both men and women likely develop lupus because of some combination of genetic and environmental risk factors. Since women have a higher risk of lupus, some researchers think that hormones or having two X chromosomes may also increase the risk in women.

3. What exactly does borderline lupus mean? How long does one remain borderline? San Benito, TX

Doctors may sometimes use the phrase "borderline lupus" when patients have some symptoms or blood work to suggest lupus, but not enough to make a diagnosis certain. This occurs commonly because some of the blood tests we use in evaluating lupus, such as the anti-nuclear antibody (ANA), can occur in people without the disease. In addition, some of the symptoms of lupus, such as joint pain, can occur from a variety of things and are not specific to lupus. Doctors therefore must look for a combination of criteria from your blood work, medical history and physical examination to make a diagnosis.

The most commonly used criteria were established by the American College of Rheumatology. If a patient meets 4 or more of the 11 criteria, then a diagnosis of lupus is likely. Sometimes, if a patient meets fewer than the required number, a doctor may designate this with a term such as "borderline lupus", "incomplete lupus", or "undifferentiated" disease. When this occurs, it is important to continue to follow up with your doctor for monitoring. Studies suggest that a small percentage of people in this "borderline" group go on to develop additional symptoms or blood findings and are eventually diagnosed with lupus or a related condition. This progression usually occurs in the first five years. However, most people do very well – they never develop additional criteria.

4. What exactly constitutes a flare? It seems it has a wide range of symptoms. Lancaster, CA

When a symptom of lupus appears, whatever it may be, this is sometimes called a "flare". For example, if someone has a new lupus rash, this is a "flare" of their skin disease. Since every person's lupus is unique, so is what constitutes a flare.

We talk about the concept of flares in lupus because for most people, symptoms and signs of the disease come and go. Most people have periods when they feel fine and then periods when their disease is more active or "flares".

5. How long can a person live with lupus. Mableton GA

Most people with lupus, in fact 80-90% of them, have a normal life expectancy. Modern treatments have made a big difference, and over the last few decades, we have seen survival for people with lupus increase dramatically. Prognosis, or how people do, depends a lot on the severity of lupus. Those with milder disease tend to do extremely well, while those with severe internal organ involvement can still die from the disease, especially if they do not receive timely and appropriate treatment. Getting appropriate medical care, taking needed medications, and living a healthy lifestyle are all things people with lupus can do to stay healthy.

6. If a person's lupus has been in remission for several years, do they still need to have the kidney test, and other test such as a CBC panel done regularly? Santa Maria, CA

Research studies that have followed people for over a decade or more suggest that lupus can become active even after a very long period of remission. We do not have a good way of predicting in which people these recurrences will occur. In my own practice, I have seen patients develop lupus symptoms after more than a decade of having no signs of active disease. The safest strategy is to continue to see your physician at regular intervals for monitoring, even when you are doing well. That way if any signs of lupus appear, some of which may be picked up only on your blood or urine tests, you can be treated right away.

7. I was wondering if your lupus is not active, can you still experience symptoms. I had my blood work done and it’s not active at this point but I feel the symptoms like I am active. Always tired, achy and losing my hair. Taylorville, IL

The correlation between blood work and symptoms is not perfect, and it is not uncommon for people to feel that their lupus is active when their blood work is normal. It is important to talk about your symptoms with your doctor and see if an underlying cause can be determined. In some cases in which people have symptoms such as fatigue, pain and hair loss, it may indeed be active lupus. However, these symptoms can also result from a variety of other things, including metabolic disorders like thyroid disease or even psychological stress or depression. In my own practice, I carefully consider all these other causes before attributing things to lupus so that I can institute the most appropriate treatment.

8. Can lupus antibodies be transmitted through blood donation, plasma, and immunoglobulin injection or products, given the fact that blood is connective tissue? Riverside, CA

We do not fully understand if lupus antibodies would cause any harm if infused into someone without lupus, although we have little reason to believe that this would be dangerous. Even if there were a small theoretical risk of a side effect for the blood recipient, we know for sure that the person receiving blood would not develop lupus. This is because lupus is not contagious or infectious, and therefore cannot be transmitted to other people.

It is important to know that when people donate blood, the components are separated. For example, red blood cells and platelets are separated from plasma and antibodies. This means that for most blood donations, antibodies, the component that some worry about, are removed. Individuals with lupus can therefore donate blood and platelets, and the American Red Cross has a policy of accepting blood donations from people with lupus.

9. How can I tell if the pain that I have is the lupus, fibromyalgia or other overlap disease? Cornelius, OR

Lupus and fibromyalgia often co-exist, and determining whether one versus the other is causing pain can be challenging. There are some things that I use to distinguish whether pain is resulting from active lupus or from fibromyalgia. For example, if an individual is experiencing a lupus flare causing pain, it is likely that they have other findings, such as joint swelling, skin rashes, ulcers in their mouth, fever, or pain with breathing (pleurisy). Also, blood tests may show signs of active lupus, such as evidence of inflammation, low blood counts, or protein in the urine. None of these findings would be a feature of fibromyalgia, and would therefore make it more likely that pain is from lupus. If such signs of symptoms are present, I might prescribe medications to treat lupus, which in turn should help the pain improve. If the pain does improve with lupus treatment, this can be an important clue that it was caused by lupus.

In contrast, sometimes people have pain and no evidence of lupus or any other disease being active. In these cases, I would look to see if the pattern of symptoms is consistent with fibromyalgia. For example, pain in fibromyalgia is often widespread, and sometimes associated with other problems, like poor sleep, irritable bowel symptoms, tension headaches, cold intolerance, anxiety or depression. Treatments for fibromyalgia are entirely different from treatments in lupus, and that is why it is important to try to distinguish the two.

10. Can there be other symptoms with discoid lupus besides the obvious skin issues? If so what are they? Spanaway, WA

In 90% of cases, discoid lupus rashes occur alone and are not associated with other symptoms. However, approximately 5-10% of people with discoid lupus will go on to develop other symptoms that are consistent with systemic lupus. In these cases, the discoid rash is one feature of a more widespread systemic disease. Your doctor may initially send blood tests or ask about these other symptoms (such as arthritis, pleurisy (pain with breathing), or fever) to look for systemic lupus.

11. Do people with lupus all go through the same problems? Houma, LA

Every case of lupus is different, and the experience of having lupus and the problems associated with the disease can be quite different. For some people, lupus is very mild and manageable and stays that way throughout the lifespan, while for others, it can cause serious organ involvement and require intensive treatment. It can be very helpful for people with lupus to speak with other patients who have had similar symptoms and problems. In my clinic, I often connect patients who are going through similar experiences so that they can support and learn from each other.

12. In your research have you found that people with lupus have more instances of cognitive difficulties? Does lupus affect how individuals are able to handle complex tasks esp. during flares? If so, does this become worse over time? New Windsor, MD

Cognitive difficulties occur in many individuals with lupus, and range from very mild to severe. Problems with memory, attention, planning, decision-making, and articulating thoughts have all been described. It is important to remember that while all of these cognitive difficulties can be caused by lupus, they can also result from other factors, such as stress, anxiety, depression, sleep deprivation or medications, especially steroids. During a flare, people may experience more cognitive difficulties from a combination of these factors. In many cases, once things like stress and steroid dose are reduced, people feel that their cognitive difficulties improve.

Not all cases of cognitive difficulties in lupus worsen over time. In fact, these symptoms almost never progress to dementia in lupus. There are things that you can do to help with cognitive difficulties in lupus, such as modifying risk factors (such as stress or lack of sleep) and developing behavioral strategies to compensate for specific problems. A neuropsychologist can be very helpful with this latter aspect. Many people benefit from a program of cognitive rehabilitation, guided by a neuropsychologist, which may entail things like learning how to use routines, lists, and repetition to compensate for cognitive difficulties and to improve quality of life.

13. Many Doctors have informed me that I should not have any immunizations as it may cause a flare of my lupus. Is this true? If so, are there any that won't cause such a reaction? This is a concern for tetanus, flu shots, and immunizations required for traveling to different countries. Waterloo, IA

We now have some good studies showing that most vaccines are safe in people with lupus and are not associated with significant flares. I recommend that my patients get a variety of vaccines, including the inactivated flu, pneumococcal, and tetanus vaccines. This is very important to prevent serious infections, especially if you are taking drugs that suppress your immune system, making these infections more serious if they occur. My recommendations are consistent with those of the Centers for Disease Control (CDC) in the United States.

There is one caveat to the above. Although most vaccines are safe for people with lupus, there is one type of vaccine that may pose a risk if you are receiving drugs that suppress your immune system: "live" vaccines. Live vaccines contain a weakened form of a live virus, which is not strong enough to cause problems in people with a normal immune system. However, in people who are taking medications that suppress the immune system, we worry that live vaccines might cause symptoms of active infection. Examples of live vaccines are measles, mumps and rubella (MMR), chicken pox (varicella), shingles (zoster), yellow fever and intranasal flu. If you need one of these vaccines, it is important to discuss this with your lupus doctor. In some instances, your doctor may decide to temporarily stop certain immune suppressing drugs before administering these vaccines.

14. I was diagnosed with lupus in late 2011. My doctor put me on plaquenil but I found it very harsh on my stomach. Do I have to take meds? I seem to feel worse on them than off. Murphy, NC

Doctors prescribe plaquenil for lupus for two main purposes. The first is to treat symptoms, and plaquenil is often one of the first medications prescribed for things like rashes, ulcers in the mouth, and joint pain because of its effectiveness and good safety profile. The second reason is to prevent flares of lupus, and doctors may prescribe plaquenil for this purpose as well. Some studies suggest that lupus patients who stopped taking plaquenil had an increased risk of flaring.

All of this said, every person's circumstances and preferences are different. For some people, the benefits of plaquenil clearly outweigh the risk of possible side effects. In other cases, side effects occur and are annoying or serious, and people cannot tolerate the medication. In your case, it is important to speak to your doctor about your options. For example, if your doctor thinks the medication is important for your lupus, you might discuss trying a lower dose to see if your stomach discomfort resolves. Also, in some people, stomach discomfort is temporary and goes away after the first few weeks on the medication, so a longer trial might be worth a try.

15. In reading about lupus, I am under the impression that many people suffer from chronic infection and it is a serious (if not fatal) aspect of the disease. Would you verify? Yakima, WA

The immune system, the body's defense against infection, is weakened in people with lupus from both the disease and the medications used to treat it. Respiratory, skin and urinary infections are the most common types of infections we see in people with lupus. People with lupus who are on powerful immune suppressing drugs, especially those on combinations of drugs that also include high doses of steroids, are at highest risk for infections. People with milder lupus who are not on these medications have a very low risk of infection, similar to the general population.

Even in our highest risk patients, we are getting better at minimizing the risk of infections by using safer combinations of medications, and also by encouraging patients to receive vaccinations. For example, I recommend that all of my patients taking immune suppressing drugs get a flu shot every year and also a pneumonia shot (called pneumococcal vaccine).