"We’ve seen how lupus damages a patient’s sense of self, whether it’s from disfiguring skin disease, or from the inability to get out of bed in the morning. I think it’s heartbreaking. My patients are often scared, they’re fearful for themselves and for their families, and they’re fearful of the unknown."- Saira Sheikh, MD
- Full-Length Track
- Lupus and its many symptoms
- Diagnosing lupus
- Preparing for a visit to the rheumatologist
- Treatment of lupus & risks for infection
- Different types of specialists
- Coping with the challenges of lupus
Ron Falk, MD: Hello, and welcome to the Chair’s Corner from the Department of Medicine at the University of North Carolina. This is our series where we explore topics related to autoimmune diseases, to help patients and their loved ones understand and manage their condition. Today’s episode will focus on lupus – also known as systemic lupus erythematosus, or SLE.
We welcome Dr. Saira Sheikh who is an Assistant Professor of Medicine and directs the UNC Rheumatology Lupus Clinic. She also directs our Clinical Trials Program for the UNC Thurston Arthritis Center. Dr. Sheikh is both a rheumatologist and an allergist/ immunologist. One of her specialties is treating patients who have lupus. Welcome, Saira.
Saira Sheikh, MD: Thank you.
Lupus and its many symptoms
Falk: What is lupus? How would you best describe it?
Sheikh: The word lupus is Latin for wolf. In the 18th century, when lupus was starting to be recognized as a disease, it was thought to be caused by the bite of a wolf. This may have been because of the distinctive facial rash characteristic of lupus that can often leave a bite-like imprint. Lupus is not a new disease. It is believed to have been described by Hippocrates around 400 BC.
It is a chronic, complex, autoimmune disease that can affect any part of the body from head to toe, including the skin, joints, organs inside the body such as the kidneys, the heart, the liver, the lungs, the brain, and many others. As in other autoimmune diseases, in lupus something goes wrong with the immune system and it starts to attack itself. It produces proteins called auto-antibodies that attack and destroy healthy tissues of the body. This results in pain, inflammation, and damage to those tissues. It mostly strikes women, in their child-bearing years, between the ages of 18-44, but men and women of all ages, including children and teenagers, can suffer from lupus. In addition, people of all races and ethnic groups can develop lupus.
Falk: There’s a broad span of what a patient may feel who has lupus. Some people with much milder disease, some with life-threatening disease.
Falk: How does a patient actually get a diagnosis of lupus? How does somebody know that they have it?
Sheikh: In my opinion, no two lupus patients are alike. The process of getting a lupus diagnosis typically begins with symptoms that a patient is experiencing that prompt the patient to seek medical attention. These symptoms could range from painful and swollen joints, to unexplained fever, unexplained weight loss, hair loss, swelling of lymph nodes, swelling of the legs, puffiness around the eyes, pink frothy urine that could indicate kidney damage, pain in the chest that occurs during very deep breathing or movement which indicates involvement of the heart or lungs, unusual sensitivity to the sun, sores in the nose or mouth, change in the color of extremities with exposure to the cold or stress called Raynaud’s phenomenon, a history of blood clots, miscarriages, seizures, unexplained fatigue, and a host of other symptoms.
There’s no single test that is used definitively for the diagnosis of lupus. It’s typically a combination of clinical symptoms, physical examination, and specialized laboratory tests, and sometimes a need for a biopsy of the skin or kidneys.
Getting a positive blood test sometimes does not mean that you have a diagnosis of lupus. For example, the ANA test or the Anti Nuclear Antibody test is commonly used to detect autoantibodies that react against components of the nucleus, or the body’s command center of cells. Most lupus patients have a positive ANA, but most patients with a positive ANA don’t have lupus.
Falk: So that means that that test is of some use, but if you have a positive ANA, that certainly does not mean that you have lupus.
Sheikh: That is correct. Approximately 20% of normal, healthy individuals have a positive ANA. A positive ANA can also be seen in other diseases, such as thyroid disease, liver disease, and other autoimmune diseases.
Falk: Or just an unexplained phenomenon.
Falk: Patients don’t necessarily have a constellation of findings or blood tests immediately on the first visit. Lupus is not one of those diseases that a person walks in and Bingo, the disease is diagnosed. It can take a while. What do you tell those folks?
Sheikh: Lupus has been called “The Great Imitator” because its symptoms can often mimic those of other diseases. One of the things that’s important to remember is that the symptoms can evolve over time. So the symptoms, may not, as you pointed out, be evident at the very first visit, but they can evolve over time. When I see patients in clinic, I tell them that the assessments that we perform are evaluations in time. So today, on this day, of 2017, you may or may not have a diagnosis of lupus, or you may have mild symptoms. I cannot promise you that in 2018 or 2019 these symptoms won’t evolve and you won’t develop more complications. But what I can promise you is that I, and other physicians, are here to navigate the uncertainty with you.
Falk: And in reality, some of those patients will have those symptoms go away. Autoimmune diseases can resolve on their own.
You’ve described lupus as “The Great Imitator,” and that’s a wonderful word choice, because you’ve given us a list of possible symptoms that a patient with lupus may have. But in reality, it’s an imitator of some other process. How do you help patients navigate that uncertainty?
Sheikh: The first important thing is to look for common causes of why patients have symptoms. The reason I say this is that lupus and autoimmune diseases are, while they are common, they are rare compared to general diseases that we see in the population. For example, as I mentioned, thyroid disease. The first most important thing is to make sure that we rule out and look for common diseases or common reasons why you may have these symptoms. In order to make a diagnosis of lupus, your doctor is looking very specifically for symptoms as well as laboratory evidence or tissue-specific evidence of inflammation.
Falk: So you are then using the composite or integrating capability of saying, “What you’re feeling could be due to lupus, let’s try to confirm this and see if in fact there’s another explanation for what’s going on.“ Lupus is common in your clinic, but not common in most primary care physician’s offices. Most of the time, it won’t be a diagnosis of lupus.
Preparing for a visit to the rheumatologist
Falk: When patients do come to a rheumatology office or an office of somebody who takes care of patients with autoimmune disease, what do you want them to bring with them? What sort of information is useful?
Sheikh: It’s very helpful when patients write down the symptoms that they’ve been experiencing, along with triggers of those symptoms. I always appreciate when patients either write down or bring in a list of medications with them to the visit, and also information about their family history, as well as any life changes potentially that could have triggered those symptoms. I think the first visit with the rheumatologist is really an opportunity for the patient to get to know the doctor, and for your doctor to get to know you.
Falk: Some of those triggers, for example, in patients who have lupus--one of the triggers might be sunlight. In fact, at the end of the day, we tell everybody who has lupus that they need to wear a big, floppy hat and slather on as much sunscreen as they can. But if you don’t know that, and you’re coming to see your rheumatologist for the first time, the possibility that when you go out into the sun and you don’t feel well and break out into a rash, won’t immediately be associated in your mind as a patient as something that you should tell your doctor about. So it’s those triggers or those symptoms that you may have that you can’t automatically put into a nice composite box, but in fact are things that you’d want them to write down and bring with them on their first visit.
Sheikh: Absolutely. I think it’s important to write down things that you’re experiencing that may or may not be relevant in your mind, for your doctor to know, but at that visit, we will work together, and your doctor will work with you to go through that list for the symptoms that you describe to help you make sense of them.
Falk: In order to have a diagnosis of lupus one needs to accumulate multiple criteria that are part of the American College of Rheumatology’s criteria for a diagnosis of lupus, and it’s useful then if patients have written down symptoms that in fact might be part of that checklist.
Treatment of lupus & risks for infection
Falk: Let’s talk for a moment about medications that are used to treat mild disease, and then maybe some more medications or approaches for therapy for more aggressive disease. There’s a risk-benefit balance between all of those types of therapy. Let’s talk about one of the most common early forms of therapy, it’s a drug called hydroxychloroquine or Plaquenil. It’s a drug that many rheumatologists use in the early therapy for lupus. Let’s start there.
Sheikh: Hydroxychloroquine or Plaquenil is what we typically think of as an immunomodulatory drug. It works slowly at the molecular level to bring about changes in the immune system that help to treat the symptoms and then prevent complications of the disease. As you mentioned, it is very commonly used. It is a drug that is taken by mouth anywhere from once to twice a day and it is typically well-tolerated, but patients who are on therapy with Plaquenil are required to have their vision checked on a regular basis, because the risk of toxicity from this medication can increase over time when patients are on this therapy.
Falk: Then there are a variety of other kinds of medications that are also used, from glucocorticoids or steroids to other kinds of immunosuppressive drugs. How do you figure out that balance between a drug that’s having a benefit versus a drug that may be too risky?
Sheikh: Because these are potent therapies with side effects, our goal is always to use the least amount of medication possible to achieve the desired benefit, but sometimes the inflammation is so active, that in order to get it under control, we have to use these potent therapies. You ask, how do we balance the risk and benefits? When we’re using these therapies, we have to closely monitor patients with regular visits to our clinic as well as laboratory data and obtaining that on a regular basis so that we’re checking blood counts, liver and kidney function to make sure that we are identifying any toxicity if it occurs and prevent any from occurring.
Falk: What can a patient do, or the patient’s family do, to decrease some of the risks? The risks are infection, when you start suppressing the immune system, the chance of having even garden variety kinds of infection let alone more serious ones, goes up. What can the patient and the family do to help here?
Sheikh: We know that patients with autoimmune diseases in general and lupus in particular are at increased risk for infection. One of the things that are important are making sure that patients are up to date on immunizations, so as a patient I recommend talking to your doctor about any immunizations that are appropriate for you before beginning immunosuppressive therapy.
Falk: Pneumonia shots, influence vaccine, shingles, all of those, as a matter of fact you need to take the live shingles vaccine before you start immunosuppressive therapy.
Sheikh: Absolutely. So a lot of these need to be administered before starting immunosuppressive therapy, but then other vaccines, such as the influenza vaccine and the pneumonia vaccine, can be administered even when you are on immunosuppressive therapy, so that’s important to remember.
Falk: And wash your hands, and wash your hands, and have everybody in the family wash their hands over and over again.
Sheikh: Right, and when you’re on immunosuppressive therapy, I think taking things a little bit more seriously than you would have otherwise, or being a little bit more vigilant about your symptoms and communicating them to your doctor is important. So if you don’t feel good, it’s important to let your doctor know.
Falk: It’s also important for the family to realize that if a person is coming into the house, or if one’s out and about and a family member sees another person has a hacking cough or clearly is infected, it’s good to steer the patient away from that potential source of a bug, and it’s good for people to be able to say that some distance is a good idea.
Sheikh: Yes, and family members and caregivers are partners in this. So to be vigilant using all of the things that you mentioned is important.
Different types of specialists
Falk: Because of the numerous potential, not necessary, but potential places that lupus may attack, what other kinds of specialists are important in the multidisciplinary care of such a patient?
Sheikh: Diagnosing and managing lupus often requires a team approach between the patient and multiple health care professionals. Depending on the organ system that’s involved, this could be a partnership that involves multiple specialists with the primary care provider, so rheumatologists who are doctors who specialize in bone, joints and the immune system, nephrologists, doctors who specialize in kidney disease, hematologists who specialize in blood disorders, hepatologists who specialize in liver disorders, and a host of other subspecialists can be involved, in addition to nurses, social workers, therapists, psychologists, physical and occupational therapists, and others.
Falk: It’s really important that you mention that other group of health care professionals that complement the care of a patient with lupus. It’s one thing to see a kidney doctor, and they may take care of kidney disease, if lupus is affecting the kidney, but I would suggest that almost everybody with an autoimmune disease has difficulty or at times struggles with trying to adapt to a disease that may come and go, for which they’re on these immunosuppressive drugs, and being able to talk with trained professionals-- therapists, psychologists, psychiatrists, other people with whom one has a good relationship with is very much part of care.
Sheikh: Yes. I feel that lupus doesn’t just affect the body. We’ve seen how lupus damages a patient’s sense of self, whether it’s from disfiguring skin disease, or from the inability to get out of bed in the morning. I think it’s heartbreaking. My patients are often scared, they’re fearful for themselves and for their families, they’re fearful of the unknown. They’re angry sometimes and frustrated, all of which are emotions that come with learning to cope with a chronic illness. I feel that our role is to help caregivers and patients through this and to help them move from merely surviving lupus to thriving with lupus.
Coping with the challenges of lupus
Falk: How do you help a patient understand the relapsing and remitting nature of the disease? So, the disease is under control, it’s in remission, the patient feels good, but in the back of the mind there’s always this concern that “This may come roaring back,” and so there’s this sense of almost hypervigilance to see if any new sign or symptom may or may not be part of this process. That can be difficult to try to figure out by oneself as a patient. When should one get back in touch with one’s physician when one thinks that he or she may be flaring but might not be sure?
Sheikh: I think maintaining good communication with your doctor is key. Something that I recommend for lupus patients is not just to see your doctor when you’re having a flare or when you don’t feel good, but the importance of regular visits, so that you and your doctor can discuss these concerns when they arise and for regular monitoring of bloodwork, urine tests, and labs so that we can pick up on these before you actually have a flare.
Falk: That’s really an important concept. It’s important to go see your doctor when you’re well, because otherwise a physician may only see you when you’re sick and think you’re constantly sick, whereas if you come when you are also well, it provides a much more balanced view. Let’s talk about some of the other lifestyle changes that your patients describe to you. What other sorts of lifestyle changes are useful and what may not be as useful?
Sheikh: Maintaining a healthy lifestyle and making healthy lifestyle choices is important, such as eating well, getting enough sleep, exercising regularly, not smoking. I think these basic lifestyle choices are key in managing your disease.
Falk: The big bugaboo, of autoimmune disease and not just lupus, it’s this sense of overwhelming fatigue. I describe it as an “animal fatigue,” like you feel the need to fall asleep and you can sleep anywhere and if you don’t go to sleep, you can’t move. That’s a sense of fatigue that is overwhelming. What do you tell patients with respect to handling those moments?
Sheikh: I agree. I think the fatigue is often a vicious cycle, so that while it’s important to exercise regularly, sometimes you may feel so tired that you can’t exercise or you can’t even get out of bed. I tell patients to take baby steps, to take it a day at a time. It’s also important to remember that some symptoms like fatigue are incredibly difficult to treat and don’t respond to traditional immunosuppressive therapy, and that’s a challenge.
Sheikh: During the flare, it’s really important to listen to your body, to rest, to ask for help, and to communicate with your doctor.
Falk: Any other words of hope for patients?
Sheikh: Due to better understanding of lupus, the prognosis for patients with lupus today is much brighter than it has been in the past. As we all work together to unravel the mysteries of lupus, I feel that there is continued hope for development of more targeted, less toxic therapeutic options that have the potential to help patients, and potentially, ultimately someday prevent or cure the disease.
Falk: A far cry from the era where people thought that lupus was caused by a wolf bite. Where are some trustworthy resources that patients can turn to that are dependable and that you would suggest?
Sheikh: The Lupus Foundation of America, both the national organization as well as the North Carolina chapter are an excellent resource, as are the Thurston Arthritis Research Center and the UNC Kidney Center. A lot of information is available on the web sites.
Falk: Thank you, Dr. Sheikh. And thanks to our listeners for tuning in. If you enjoy this series, you can subscribe to the Chair’s Corner on iTunes or like us on FaceBook so you’ll know when we post our next episode. Thanks so much for listening.
The Chair's Corner is an educational podcast hosted by Dr. Ron Falk, Department of Medicine Chair at the University of North Carolina School of Medicine.