The Lupus Foundation of America has taken to calling lupus “the cruel mystery.” Lupus has no known cause and has no cure.
Lupus strikes people for unknown reasons and can appear at any age, although it is most prevalent in young women. The severity of the disease varies from person to person and symptoms can come and go like random attacks.
The disease turns the body against itself. It sends soldiers of the immune system to attack, rather than protect, otherwise healthy organs and tissues.
Around five million Americans have lupus and 16,000 are newly diagnosed every year. Fortunately, there are treatments available that can manage the effects of the disease. But like any treatment for a chronic disease, there are side effects.
Dr. Anca Askanase told dailyRx that it comes down to understanding the severity of the disease, the severity of the side effects and balancing the benefits and the risks.
Dr. Askanase is an assistant professor at New York University's School of Medicine. She is a lupus researcher that truly enjoys taking care of lupus patients. “I think that I can make a difference in patients' lives and that is very rewarding,” she said.
dailyRx spoke with Dr. Askanase about what new lupus patients should know about what lies ahead.
dailyRx: What's the most important thing to know when a patient is newly diagnosed and learning about treatments for lupus?
Dr. Askanase: For someone who is newly diagnosed, understanding the disease is extremely important for both the doctor and the patient; there is a learning curve on both sides. The rheumatologist needs to learn the expected course of the disease for that individual. The patient needs to learn more about the disease and the options.
I think that in the beginning, keep an open mind on both sides as to where the future could go. By that I mean, don't expect the worst, or the best, on both sides. It's a good recipe for sanity.
A lot of newly diagnosed patients have the tendency to envision the worst. But the majority of patients fall into the mild to moderate categories. So there's no reason to expect the worst.
dailyRx: How much do people know when they come in and are diagnosed with lupus?
Dr. Askanase: Some have already read everything that there is. But some have no clue.
It's the doctor's job to point people to good sources for learning. They need to be taught by the rheumatologist in person, but also do their own reading about lupus.
dailyRx: What treatments are currently available?
Dr. Askanase: The options depend on the severity of the disease. Someone with mild lupus might only need Plaquenil, prednisone, or non-steroidal anti-inflammatories, and do well with those.
Someone with moderate disease might need all of the above meaning Plaquenil, a higher dose of prednisone and non-steroidals. But at some point immunosuppressants may be required.
But for those with severe disease, it's always Plaquenil. We think Plaquenil actually allows for better performance of other lupus drugs.
Plaquenil is always part of the treatment of lupus. Someone with severe disease also requires a high dose of prednisone and immunosuppressants earlier on in the course of the disease.
daily Rx: What advantages, drawbacks and complications of treatment can patients expect?
Dr. Askanase: There are side effects related to lupus medications that are part of the way the drugs work, and the unintended consequence of the desired outcome. That means a certain level of immunosuppression is part of the mechanism of action of lupus therapies. Lupus is a hyperactive immune system.
We expect that the drugs we use to treat lupus take the immune system from the hyper state to normal, [and] that's what we achieve hopefully 99 percent of the time. Occasionally we overdo it and put the patient at risk for infections and malignancies. These [side effects] are part of the natural mechanism of the drug, or how the drug works to control lupus. It comes with the territory in a way.
Other drug-specific side effects can be equally devastating, as can be the case with high doses of prednisone that can produce weight gain, diabetes, hypertension. Weight gain can be distributed in a bad and unpleasant way. It's around the face and around the waist. It's a very unsightly weight gain, but [the weight gain] is also proof of the drug working elsewhere. But it could be devastating for someone that's young and already has issues with weight control.
[For] people on high doses of steroids there [can be] bone side effects [which can] produce bone thinning, osteoporosis, [and] avascular necrosis. Avascular necrosis is a common complication of steroid therapy where small or large areas of bone die and that damage the smoothness of the articular surfaces that can lead to secondary osteoarthritis.
Other immunosuppressants can cause gastrointestinal intolerance, or abnormalities with the liver and blood counts that need to be monitored.
It's the doctor's job to look out for the side effects, and some acceptance on the part of the patient.
One of the treatments that we use can, Cytoxan, can reduce fertility.
daily Rx: Do treatments for lupus affect everyday life?
Dr. Askanase: Patients with severe active lupus are enormously fatigued, so getting up in the morning can be difficult.
On the other hand, high doses of steroids make people hyper. It's like too much caffeine...you can't quite channel that extra energy.
High doses of prednisone can make people hyper to point of anxiety and panic. You have someone who is already tired because they're in the midst of a bad disease, and now they can't even sleep.
What questions should a patient ask their doctor before they agree to a specific course of treatment?
Patients always need to ask for options. Patients need to understand the balance between severity of the condition and severity of the side effects. That's what it comes down to.
It's looking at the risks of what we are trying to treat and the risks of the medication. If the risks of the disease itself are higher than the risks of the medication, I think you have to opt for the medication.
daily Rx: What are the risks of not treating lupus?
Dr. Askanase: The risks of letting the disease go untreated are grave. People can die from lupus. Death from lupus happens less now than it has in the past because we understand how to keep it in check, but lupus left untreated can kill.
I think that is the biggest risk. Of course, that risk does not apply to vast majority of lupus patients, but it's there.
In those situations where life and death are the options, I think that a patient clearly understands that the health risks posed from medications are of very little consequence.
In situations where it's more in the "grey zone", in moderate disease, the balance of risk is more difficult to establish.
daily Rx: Are there new treatments or medications on the horizon?
Dr. Askanase: The lupus community has been very excited about availability of Benlysta. We've all started treating our patients with Benlysta and have had success. We're happy to have a new FDA drug for a disease that hasn't seen FDA approved drugs in more than half a century.
Because of that success, there's big interest from pharmaceutical companies to develop more drugs for lupus. There are a couple of drugs that are now in Stage 3 clinical trials.
daily Rx: Do those drugs have anything in common, in terms of how they work on the disease?
Dr. Askanase: The B lymphocyte that makes the auto-antibodies is the target for the majority of these drugs.
In normal situations, antibodies are made to be protective, meaning they fight infections like cold, or a flu. In lupus, antibodies are directed against a person's own healthy tissues and organs. The way we have thought of treatments is to stop the body from making these antibodies that are damaging.
daily Rx: So is that where the immunosuppression comes in?
Dr. Askanase: If we could only target the B cells that are making bad antibodies that would be wonderful, but we're not there yet. In the process, we kill the B cells that are the good ones.
daily Rx: Where is research focused now?
Dr. Askanase: Obviously there's a lot of interest in being able to specifically target the pathogenic B cells. But we're not that sophisticated yet.
We will be. There's a lot of research underway.
daily Rx: What advice would you give a patient who has just received a diagnosis of lupus about how to move forward in their life?
Dr. Askanase: There's a fine balance between appropriately reacting to symptoms, and being able to have a life and not let the disease overwhelm you. Trying to find that balance is a work in progress and may require a lot of help from a rheumatologist and from a psychotherapist.
I think that it's important for people to keep working and being active in their family, community and their workplace. I think that continuing to work and take care of things that people have always taken care of is anchoring.
No matter how severe it is, lupus should take a secondary role.