Actor Ashton Kutcher recently announced he suffered an episode of a rare autoimmune disorder called vasculitis.
To find out more, I spoke with Penn Medicine Vasculitis Center Director and Chief of Rheumatology Dr. Peter Merkel, a national leader in the field of the diagnosis and treatment of vasculitis.
Q: What is vasculitis?
A: Vasculitis is really a family of diseases that have in common inflammation of blood vessels. Because blood vessels go all over the body, there are all sorts of different types of vasculitis and different things that it can do. It's about 20 different diseases, actually. They're all considered rare, and they're all generally organ or life-threatening.
When we use the term vasculitis, it can be used for other things as well can cause vasculitis, but we generally mean this group of diseases for which we don't have a clear understanding of their cause, and deal with multiple systems in the body. We have blood vessels in our body that range from the aorta, which is the big blood vessel coming out of the heart which is huge, to the small microscopic vessels in all sorts of places. That's a big, big range. So some types of vasculitis affect the larger arteries, some the medium, some the small, and that makes a difference. So each type of vasculitis has a different pattern of involvement.
Q: It's interesting too, because it's it's tough to diagnose at first, isn't it?
A: Sometimes it's very obvious when it's very overt, or there are very dramatic findings. But sometimes it's delayed. We've actually published data on this, that there are times when the delay in diagnosis can be weeks, months, or even years for some types of vasculitis.
We are working both with educating physicians and with other research means to try to figure out how to improve that overall in medicine to reduce the time to diagnosis because the sooner you get a diagnosis, the sooner you can get effective treatment and prevent some of the damage that might happen from vasculitis. They are an unusual set of diseases.
Q: What should people and physicians be looking for? Because it is a puzzle and it's different with each person.
A: It is a puzzle and that's what we do, and the work I've done for 25 years is working on rare, unusual diseases that are puzzles.
Many physicians are certainly aware of vasculitis and it comes up on what we call the differential diagnosis, or when you're thinking about what could my patient have in front of me. The range of findings is so broad that almost anything could be vasculitis. But there are a series of things that are more likely and that could include certain skin changes, kidney problems, certain neurologic diseases, and certain types of inflammation. You start thinking about vasculitis more quickly. That can also be caused by a variety of different drugs. You certainly want to recognize that so you can stop that medication and other toxins and some infections can lead to vasculitis as well.
Q: I'm talking to you now because of actor Ashton Kutcher and his admission that he suffered from a vasculitis episode a year or so ago. When a celebrity comes forward with something like that, does that help you get the word out about something that's so rare?
A: I'm pleased to hear that he is at least seemingly recovered or done very well. That is of course what we want for everybody. When somebody famous says they have vasculitis, it obviously shines a light on this rare set of diseases.
A rare disease is one that is defined as having fewer than 200,000 people in the United States at any one time with that disease, which sounds like a lot of people, but that's actually pretty rare. Somebody you know and love probably has a rare disease. As I like to say they're rare until someone you know or love has it, and then they're not rare anymore because then you start hearing about it.
So when a celebrity has a rare disease or something like vasculitis, it does bring awareness to it. And I think that's good, more education of the general public. You obviously don't want any one individual to suffer from it.
Q: Vasculitis is incurable, but it's treatable. So what do you do to help patients ease the inflammation and the other symptoms that they have?
A: We think we can overcome some types. If they're caused by a drug, you can stop the drug if they're caused by certain vaccines. You can treat.
I want to make it very clear, that we are working towards a cure for every type of vasculitis. That is part of the work that I've done for decades. That should be our goal, and it is not unrealistic. It is aspirational, but it is what we should be doing.
But in the last 20 years, we've improved our treatment for vasculitis. Remarkably, we have more effective medications, and we have safer medications, and so we've been able to treat many more types of vasculitis and many more people in a much better fashion than we used to.
How do we treat? Well, because it's ‘itis’ inflammation, we need to stop that. We need to put the fire out. And so we use a lot of drugs called glucocorticoids, which some people call steroids, which is a bad term because it's not the same as bodybuilder steroids. We use prednisone and drugs like it. These drugs work very quickly to reduce inflammation, but they do so at a significant cost. They are quite toxic. They cause a lot of side effects that really bother patients.
These diseases, I should make it clear, we consider them autoimmune diseases. So your immune system, which normally is good, fights infections. It gets you ready to fight infection. So when you get a vaccine, it creates antibodies. Your immune system is your friend. It is very, very helpful, and keeps us alive.
Sometimes the immune system goes awry, and it starts attacking yourself when it starts attacking a person itself. That's called an autoimmune disease, and there are all sorts of autoimmune diseases.
Vasculitis is one type of those. And that's a problem. So the answer there is to actually suppress the immune system to lower the activity of the immune system. But it's a little bit. You've got to be careful.
We want to lower inflammation and immunity without overloading it and putting people at risk for infection, so it's a delicate balance. We've gotten better at it with much more (of) what we call targeted drugs, drugs that are very specialized because they don't knock everything out.