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A closer look at the autoimmune disease vasculitis

When various forms of news media last week reported the cause of death of Harold Ramis, the writer/director/actor, as complications from the “rare autoimmune disorder vasculitis,” I can promise you there were many people who read that and said, “Huh?” for very personal reasons. These are people who, like me, knew that these reports weren’t quite right. Vasculitis is actually a family of at least 15 forms of this disease group and one not so rare when all those who have some form (perhaps as many as 3 million) are added together.

Research and clinical trials on vasculitis have been carried on in a handful of centers around the world. One long-time investigator in this area, also a teacher and clinician, is here at Stanford: Cornelia Weyand, MD, PhD, division chief of immunology and rheumatology. Wayand’s e-mail box was flooded last week, so we asked her to answer some basic questions here about the vasculitis family.

I understand the vasculitides are a family of diseases. Is there something all forms have in common?

A diagnosis of vasculitis means that there is inflammatory disease in the blood vessels.

All organ systems in the body have blood vessels. Therefore, all organ systems can be affected by vasculitis. Blood vessels provide oxygen and nutrients to the tissues. Inflamed blood vessels have a tendency to become blocked. In that case, the tissues do not get blood supply anymore, causing serious complications. In some cases, the inflamed blood vessel bursts, causing life-threatening bleeding. This complication is particularly serious if the body’s largest blood vessel, the aorta, is affected. A leak in the aorta is incompatible with life.

What insights into vasculitis have we gotten from research?

My research team has been involved in vasculitis research for the last decade. We have been trying to find answers to the questions most patients ask at one point in the course of their disease:

A. Why did I get this disease?

Vasculitis results from a faulty immune response. Cells of the immune system attack the blood vessel and cause tissue injury. The blood vessel responds to the attack by either closing up or by rupturing. We have been able to identify the immune cells that initiate and sustain vasculitis. Remarkably, cells that induce disease are identical to cells that protect the body. We have also learned that blood vessels have specialized sensor cells in them that keep a dialogue with the immune system and start the inflammation.

B. How can my disease be treated or prevented?

We cannot prevent vasculitis, but since the disease takes a course of flares and remission, we may be able to prevent the next disease flare.

Vasculitis is treated by suppressing the immune system. One of the most effective drugs is cortisone. Some patients need it in large doses and we are very cognizant of side effects.

We have devoted our research effort to develop new means of therapy. To accomplish that goal, we have developed a system in which we can induce vasculitis and then test new therapies. This system involves the transplantation of human blood vessels into mice. If such mice are supplied with immune cells from our patients, vasculitis develops in the engrafted blood vessel. We can treat that inflammation in the mice and can easily take a biopsy from the blood vessel to check what we have achieved and how therapy actually works.

C. How do you know whether my disease is active or not?

This is one of our greatest challenges as we take care of our patients. We cannot just go and take a tissue biopsy of our patients every time they come and see us. We have a research project in place which is aimed at developing biomarkers of vasculitis in a blood sample. We isolate out the immune cells of the patient and, by applying cutting edge technology, we assess these immune cells to get information how likely or unlikely these cells would cause inflammation.

Are these conditions often misdiagnosed – and, if so, why?

I’ve learned from my patients that misdiagnosis occurs quite frequently. The reason is that each vasculitis by itself is a rare disease. Thus, physicians may only see a few cases in their practice and are just not familiar with the disease. The other reason is that vasculitis can easily mimic another condition, making it difficult to “nail down” that there is blood vessel inflammation. Yet, another reason is that we don’t have highly specific blood tests to help with the diagnosis. In some forms of vasculitis, we have autoantibody tests, that are helpful, but in other forms different diagnostic testing has to be applied. Finally, many different organ systems can be involved, e.g. the eyes, the ears, the nose, the lungs, the heart, the gut, the kidneys, the nerves, the skin, the brain, etc. Thus, patients are seen by many different specialists.

If one person in a family has a form of vasculitis, does this mean others have an increased risk?

There is genetic risk that contributes to vasculitis, but its impact is relatively small. Most patients we see in Vasculitis Clinic do not have a family member that has a similar disease. The genetic risk may be more visible on the population level. Certain populations are at higher risk to be affected by vasculitis. One example is giant cell vasculitis which occurs with the highest frequencies in individuals of Scandinavian origin and thus is seen more frequently in Iceland, Norway, Sweden, and Denmark.

Can you tell us more about treatments, and which ones have proven most effective?

As mentioned above, corticosteroids remain the cornerstone of therapy for almost all vasculitides. Depending on the type of vasculitis, we now have an armamentarium of other drugs, which we use as “steroid-sparing agents”. That means we combine them with corticosteroids to be able to reduce the dose of Prednisone much faster.

All medications used in the therapeutic management of vasculitis have in common that they suppress the immune system, they are so-called immunosuppressants. That means that our patients are at risk to acquire infections and we have to be very careful.

A major challenge is that vasculitides often have to be treated over many years. Most patients will receive several different treatment regimens over the course of their disease.

The art of vasculitis management lies in the Goldilocks Rule: treat enough to suppress the disease, don’t treat too much to avoid loss of protection for the patient.

8 Responses to “ A closer look at the autoimmune disease vasculitis ”

  1. Ana Says:

    Hi, I too have been diagnosed with Polyartritis Nodosa in 2009 after being misdiagnosed with fibromyalgia. The symptoms started early but I mistook them for having RA which I was diagnosed in 1995 with. Having been on Plaquenil for the past 10 years has made me wonder whether that attributed to my having PAN? My specialist has told me that what I have is so very rare (97 documented cases world-wide, but who knows now?) and yes I had a biopsy done to determine what type of the disease I had. The disease is painful, debilitating, and has basically ruined my life to where it was once. On the onset i was put on heavy doses of prednisone, weekly injections of methotrexate and continue to take plaquinel. Was given oxycontin for pain and also take sleeping pills. As a result chronic fatigue has become a way of life for me and the inward burning which has been caused by nerve damage attributed by the disease I live with 7/24. I am unable to work full time and even finding work part time with this illness is impossible as I do not know I will be feeling the next day. I would really like to know what more research is being done because it appears that there are people in my family spread out that have been affected by this disease by other names of the disease. I appreciate your time and look forward to seeing more research done in this area and pray that one day this disease will be easier to detect. I lay in a hospital for 2 weeks, misdiagnosed and basically had I not sought out further medical attention I would not be here and the conclusion would have been not one of dying from vasculitis but of fibromyalgia as was reported to me.
    Thank you for your time. Ana

  2. Helen Deines Says:

    Our oldest daughter almost died about four years ago at the onset of one of these diseases. After six weeks in the hospital (2+ on a vent) and three in rehab, she ultimately went to NHI, but still had no diagnosis. However, last month she was ultimately diagnosed with microscopic polyangiitis. Over these last four years, however, her care has been excellent. She lives a relatively normal life with the disease controlled by immuran. She is exhausted much of the time, but enjoys her friendships, helps others, exercises as possible, and volunteers as able. All research gives hope.

  3. Elizabeth costill Says:

    I have had SLE for 19 years. In the last two years I have had episodes of iron defincicy anemia which appear to be GI bleeding. I have had blood transfusions, three rounds of ferritin infusions, three colonop., three endoscopes, and two capsuale ebdoscopes . Nothing has shown up on my GI tests, my hematologist feels the blood work is indicative of a GI problem. My rheumotogist and I have talked about the possibility of vasculitis. Has anyone else had problems with blood loss and iron defin. anemia. I have stomach pain occasionaly, and bouts of severe cramping and diapherra that comes and goes.I am just very frustrated and would like to find out what is wrong. Thanks so much.

  4. Jean Says:

    I am frustrated and discouraged..bear with me.
    Before 2006 I was an active relatively healthy person who danced at weddings, ran up and down steps two at a time, and hopped up onto countertops to put things away. No joint issues.
    I had a head on collision with a drunk driver and thankfully, walked away with only (I guess, “only”) tissue and ligament tears in my back, resulting in chronic pain in my thoracic back and a plethora of strange symptoms that started within a few days post accident.
    I awoke about a week after the accident and could hardly move any joint in my body. It was like I was made of stone. My hands in particular and fingers were almost atrophied. I got out of bed and almost fell to the floor, as my feet felt foreign to my body and my ankles felt as if they could not hold my body weight. (I weigh about 106 pounds) My feet were red and puffy. I could not hold a coffee cup or trust myself to grip anything as I did not have strength in my hands. It was terrifying. I didn’t know if I was having a stroke or what was going on. My joints were affected on both sides of my body.
    I went to my GP and they ran a blood panel. The intial bloodwork came back with a positive ANA for Lupus. They ran another and it was negative than another that was inconclusive?? My GP referred me to a Rheumatologist.
    In the meantime, both of my calves swelled to almost twice their size and were hot and red. She ordered an ultrasound to rule out a blood clot. Their was no clot, so that was a relief, but what the heck was going on? What was invading my one time healthy body? It had to have something to do with the trauma sustained in the accident. That could not be coincidental. I was flipped around in the middle of the road and pushed against a guardrail in a head on collision. My nose was broken and my chest had a huge bruise from air bag deployment.
    I saw the Rheumatologist for awhile and he concluded it was Mixed Connective Tissue Disease and not Lupus, which was better. He also concluded I had Fibromyalgia, as I had great sensitivity, particularly on my feet.
    I was never sold on the Fibromyalgia diagnosis. I always felt that to be something I never wanted to tell anyone I had, because it seemed like the Hypochondriac Syndrome. Everyone has it. Nonspecific tests to prove or disprove. Just a big name to give you an excuse to say you hurt all over and are tired and have about a billion other symptoms to blame on it…Can’t concentrate..etc. It’s the “coverall” disease!
    I am saying this because I do hurt all over and I don’t know what or why, but I don’t think that is what it is. Fibromyalgia is probably real, but it will never ever be taken seriously, unless there is a test that proves beyond a shadow of a doubt that you indeed are SUFFERING from something REAL.
    Ok, I got off subject. So where I am now is, I have stopped long ago going to the Rheumatologist because he was an insensitive SOB. I heard him berate an 80 year old man in the next room until I wanted to punch him and he made me want to cry every time I went there. No Doctor has a right to make their patients feel like crap, when they already do.
    I just started taking some aspirin and Ibuprofen and sucked it up. It waxed and waned and I went through about 50 pair of shoes (that is no exaggeration) trying to find a pair that did not hurt my feet. I used to love shoes and had all kinds, but since the accident, every shoe hurt and my feet did not belong to me anymore.
    So…fast forward to now. Eight years of joint pain and back pain and I can’t say I have swelling much except for my ankles and feet some. But in the past, I would say 2 years or so, I have developed a web like rash on my outer calf and now it has developed on the inner calf of the right leg as well and it is getting darker.
    At first I only noticed it when I bathed and thought it to be the result of my hot tub water, but noticed it not fading when I toweled off. It has now turned to a red/purple shade and is there all the time. I also noticed the second toenail on my left foot has a brownish red discoloration and it is ridged in the nail. It looks like a toenail that has been smashed but it hasn’t. I didn’t think anything of it, but saw a few pictures on Vasculitis and it looked similar. I’m not trying to diagnose myself but it made sense.
    I started piecing together my issues. My nightmare of dry eye issues where I have had to pull over on the interstate and put drops in to be able to see, and to stop the stabbing pain behind my eyes. This has gone on for years. My vision has been affected a great deal. I am worried I will lose it. I got new glasses once thinking that would help, and it didn’t. Dozens of kinds of sprays. I’ve tried them all.
    I have sores in my nose that are like craters and it is so dry I have to put Vaseline inside to lubricate it. And lastly, I have an ulcer the Doctor described as “the size of a silver dollar” and Esphagitis. I at times have difficulty swallowing even water, like I get a bubble and have to pound my chest to get some relief.
    So I am a certifiable mess. I am sick and tired of being sick and tired. I am 54 years old and love life and dancing with my granddaughters. I only wish I could do this without pain and after they leave have the exhaustion that one would feel after a race.
    Any input?

  5. Kathy Says:

    I’m not a doctor so this is not formal medical advice, but your dry eyes, nose, joint pain and other symptoms sound like Sjogrens Syndrome which is very real. It is also commonly misdiagnosed; average time it takes to get a diagnosis is 5 years. It’s also commonly missed; They most likely tested you for antibodies to Sjogrens when they did a rheumatic profile. But some people are what is called “sero-negative” meaning they don’t produce antibodies to rheumatic disease when they are n fact positive for one or more. I would look for a rheumatologist who knows about Sero-negative disease. A lot of times they run a rheumatic profile, and they don’t consider the possibility of a patients being sero-native.
    Notice how if you read about Sjogrens that it will say something to the effect of ” 85% o people with Sjogrens will have antibodies” or something similar.
    You may want to google ‘sero-negativity” or you can look it up on pub-med or some other site like that. I hope this helps.
    Sjogrens is far more than “dry eyes and dry mouth” it can cause severe joint pain and severe problems with any of the moisture producing glands; Good luck, hope this helps.

  6. Wendy Says:

    Jean, I so wish I could talk with you. I have similar issues and was strong and healthy two years ago. We are the same age. I wonder if you’ve thought of or been checked for sjogrens syndrome? I suggests this to you because I have vasculitis and sjogrens as well as a few other AIDs. Sjogrens attacks your saliva glands and causes dry mouth, lips, nose and the worst is the eyes. I also have the ankles hurting so badly at times that I feel that my legs won’t hold me and all the symptoms you describe are very similar. Wouldn’t hurt to find another Rheumatologist and be tested. Good luck to you.

    Liked this article on Vasculitis..actually one that explains it in terms I understand. Thank you.

  7. Keith Says:

    I have been recently diagnosed with carotid artery dissection and an associated pseudoaneurysm. I had a recent CTA and that revealed another dissection and some other weird arterial formation. My neurosurgeon thinks I may have vasculitis, has anyone else out there experienced these issues associated with vasculitis? I seem unable to get answers, do any of you have recommendations for doctors who have had success treating vasculitis? I don’t mind travelling to get answers and good treatment. Thanks……………

  8. Domonique Says:

    In September 2015 I lost my vision in my left eye. It was brought on suddenly and within a matter of hours I could not see what was in front of me. All detail was lost. Come to find out I had a detached retina but what worried the doctors was my right eye was heading in the same direction as the left.I went through a lotbof testing and blood work and what they came up with was a rare disease called Eales Disease with secondary vasculitis. I underwent eye surgery to fix the detached retina and to try to stop the bleeding blood vessels along with other problems in my left eye. A few weeks later they did a lot of laser work in my right eye to hopefully prevent or prolong the right eye from getting worse. I am currently looking for others who have experienced this or has been diagnosed with Eales Disease and anyone out there to whom I can speak with about this form of vasculitis.


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