a special program of the National Emergency Medicine Association (NEMA)

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Week: 594.6 

Guest: Dr. Michelle Petri, Head of Lupus Center, Johns Hopkins Med. School 

Topic: Lupus treatment update 

Host/Producer: Steve Girard

NEMA: Lupus is a disease that until recently defied definition. It strikes everywhere, and leaves many people severely affected. There have been many advances in the treatement and understanding of the disease...and today we have one of the experts with us...Dr. Michelle Petri of Johns Hopkins Hospital. Dr. Petri, let’s talk about what we know about lupus...  

PETRI: Systemic lupus erythemetosus is a chronic autoimmune disease, and the basic thing that goes wrong is that the immune system, which is supposed to fight infection, starts to attack normal body organs. The most common organs that are attacked are the skin, causing skin rashes...the kidneys, causing kidney failure...and the joints, which can cause terrible swelling, what we call polyarthritis, because it’s usually many joints at the same time that are affected. We try to control lupus by giving medications that suppress the immune system...especially a medication called pregizone. This isn’t a cure, though. At best what we do is control the disease. There is certainly much better survival nowadays than there was in the 1950’s, when half the patients with lupus would die within 5 years. Now, the 20 year survival is 95% or better in most centers. However, the patients who survive...almost half of them are surviving with permanent organ damage. So, the real problem that we have is that the quality of life of patients with lupus is not very good. In fact, it’s about equal to the quality of the life of patients who have HIV infection.  

NEMA: I understand certain sections of the population are more severely affected...  

PETRI: Typically, this is a disease of women...because 90% of patients who have lupus are women. It is more common in certain ethnic and racial groups. About 1 out of 400 caucasian women have lupus...but about 1 in 250 African American women have lupus, and we also believe it is more common in Hispanics and in Asians.  

NEMA: There are some areas of research and treatment that have looked promising since I spoke with you last year...  

PETRI: There are actually quite a few clinical trials underway of new approaches and new medications for lupus. One of the new clinical trials involves a natural hormone that we all make called DHEA. Many people may have seen this being sold in health food stores, because there’s great interest in DHEA for multiple conditions, not just lupus. The reason there’s interest in lupus, is the women who have lupus have very low levels of DHEA, and these low levels may be contributing to abnormalities in their immune systems...especially in their creation of cytokines, which are messengers in the immune system. There is a national, multi-center trial underway of DHEA...to determine if it helps disease activity. It appeared to be very promising in small, initial trials that were done at Stanford University in California, and in addition to helping disease activity in lupus in those trials, DHEA also appeared to prevent some of the complications of pregnizone...especially osteoporosis. So I think many patients are hopeful that the DHEA trial will prove that this drug is beneficial to patients, because DHEA is very safe. Perhaps as many as 1 out of 4 women...to 1 out of 3 women who take DHEA may get acne, but acne is very easily treated. Other than acne, side effects are almost unheard of. There are also several clinical trials either already underway, or about to begin for new approaches for lupus kidney disease. Unfortunately, even with modern treatment, including chemotherapy...many patients who have lupus kidney disease have such severe scarring in their kidneys that they require dialysis or kidney transplant when their kidneys fail.

A new approach that’s being developed to treat kidney lupus is to prevent the immune system from making the lupus antibodies that attack the kidneys. This is called a ‘B cell toleragen’, because the ‘B cells’ are the lymphocytes that make lupus antibodies, and tolerance means that they will no longer recognize the kidney as being abnormal or foreign, and will stop attacking it. These kinds of approaches would mean, if they’re effective, that patients with lupus...even with the most severe forms of kidney disease, might not need chemotherapy...and for young women, this would be a big step forward. Because the chemotherapy we use for bad kidney disease is called ‘cytoxian’, can cause permanent ovarian failure - sterility. And since many of the young women who are struck down with lupus have not had families. Being able to avoid cytoxian would help their quality of life immensely.  

NEMA: We’ve talked briefly about those lupus victims who are losing the battle against lupus, even with chemo and pregnizone...  

PETRI: And because lupus can still be a fatal disease, some investigators have looked for very aggressive methods of treating it. And one that is being considered, and is already being tested at several centers in the United States is bone marrow transplantation. Now we know from some very early studies that were done in patients with lupus that actually had cancer, that sometimes the lupus would go away if the patient with cancer was given a bone marrow transplant. And this what really sort of whetted the interest of investigators into using this approach for the patients whohave the very worst forms of lupus. Now, when bone marrow transplanst are done for patients with cancer, what’s usually done is the person with cancer is treated initially to destroy their own immune system with radiation and chemotherapy, and then they’re given a bone marrow transplant from someone else. Hopefully from someone who matches them quite closely, so there won’t be a problem with rejection. But for patients with lupus, this is thought to be too drastic, because if you get a bone marrow from someone else, (edit this term) there is a chance of complications down the road, especially a complication called ‘graft versus host’, where the bone marrow actually starts to attack the person..and that can be a deadly complication. So most physicians and investigators didn’t want to do bone marrow transplants for patients with lupus if the risk of death was too high. If the risk of complications from the procedure was too high. So it was thought important to look at a new approach, and a new approach was the use the person’s own bone marrow...and this is called an ‘otalogous’, meaning a person’s own bone marrow...stem cell transplants. Stem cells are the cells in the bone marrow that are so essential to allowing the bone marrow to procreate, proliferate and repopulate the bone marrow. Now in order to do a bone marrow transplant, what is going to have to happen with a patient with lupus is that she will first donate her bone marrow, and then the bone marrow has to be cleaned of all the lymphocytes - the ‘B cells’ and ‘T cells’ that were causing the disease...so that only the stem cells, the progenitor cells of the bone marrow, are left. While the bone marrow is being cleansed of all these bad cells, the person herself will have to undergo some preparation to kill the lymphocytes in the rest of her body...usually with chemotherapy and with radiation. And then her own stem cells will be given back to her, after again they’ve been cleansed of those ‘B and T cell’ lympocytes that were causing the lupus problem. It’s been done in a few patients. We don’t know at this point whether it would offer a person an opportunity of a cure. Perhaps all we willreally be offering patients is a long period of remission. It is quite an imposition in a person’s life, because it usually requires three to four weeks in the hospital, perhaps even longer, while the person’s stem cells are replicating themselves. Until they do, the person is at very severe risk of infection. So, this is not ever become a therapy that’s offered to everyone with lupus, it will probably only be offered to patients who have the very worst forms of lupus.  

NEMA: It does seem like a dangerous, last chance scenario...  

PETRI: To spend four weeks in the hospital, and knowing that during those four weeks that they would be at risk of life-threatening infections because their own immune system has not yet been reconstituted...that’s a big decision for any young woman with lupus to have to make. We are concerned about this kind of procedure because there is a genetic predisposition to lupus, and we’re afraid that even if all those B and T lymphocytes that are making the lupus antibodies are cleansed from the woman’s bone marrow before it is given back to her, her own stem cells are genetically capable of making the same mistake again. And causing new B & T lymphocytes to be made that makes that lupus mistake, and start to attack cells. So, this is a procedure right now that is under investigation, but is still quite controversial, because we don’t know what the long term effects might be, and we don’t know whether we’re talking about a period of remission for a patient who’s been critically ill, or whether for some patients this might represent a cure.  

NEMA: The DHEA trials...how long will they be going on?  

PETRI: Ongoing right now, and in fact the trials have not yet completed their enrollment quotas. So, we’re probably about a year and a half away from completing this most recent DHEA trial, which is underway.  

NEMA: There seems to be a lot of promise in treatments since we last spoke...  

PETRI: I think it’s very promising, especially since there are pharmaceutical and biotechnical firms that are very interested in treatments for lupus. For too many years, lupus has been that forgotten women’s disease...where people were suffering, people were dying, but that there weren’t new approaches because companies weren’t interested in the disease. I’m glad that it’s changing, but it’s way overdue!  

NEMA: Where can people get more information on lupus and its treatments?  

PETRI: There is the Lupus Foundation of America, which is in Rockville, Maryland. The National Arthritis Foundation in Atlanta, Georgia is also a wonderful sources of information on lupus. The National Institutes of Health also has a patient information line. And for patients who are interested in new clinical trial for lupus...they are welcome to call our office.  

NEMA: Dr. Michele Petri...head of the Lupus Center at Johns Hopkins Hospital in Baltimore. For more information, contact the Lupus Foundation of America, in Rockville, Maryland.  

SPOT: 15 years in the prevention of heart disease, stroke and trauma - The National Emergency Medicine Association. This show is just part of what NEMA does. We send out millions of pieces of prevention information to people around the country, give grants to organizations in research, public information and emergency services, and have been instrumental in the creation and expansion of the Chest Pain Emergency Room movement. To play a role, call 800-332-6362.  

NEMA: Thanks for joining us for today’s program. If you have any comments or suggestions, contact this station. Or visit our home page at: www.nemahealth.com/ ...for a look at transcripts of this or past programs, or to find out more about the National Emergency Medicine Association. I’m Steve Girard at The Heart of the Matter.