Gary S. Hoffman, M.D.

Harold C. Schott Chair of Rheumatic & Immunologic Diseases

Center for Vasculitis Care and Research

Cleveland Clinic

Cleveland, Ohio                              

January 2006

            2005 has been a critical and interesting year for our organization. Most of our members have supported the name change from the Wegener’s Granulomatosis Association (WGA) to the Vasculitis Foundation (VF). Others have felt a trust had been betrayed. Explanations were offered to try to convey the scientific and organizational logic for the change. Still, unfortunately, not everyone has "gotten it". In this article, we will try to make the reasons for change as clear as possible and hope that all of our readers will become better informed and most importantly satisfied that this is not only the right thing to do, but also the only logical course to follow.

            Almost all forms of vasculitis (inflammation of blood vessels) remain unsolved mysteries. The few for which causes are known include those in which infectious agents incite inflammation in the vessel wall. Others may be triggered by medication reactions. Rarely cancer cells directly invade the vessel and may cause vasculitis. These are a minority of all cases. Most forms of vasculitis present questions to doctors and researchers that are the same regardless of what form the disease takes or what unique name we give it. Examples of such questions are-

1. What has changed within the vessel or affected tissue that has caused white blood cells to be lured to it and incite injury?
2. Because white blood cells do not attack every vessel or every tissue, are there factors that protect some organs and not others?
3. What abnormalities might be present in immune cells that led them to be misdirected from their mission of protecting you to now hurting you (autoimmunity)?
4. What instructions, within the cell’s blue prints (genes), have been turned on or off that cause it to behave badly?
5. What changes occur in immune cells and injured organs when certain treatments induce remission? What changes when relapses occur?
6. Why do some areas respond more rapidly and completely to treatment and others lag behind or fail to respond at all?
7. What blood tests can we use to detect a relapse before it occurs and then start treatment before damage can take place?

            If researchers were able to answer these questions for one form of vasculitis, there is no doubt that the answers would be relevant to all other forms. This, in part, explains why doctors and researchers devoted to making life better for patients with vasculitis do not just work on one single type of vasculitis. The answers may come from any type and then be applied to the others. One obvious example is seen in the very common therapies that are used for this whole category of illnesses. It is no surprise that consultants for the WGA, now the VF, have a long history of providing care and conducting research for not only WG, but also Takayasu’s arteritis, polymyalgia rheumatica (PMR), giant cell arteritis (GCA), Churg Strauss syndrome, polyarteritis nodosa, microscopic polyangiitis, central nervous system vasculitis, Behcet’s disease, Henoch Schonlein purpura (HSP) and Kawasaki disease and others. Currently established international vasculitis workshops bring experts together who study all forms of vasculitis because what is learned about one disease informs about others. A break-through in Behcet’s disease or GCA can become a break-through in WG, CSS, HSP, etc. Indeed the landmark work for the treatment of WG at the NIH in the 1970’s provided a roadmap for the treatment of ALL severe forms of vasculitis. Thus, just as doctors who are devoted to WG must also be well studied in other forms of vasculitis, the same should be said of the curious mind of affected patients and their families.

            These issues were among those addressed at the first formal retreat of the Board of the VF and its consultants on January 13 in Chicago. The commonalities shared by the vasculitides led to discussions of how we can do better for all patients with vasculitis. Agreement was reached in many areas, some of which you will find quite interesting. Let’s examine some lines of discussion from the meeting:

1. Doctors for people are not the only ones interested in vasculitis. So are veterinarians! Did you know that many different types of vasculitis have been found in animals? Mice, chickens, dogs, horses and even elephants, and that is only a small incomplete list. Many of these diseases have been found to have infectious causes and in most cases, occur in animals that have well understood immune abnormalities. We concluded that "Vets" should join in the problem-solving exercises to find causes and cures for vasculitis. This is not ongoing and should be.
2. Because certain forms of vasculitis are known to be due to infections and the majority of infectious agents in the universe cannot be identified in hospital laboratories, using routine tests, it was agreed that infectious disease specialists should help in finding the cause(s) of vasculitis.
3. Sometimes breakouts of vasculitis occur within common times and places, suggesting an infectious or other environmental cause. Epidemiologists are specifically trained to examine these events and trace illnesses to their root causes. They too should be part of the search to help patients with vasculitis.
4. Children suffer from many of the same diseases as adults, but we all know that they are also prone to illnesses that are not seen in adults. Conversely, adults may not be prone to certain childhood diseases. Examples include Kawasaki’s disease, a form of vasculitis that almost only occurs in children. Giant cell arteritis, on the other hand, never occurs in children and affects adults for whom the average age is 74. What factors establish vulnerability at different stages in one’s life? We agreed that Pediatricians need to play a greater role in this dialog.
5. So much of illness is genetically determined. This is not as obvious as one gene causing one disease, but instead is more likely to be complex sets of formulas in which the interplay of many genes may complete a pathway that leads to disease expression. It was agreed that geneticists need to become more involved in vasculitis research.

          How about health care services for patients with vasculitis? Are there delays in diagnoses? Do judgment errors occur in diagnosis and treatment? Are there adequately trained doctors with vasculitis experience in every city, region or state? The answers are obvious to every reader. There are inadequate services, expertise and delays and errors do occur. The solution? We need to train experts to be so numerous and well informed so that these problems are lessened and in time eliminated. The National Institutes of Health (NIH) provides some funds to train vasculitis experts, some individual universities/medical centers have designated modest parts of their budgets for vasculitis training --- but this is barely enough to provide for sorely needed services and researchers.

            The VF is committed to raising monies to help train vasculitis specialists.

            In Chicago, discussions with NIH administrators and the VF were also directed at joint sponsorship of retreats that would bring experts of every type together, devoted to vasculitis, to examine our knowledge base, treatment protocols, patient needs and research agendas, to ensure that the best strategies were in place to bring us closer to better treatments and ultimately cures for all forms of vasculitis.

            Does the notion of economy of scale apply to vasculitis? You bet! Can 12-13,000 American patients with WG influence government funding agencies and philanthropies to provide the 10s or 100s of millions of dollars necessary to solve the WG mystery? It has not happened so far. Is it more likely that such funding sources will respond to a lobby of 2 million vasculitis patients and their doctor advocates who appreciate the features common to these diseases, the burden of disease for patients, the resultant impact from disability, family dynamics and effects on national health care costs? It is really quite simple. All vasculitis patients need to band together, to advocate for each other, to petition for better care, more wide-spread training of doctors who should be able to recognize and treat their diseases in a timely fashion, using state of the art diagnostic tests and therapies, and funds for patient education and research. Who will lead this charge? It was agreed that the VF should be that organization. Changing the name from WGA to VF was not just a name change. It represents a new vision, and a new mission.

            During the next year the VF will be asking interested individuals, including members of disease-specific advocacy groups to consider applying for positions on the VF Board. To serve our patients best, we will try to identify roles for all who are willing to serve. Board members will be selected based on past experience, skills, intentions and as all of our 3rd grade teachers told us was so important, whether he or she "works and plays well with others." The consultant group will also expand to include people with many new skill sets, including veterinarians, specialists in pediatrics, infectious diseases, genetics and epidemiology…. and whoever we need to get us to better therapies and cures!!!!

We know the VF will succeed. So many people have devoted so much, for so long, and asked for nothing other than a better life for our patients. Failure is not an option.

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