Giant Cell Arteritis
Wegener's Granulomatosis
Churg Strauss Vasculitis
Takayasu's Arteritis
Polyarteritis Nodosa
Central Nervous System Vasculitis
Cryoglobulinemia
About
Vasculitis
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Giant Cell Arteritis Wegener's Granulomatosis Churg Strauss Vasculitis Takayasu's Arteritis Polyarteritis Nodosa Central Nervous System Vasculitis Cryoglobulinemia
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About
Vasculitis
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Vasculitis is a general term used to refer to a group of diseases that are characterized by inflammation of blood vessels. Vasculitis can affect any organ in the body. The likelihood for severe illness and death is greatest when vasculitis affects many organs. However sometimes even when it occurs in only one organ (e.g. brain, eyes, intestines, and kidneys), severe consequences may follow. In only a minority of instances, have the causes of vasculitis been identified. This presents a major limitation in formulating effective therapies. Another barrier to progress has been an incomplete understanding of factors that predispose patients to be susceptible to vasculitis. With the dawn of the new millennium and availability of new biomedical techniques, there is great hope that we can identify environmental factors and immune susceptibility defects that lead to vasculitis.
Scope of Vasculitis
There are over 30 different types of vasculitis. Each is identified by different names, which most often recognize the first author who described the illness or the author of a seminal article about the illness. Consequently, there is Wegener’s granulomatosis (Friedrick Wegener), Takayasu’s arteritis (Mikito Takayasu), Churg-Strauss syndrome (Jacob Churg and Lotte Strauss), Kawasaki disease (Tomisaku Kawasaki) and so on. Other diseases have been named for the clinical setting in which they occur (allergic vasculitis) or based on biopsy features (giant cell arteritis).
The prevalence of certain specific types of vasculitis that have been well studied in the United States is about 3/100,000 population for Wegener’s granulomatosis, 6/100,000 for hypersensitivity (allergic) angiitis, 3/100,000 for polyarteritis nodosa, 20/100,000 for giant cell arteritis of the elderly, and 1/100,000 for Takayasu’s arteritis. For just this small sample of selected vasculitides, the combined prevalence is over 30/100,000 population. About 100,000 Americans per year are hospitalized for care of vasculitis. Children and adults, males and females, and individuals of any ethnic background may be affected. Whereas these numbers are relatively small, compared to heart disease or cancer, the impact of vasculitis is substantial. Systemic vasculitis is often life threatening and likely to produce disability or death. For example, in the case of Wegener’s granulomatosis, approximately 1500 patients are hospitalized for this illness in the US every year. Eleven percent die in the course of hospitalization, 31% become totally disabled in performing their usual occupation and 20% become partially disabled over 5 years from the time of disease onset. Between loss of personal income and hospitalizations, Wegener’s granulomatosis alone costs our health care system and patients over $40 million per year. This figure does not take into account the loss of income that results from an 11% mortality rate among hospitalized patients with this illness (Hoffman GS et al. Arthritis and Rheumatism. 41:1998; Cotch et al.: Arthritis Rheumatism. 39:1996).
Giant cell arteritis (GCA) principally affects patients over 50 years old (average age is 70). In some regions the annual incidence in patients >50 y.o. is 20-30 per 100,000 population (Denmark, Finland, Norway, Iceland and Minnesota). Another way of looking at these figures is 1 in 400-600 persons >50 y.o. may suffer from GCA. Although a minority of patients die from this form of vasculitis, GCA can be disabling and lead to stroke and/or blindness in up to 5% and 20% of cases, respectively (Baldursson et al: Arthritis Rheumatism 37:1994; Gran JT and Myklebust G: J Rheumatology. 24:1997).
Kawasaki disease (KD) has become the leading cause of acquired heart disease among children in Japan and North America. In Japan alone there are 5,000-6,000 new cases per year and 120-150 cases per 100,000 children less than 5 years old. In the US the annual incidence is 4-15 cases per 100,000 children less than 5 years old (Burns JC et al. Pediatrics 106:E27.2000). Although vasculitis of the coronary arteries is responsible for heart disease, vasculitis may affect other medium sized and large vessels as well. Two percent of children with KD die, usually due to myocardial infarctions.
Vasculitis commonly involves the kidney and can lead to end stage renal disease (ESRD) requiring dialysis and/or transplantation. The incidence of renal vasculitis in Europe is 15/million/year, which equates to 5,000 new cases in the EU countries (population 350 million). The mortality of this presentation is 25% at two years by which time at least one quarter of survivors will have renal failure. ESRD has an annual cost of $35,000/patient, which translates to an additional cost to the EU, for this subgroup of patients, of $300 million/year. These outcomes could be improved, and costs reduced, by earlier, more effective intervention.
Because these and related disorders are uncommon, medical expertise to provide care is limited, care may be compromised, and unnecessary morbidity and mortality often occur. Even in the best of hands, treatment in many cases of vasculitis is not curative and toxicity from therapy is universal. This in turn leads to permanent sequelae in many patients.