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Epidemiology of large-vessel vasculidities

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The systemic vasculitides are multisystem disorders characterised by the inflammation of blood vessels and tissue necrosis. Classified by the size of the vessels affected, the large vessel vasculitides include giant cell arteritis (GCA) and Takayasu's arteritis (TA). These are anatomically, epidemiologically and clinically distinct conditions. They are often associated with considerable morbidity and mortality. The classification of vasculitis has been an area of controversy for many years and current classification criteria remain suboptimal. Although intensive efforts are under way to improve them, a further understanding of the aetiology and pathogenesis of these diseases is required to develop more sensitive and specific diagnostic tests. These efforts, however, have been hampered by the low prevalence of these diseases. The establishment of national and international registries is encouraged to enhance valuable data collection. These are anatomically, epidemiologically and clinically distinct conditions. This article summarises the current classification systems for systemic vasculitis and their limitations. We also review the presently known epidemiology, risk factors and morbidity and mortality associated with GCA and TA.

Section snippets

Descriptive epidemiology

Vasculitis is considered to be primary when there is no known underlying aetiology, and secondary, if associated with an infection (e.g., human immunodeficiency virus (HIV), hepatitis B or C) or an underlying connective tissue disease (e.g., systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA)). Histopathological examination is the main basis for diagnosing these diseases; however, often is not feasible. Hence, to classify diseases for research purposes, we must rely on other

Classification

The presently available classification systems for vasculitis remain suboptimal. Many attempts have been made to reclassify these diseases and currently, the two mostly widely used remain the 1990 American College of Rheumatology (ACR) criteria and the Chapel Hill Consensus conference (CHCC) criteria.

Looking back, in 1952, Pearl Zeek was the first to propose a classification system for primary vasculitis based predominantly on blood vessel size [1]. She described five separate disorders

Case definitions

To allow comparisons and simplify the epidemiological analysis, the vasculidities are subdivided into primary (if there is no associated underlying disease) and secondary (a well-defined associated disease is present). The primary vasculidities are further classified into the size of the blood vessels predominantly involved. We will focus on adult large-vessel vasculitis using the generally accepted ACR criteria, Chapel Hill consensus definitions and the more recent attempt to merge them [12].

Large-vessel vasculitis

Giant cell arteritis (GCA) is also known as temporal arteritis and preferentially affects medium-and large-sized arteries in the elderly. It is the most common primary systemic vasculitis in adults and, by definition, occurs in those over the age of 50 years. In Europe and North America, the estimated prevalence is 200 per 100 000 and the incidence is 20–30 per 100 000 [13]. Although the condition had been considered less common among Hispanics, recent evidence has challenged this notion.

What are the time trends?

There is currently conflicting data as to whether there is an overall increase in the incidence and prevalence of GCA. A recent, 25-year epidemiologic study in northwestern Spain demonstrated a statistically significant increase in the incidence of GCA when comparing rates over five consecutive time periods from 1981 to 2005. The highest annual incidence rate in those over 50 years of age occurred between 1996 and 2000 [20]. Similar results were seen in studies in Olmsted County [18], *[43] and

Morbidity and mortality

The spectrum of clinical manifestations associated with GCA ranges from nonspecific complaints such as headache and myalgias to specific organ dysfunction such as visual loss, arm claudication or stroke. The major morbidity is related to both ischaemic complications of the disease and the immunosuppressive treatment. Partial or complete loss of vision occurs in 15–20% of patients in most series, whilst cerebrovascular ischaemic events affect 3–4% and thoracic artery aneurysms 7% of cases.

Gender

As in the majority of auto-immune diseases, GCA affects women 2–3 times more commonly than men. This difference appears to be more marked in the northern parts of Europe. Only in one earlier Spanish study [53] and a more recent study from Tunisia [39] were males reported to be predominant in GCA. However, a more recent analysis of extended data from the same Spanish population shows that the incidence of GCA was actually slightly higher in women, but not statistically significant [20].

In TA,

Difficulties in obtaining this information: what are the weaknesses of the data reported above?

The majority of data available in GCA comes from the Scandinavian countries, although there is more recently a growing body of studies from other areas. It is interesting to note that in the United States, there is only one population-based study. There remains a paucity of data from Asia, Africa, Australia and South America. The methods in which patients were diagnosed with GCA vary from study to study, but generally involved a hospital-based retrospective review of medical charts to determine

Summary

Classification systems for systemic vasculitis remain an area of ongoing review. However, without a reliable gold standard test, there will always be inaccuracies. GCA and TA are both relatively uncommon diseases that rely on large cohort studies to detect an adequate number of cases. This is not always practical. The development of multinational databases for patients with these conditions would certainly aid this effort. With improvements in the understanding of the aetiology of these

References (110)

  • J. Davson et al.

    The kidney in periarteritis nodosa

    QJM

    (1948)
  • J.G. Lanham et al.

    Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome

    Medicine (Baltimore)

    (1984)
  • J.F. Fries et al.

    The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary

    Arthritis and Rheumatism

    (1990)
  • G.G. Hunder et al.

    The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis

    Arthritis and Rheumatism

    (1990)
  • D.A. Bloch et al.

    The American College of Rheumatology 1990 criteria for the classification of vasculitis: patients and methods

    Arthritis and Rheumatism

    (1990)
  • J.C. Jennette et al.

    Nomenclature of systemic vasculitides. Proposal of an international consensus conference

    Arthritis and Rheumatism

    (1994)
  • N. Basu et al.

    EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis

    Annals of the Rheumatic Diseases

    (2010)
  • B. Hellmich et al.

    EULAR recommendations for conducting clinical studies and or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis

    Annals of the Rheumatic Diseases

    (2007)
  • R. Watts et al.

    Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies

    Annals of the Rheumatic Diseases

    (2007)
  • L.J. Liu et al.

    Evaluation of a new algorithm in classification of systemic vasculitis

    Rheumatology

    (2008)
  • R. Suppiah et al.

    Advances in the classification of primary systemic vasculitis

    International Journal of Advances of Rheumatology

    (2009)
  • P. Elling et al.

    Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection

    Journal of Rheumatology

    (1996)
  • P. Franzen et al.

    Giant cell arteritis and polymyalgia rheumatica in a region of Finland: an epidemiologic, clinical and pathologic study, 1984–1988

    Journal of Rheumatology

    (1992)
  • O. Baldursson et al.

    Giant cell arteritis in Iceland. An epidemiologic and histopathologic analysis

    Arthritis and Rheumatism

    (1994)
  • V. Petursdottir et al.

    The epidemiology of biopsy-positive giant cell arteritis: special reference to cyclic fluctuations

    Rheumatology (Oxford)

    (1999)
  • C. Salvarani et al.

    Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a 50-year period

    Arthritis and Rheumatism

    (2004)
  • C. Salvarani et al.

    Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in northern Italy

    Arthritis and Rheumatism

    (1991)
  • M.A. Gonzalez-Gay et al.

    Giant cell arteritis in Northwestern Spain. A 25-year epidemiologic study

    Medicine

    (2007)
  • C.A. Smith et al.

    The epidemiology of giant cell arteritis. Report of a ten-year study in Shelby County, Tennessee

    Arthritis and Rheumatism

    (1983)
  • F. Jonasson et al.

    Temporal arteritis

    Scottish Medical Journal

    (1979)
  • M. Sonnenblick et al.

    Giant cell arteritis in Jerusalem: a 12-year epidemiological study

    British Journal of Rheumatology

    (1994)
  • L. Pereira et al.

    Giant cell arteritis in Asians: a comparative study

    British Journal of Ophthalmology

    (2010)
  • Q. Zeng et al.

    10-Year epidemiological study on rheumatic diseases in Shantou area

    Zhonghua Nei Ke Za Zhi

    (1997)
  • R.D. Wigley et al.

    Rheumatic diseases in China: ILAR-China study comparing the prevalence of rheumatic symptoms in northern and southern rural populations

    Journal of Rheumatology

    (1994)
  • J.F. Cullen et al.

    Giant cell (temporal) arteritis in Singapore: an occult case and the rationale of treatment

    Singapore Medical Journal

    (2010)
  • I.A. Chaudhry et al.

    Epidemiology of giant cell arteritis in an Arab population: a 22-year study

    British Journal of Ophthalmology

    (2007)
  • J. Llorca et al.

    Lack of association between altitude and incidence of giant cell arteritis in northwest Spain [letter]

    Clinical and Experimental Rheumatology

    (2004)
  • S. Hall et al.

    Takayasu arteritis. A study of 32 North American patients

    Medicine

    (1985)
  • A.U. Waern et al.

    Takayasu’s arteritis: a hospital-region based study on occurrence, treatment and prognosis

    Angiology

    (1983)
  • K. El-Reshaid et al.

    Takayasu’s arteritis in Kuwait

    Journal of Tropical Medicine and Hygiene

    (1995)
  • E. Reinhold-Keller et al.

    Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register

    Arthritis and Rheumatism

    (2005)
  • R. Watts et al.

    The epidemiology of Takayasu arteritis in the UK

    Rheumatology (Oxford)

    (2009)
  • M. Vanoli et al.

    Takayasu’s arteritis: a study of 104 Italian patients

    Arthritis and Rheumatism

    (2005)
  • R.C. Lawrence et al.

    Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part II

    Arthritis and Rheumatism

    (2008)
  • E. Reinhold-Keller et al.

    Giant cell arteritis is more prevalent in urban than in rural populations: results of an epidemiological study of primary systemic vasculitides in Germany

    Rheumatology (Oxford)

    (2000)
  • S. Kobayashi et al.

    Clinical and epidemiologic analysis of giant cell (temporal) arteritis from a nationwide survey in 1998 in Japan: the first government-supported nationwide survey

    Arthritis and Rheumatism

    (2003)
  • T. Nasu

    Takayasu’s truncoarteritis in Japan: a statistical observation of 76 autopsy cases

    Pathologia et Microbiologia (Basel)

    (1975)
  • C. Salvarani et al.

    The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern

    Annals of Internal Medicine

    (1995)
  • C. Nordborg et al.

    The epidemiology of biopsy-positive giant cell arteritis: special reference to changes in the age of the population

    Rheumatology (Oxford)

    (2003)
  • J.T. Gran et al.

    The incidence of polymyalgia rheumatica and temporal arteritis in the county of Aust Agder, south Norway: a prospective study 1987–94

    Journal of Rheumatology

    (1997)
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