12Epidemiology of large-vessel vasculidities
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
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An unusual case of Takayasu arteritis presenting as isolated severe aortic regurgitation: A case report
2023, International Journal of Surgery Case ReportsRadiologic Imaging in Large and Medium Vessel Vasculitis
2020, Radiologic Clinics of North AmericaAortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases
2020, Presse MedicalePrognosis and future developments in vasculitis
2018, Best Practice and Research: Clinical RheumatologyCitation Excerpt :There was, however, a signal suggesting a specific risk in the context of thoracic dissection; a more recent follow-up study of the cohort indicated that all aortic manifestations may indeed be associated with increased mortality (HR: 3.4; 95% CI: 2.2, 5.4) [62]. Furthermore, mortality due to ruptured aortic aneurysms in patients with GCA is estimated to be approximately 80%, compared to that of 65–75% seen in all other patient groups [63]. The true hazard of large-vessel involvement in the context of sensitive modern imaging has yet to be fully characterised.
Outcomes of percutaneous coronary intervention and coronary artery bypass grafting in patients with Takayasu arteritis
2017, International Journal of CardiologyCitation Excerpt :The inflammatory process initially leads to thickening of the arterial wall and may result in stenosis, occlusion, dilatation or aneurysm formation [4]. The degree of arterial lesions and the extension of vascular involvement usually determine the heterogeneity of clinical manifestations [3]. As arterial lesions ensue, more characteristic features of TA may be found such as dizziness, hypertension, decreased or absent peripheral pulses, blindness or visual disturbances, limb claudication, syncope, angina pectoris, myocardial infarction or even sudden cardiac death [5,6].
Clinical manifestations of Monckeberg's sclerosis. Report of case and literature review
2017, Revista Colombiana de Reumatologia