Vasculitis is a heterogeneous group of mostly autoimmune-mediated inflammatory diseases. These rare disorders are characterized by the presence of inflammatory cells in the vessel wall with damage to mural structures, which may lead to artery dilatation, or more often to stenosis/occlusion and downstream tissue ischaemia. The inflammation may be focal or widespread throughout the entire vascular bed of a particular organ/artery, or even affecting several organs and different vessels. Vasculitis is classified based on the location and size of affected vessels. Vasculitis is a heterogeneous group of mostly autoimmune-mediated inflammatory diseases. These rare disorders are characterized by the presence of inflammatory cells in the vessel wall with damage to mural structures, which may lead to artery dilatation, or more often to stenosis/occlusion and downstream tissue ischaemia. The inflammation may be focal or widespread throughout the entire vascular bed of a particular organ/artery, or even affecting several organs and different vessels. Vasculitis is classified based on the location and size of the affected vessels. Vasculitis can occur as a primary condition or may be secondary to another disease (connective tissue disorder, viral infection, malignancy, etc.). The inflammation may be infectious or non-infectious, the latter being more common. In particular, isolated aortitis have to be dichotomized into infectious entities, caused by direct invasion and proliferation of pathogens with resultant inflammation, versus non-infectious ones. The pathogenic mechanism of most non-infectious vasculitic disorders has not been completely elucidated yet. Histopathology and immunopathology studies reveal inflammation of the artery wall with predominance of CD4+ T lymphocytes and macrophages, which undergo granulomatous organization with formation of giant cells. There is also a loss of vascular smooth muscle cells (VSMC) and elastic fibres that may facilitate aneurysm formation. The term large-vessel vasculitis (LVV) covers a spectrum of primary vasculitis predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA), previously known as Horton’s disease or temporal arteritis, and Takayasu’s arteritis (TA). These two differ in the age of onset with GCA rarely occurring before the age of 50 and TA rarely after 40 years of age. Clinical manifestations result from arterial stenosis and/or occlusion leading to tissue ischaemia and dilatation with rupture or dissection as a potential complication.

Large Vessel Vasculitis

章节
发表日期：

2026年

查看全文

Debora Karetová,
Josef Marek

摘要

Vasculitis is a heterogeneous group of mostly autoimmune-mediated inflammatory diseases. These rare disorders are characterized by the presence of inflammatory cells in the vessel wall with damage to mural structures, which may lead to artery dilatation, or more often to stenosis/occlusion and downstream tissue ischaemia. The inflammation may be focal or widespread throughout the entire vascular bed of a particular organ/artery, or even affecting several organs and different vessels. Vasculitis is classified based on the location and size of affected vessels. Vasculitis is a heterogeneous group of mostly autoimmune-mediated inflammatory diseases. These rare disorders are characterized by the presence of inflammatory cells in the vessel wall with damage to mural structures, which may lead to artery dilatation, or more often to stenosis/occlusion and downstream tissue ischaemia. The inflammation may be focal or widespread throughout the entire vascular bed of a particular organ/artery, or even affecting several organs and different vessels. Vasculitis is classified based on the location and size of the affected vessels. Vasculitis can occur as a primary condition or may be secondary to another disease (connective tissue disorder, viral infection, malignancy, etc.). The inflammation may be infectious or non-infectious, the latter being more common. In particular, isolated aortitis have to be dichotomized into infectious entities, caused by direct invasion and proliferation of pathogens with resultant inflammation, versus non-infectious ones. The pathogenic mechanism of most non-infectious vasculitic disorders has not been completely elucidated yet. Histopathology and immunopathology studies reveal inflammation of the artery wall with predominance of CD4+ T lymphocytes and macrophages, which undergo granulomatous organization with formation of giant cells. There is also a loss of vascular smooth muscle cells (VSMC) and elastic fibres that may facilitate aneurysm formation. The term large-vessel vasculitis (LVV) covers a spectrum of primary vasculitis predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA), previously known as Horton’s disease or temporal arteritis, and Takayasu’s arteritis (TA). These two differ in the age of onset with GCA rarely occurring before the age of 50 and TA rarely after 40 years of age. Clinical manifestations result from arterial stenosis and/or occlusion leading to tissue ischaemia and dilatation with rupture or dissection as a potential complication.