Immunopathophysiology of Large Vessel Involvement in Giant Cell Arteritis — Implications on Disease Phenotype and Response to Treatment

Abstract

The disease affects, mainly, the large-and medium-sized extracranial branches of the carotid artery and, classical clinical features, such as headache, jaw claudication, scalp tenderness and visual impairment, are closely related to this marked cranial tropism of GCA. [3] On a histopathological basis, GCA involves all layers of the arterial wall, including the adventitia. Inflammatory lesions consist of activated T cells, dendritic cells (DCs) and macrophages. These lesions are believed to be the histopathologic hallmark of GCA and are characterized by a predominance of mononuclear infiltrates or granulomas, usually with multinucleated giant cells. [4] Besides the inflammation of the carotid branches, involvement of the great arteries, such as the aorta and its main tributaries, was initially recognized in the late 1930s and reported sporadically thereafter in necropsy or histopathologic studies of surgically resected tissues. [5,

Giant cell arteritis and systemic sclerosis: a rare overlap syndrome

“…7 However, the presence of typical GCA in this patient may suggest a rare overlap syndrome since the pathogenetic mechanisms of these diseases differ considerably. 8 The use of glucocorticoids is considered to be the gold standard in treatment of GCA. Especially patients at high risk of visual loss or other macrovascular complications should start therapy with prednisone or equivalent as soon as possible.…”

The Aortic Pathologies: How Far We Understand It and Its Implications on Thoracic Aortic Surgery

“…Tunica media injury induces the genesis of laminar medial necrosis [ 18 ] . The pathogenesis of GCA involves recruitment and stimulation of antigen specific T cells, IFN-γ upregulation, and activation of macrophages in the adventitial layer, which secrete IL-1, IL-6, and MMPs ( Figure 1 ) [ 5 ] . In TA, the pathogenesis has not yet been elucidated ( Figure 2 ).…”

“…The aetiology in many cases is unknown. Sometimes it can be attributed to recent infection, especially of a viral nature [ 5 , 6 ] . Thickening, narrowing, weakening, or scarring of the vessel wall is observed, which restricts blood flow.…”

“…Thickening, narrowing, weakening, or scarring of the vessel wall is observed, which restricts blood flow. Takayasu's arteritis (TA) and giant-cell arteritis (GCA) are subtypes of vasculitis and affect larger arteries such as the aorta [ 5 ] .…”