Pathogenic principles in giant cell arteritis

In giant cell arteritis, an immune insult in the vascular wall initiates a reaction in the artery that leads to structural changes, intimal hyperplasia, and luminal occlusion. The mechanisms triggering the immune stimulation are unknown; however, the process is strictly dependent on T cells that are found in the vicinity of the vasa vasorum in the adventitia and that produce interferon-gamma. The major effector cells in the artery are macrophages and giant cells that are ultimately under T-cell control but assume different functions depending on their location in the arterial wall. The response of the artery to the injury is maladaptive and includes mobilization and proliferation of smooth muscle cells in conjunction with matrix production and neoangionesis, resulting in the formation of a lumen-obstructive neointima. Heterogeneity in the immune insult and the resulting arterial response patterns correlate with variations in clinical disease.