Clinical diagnosis and management of large vessel vasculitis: giant cell arteritis

Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA) and Takayasu arteritis (TA). Less commonly LVV occurs in various Other Diseases. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Occasionally LVV is discovered unexpectedly on pathological examination of a resected aortic aneurysm. Clinical evaluation is often unreliable in determining disease activity. Moreover, the diagnostic tools are imperfect. Acute phase reactants can be normal at presentation and available imaging modalities are more reliable in delineating vascular anatomy than in providing reliable information on degree of vascular inflammation. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use. Several steroid-sparing agents have also shown some promise and are currently in use. Endovascular revascularization procedures and open surgical treatment for aneurysms and dissections are sometimes necessary, but results are not always favorable and relapses are common. This article, the first in a series of two, will be devoted to GCA and isolated (idiopathic) aortitis, while TA will be covered in detail in the next article.