Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are the two main subtypes. GCA is the most common vasculitis affecting adults aged 50 years or more, while TAK is a rare vasculitis that affects younger individuals mainly under 40 years. Clinical presentations vary from asymptomatic to significant systemic symptoms such as fever, weight loss, and symptoms that result from aortitis and high inflammatory markers; C-reactive protein (CRP); and erythrocyte sedimentation rate (ESR) levels. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use.
Category
Immune system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A44 Vasculitis
H01465 Large-vessel vasculitis