Biologic Treatment of Large-vessel Vasculitides

Valentin S. Schäfer; Jochen Zwerina


Curr Opin Rheumatol. 2012;24(1):31-37. 

In This Article

Abstract and Introduction


Purpose of review: Glucocorticoids are the mainstay of therapy for giant cell arteritis (GCA) and Takayasu's arteritis. However, a significant proportion of patients have a glucocorticoid-dependent or resistant disease course and serious adverse events are frequent. This article summarizes the current evidence on the use of biologic treatments in large-vessel vasculitis (LVV).
Recent findings: Antitumour necrosis factor (TNF) treatment strategies have failed to show efficacy in GCA, whereas their role in Takayasu's arteritis is yet unclear. Preliminary reports on the use of the anti-interleukin-6 receptor antibody tocilizumab (TOC) in both GCA and Takayasu's arteritis emerge. TOC was both able to spare glucocorticoid doses but could also induce remission in untreated GCA patients when used as monotherapy. There is little evidence for the use of biologic drugs in LVV associated with systemic rheumatic diseases. Case series suggest efficacy for anti-TNF antibodies in aortitis associated with relapsing polychondritis and pulmonary artery aneurysm associated with Behcet's disease.
Summary: TNF inhibition does not seem to save glucocorticoids or prevent relapses in GCA. Blocking TNF may provide benefit to difficult-to-treat patients with Takayasu's arteritis, relapsing polychondritis and Behcet's disease-associated LVV. Emerging reports on the use of TOC suggest an important role of IL-6 in the pathogenesis of large-vessel vasculitides and deserve further investigation. The use of TOC monotherapy to induce remission in untreated GCA patients proves fascinating.


Noninfectious rheumatic disorders can cause inflammation of the aorta and its main branches.[1] Two systemic vasculitides affect primarily large vessels: giant cell arteritis (GCA) and Takayasu's arteritis. In addition, several systemic rheumatic disorders including relapsing polychondritis, Behcet's disease and many others can present with aortic involvement (Table 1).

Aortic inflammation typically presents with distinct histopathological features: granulomatous inflammation is the main feature of GCA and Takayasu's arteritis and may also be observed in Crohn's disease and rheumatoid arthritis, whereas lymphoplasmacytic inflammation is frequently encountered in relapsing polychondritis, Behcet's disease, ankylosing spondylitis, IgG4- related systemic disease, etc.[2] The most common localization of large-vessel vasculitis (LVV) in these diseases is the aortic root and ascending aorta. However, some disorders such as GCA and Takayasu's arteritis typically affect the whole aorta and all branches. Clinical symptoms may range from an asymptomatic course to systemic symptoms such as fever, weight loss and high C-reactive protein levels. Aortitis of the ascending aorta may result in dilatation and thus aortic insufficiency causing life-threatening aortic dissection. If other parts of the large vessels are affected, both dilatation and narrowing of the vascular lumen with its clinical consequences are observed.

The mainstay of treatment in GCA and Takayasu's arteritis is medication with glucocorticoids, although this is less established in the aforementioned systemic rheumatic disorders in case of affection of large vessels.[3] There is no clear evidence on the use of adjunctive immunomodulating treatments in these diseases. Anti-cytokine treatments with monoclonal antibodies and fusion proteins ('biologicals') have revolutionized drug therapy of several inflammatory rheumatic diseases and could also be beneficial in large-vessel vasculitides. We therefore summarize current literature on the treatment of large-vessel vasculitides with biologic agents.


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