The use of tumour necrosis factor (TNF) inhibitors has so far been disappointing in giant cell arteritis [114], and remains relatively untested in Takayasu’s arteritis.

It is unlikely that such therapies will be used www.selleckchem.com/products/Rapamycin.html in Kawasaki disease or polyarteritis nodosa. For ANCA-associated vasculitis it is important to consider a biological approach, given the greater understanding of the underlying pathology. Long-term use of etanercept has proven disappointing in Wegener’s granulomatosis [115], although short-term use of TNF inhibitor therapy has been effective in acute disease [116]. Infliximab has been used in a study of 28 patients with systemic vasculitis, resulting in 88% achieving remission but severe infections in 20% [117]. Rituximab is a chimeric monoclonal IgG1 antibody directed against CD 20 leading to the destruction of B cells via complement-mediated lysis and antibody-dependent cellular cytotoxicity. Because ANCA are involved

in the pathogenesis of small vessel vasculitis, it stands to reason that rituximab may be an effective and safe treatment. It might be postulated that ANCA-positive disease would respond better than ANCA-negative vasculitis. There is evidence of benefit in using rituximab in Wegener’s granulomatosis to achieve remission in patients who have failed conventional selleck chemicals therapy, but given the small numbers of published cases there is a need for large randomized controlled trials, which are currently under way [118]. These include the RAVE study comparing cyclophosphamide with rituximab in inducing remission in patients with severe ANCA-associated vasculitis. A potential

problem in AASV is that the full therapeutic Ixazomib nmr effect of rituximab may be delayed for up to 3 months, and so may not have a role as a single agent in patients with rapidly progressive disease. It might be expected that rituximab would work better in antibody-positive disease, but this has not been shown. Imatinib mesylate is an inhibitor of a class of tyrosine kinases and inhibits T cell activation and proliferation. In vitro it impairs conversion from naive to memory T cells after T cell activation using cells from patients with AASV [119]. It was found to inhibit platelet-derived growth factor (PGDF)-mediated responses strongly in myointimal cells in giant cell arteritis and may have therapeutic potential to limit ischaemic complications in large vessel vasculitis [120]. The vasculitides remain a challenge in terms of diagnosis and treatment. The recognition of disease remains unsatisfactory in the absence of any gold standard tests. The clinical presentation and correct use of appropriate laboratory tests, imaging and pathology are essential to assist in making an early diagnosis. The patient should be assessed by clinicians familiar with vasculitis to plan treatment.