Typical clinical features indicating active disease include new loss of pulses, painful vessels (typically carotidynia) and new bruits. Initial therapy is with high-dose glucocorticoids usually in combination with a steroid sparing agent. An open-label study of patients, who were refractory to glucocorticoid therapy, showed that weekly low-dose methotrexate was effective in inducing remission in 13 RO4929097 concentration of 16 cases [86]. In a prospective study of 65 newly diagnosed Takayasu’s

arteritis patients treated with azathioprine and prednisolone and followed-up for 1 year, therapy was safe, well tolerated and effective in ameliorating systemic symptoms and laboratory measures of disease activity within 3 months. Although it did not reverse angiographic lesions, it did halt disease progression [87].

Maintenance.  Despite glucocorticoid therapy, subclinical disease can persist, as demonstrated on magnetic resonance imaging. Approximately half of all Takayasu’s arteritis patients have chronic active disease for which glucocorticoid therapy alone does not provide sustained remission [88]. Therefore, the use of adjunctive therapy in addition to glucocorticoids is common, both to improve disease control and to reduce overall steroid use [17]. Methotrexate has been used in refractory cases of Takayasu’s arteritis. In one study, eight of the 16 patients who achieved remission on initial methotrexate and glucocorticoid JQ1 molecular weight therapy sustained remissions lasting 4–34 months (mean 18 months), and four patients did not require further glucocorticoid or methotrexate therapy. However, three patients experienced disease progression despite treatment. PRKACG Patients were followed-up for a mean period of 2·8 years. Further long-term studies are required to assess the durability of

remission and the need for long-term maintenance therapy in this subset of patients [88]. Takayasu’s arteritis may result in permanent stenosis, despite remission of the disease. It is important to differentiate the features of disease for which further immunosuppressive agents are required, from abnormalities due to damage to vascular anatomy in which surgical intervention is more appropriate [88]. Reconstructive surgery should be undertaken at expert centres and preferably during the quiescent phase of the disease [17]. Polyarteritis nodosa and Kawasaki disease are the two major categories of medium-sized vessel vasculitis. Both have acute necrotizing arteritis with inflammatory aneurysm formation. Patients with polyarteritis nodosa present with a multi-system illness with constitutional features such as weight loss, fever, myalgia, development of a rash, neuropathy or abdominal ischaemia. Polyarteritis nodosa is associated commonly with hepatitis B infection. Induction.