Page 308 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

282 Dimitri Chanouzas and Matthew David Morgan

Treatment
Treatment of TAK can be divided into pharmacological therapy aimed at suppressing

inflammation, and revascularization. Corticosteroids are the mainstay of pharmacotherapy
although methotrexate, azathioprine, anti-tumor necrosis factor (TNF) therapy, and
cyclophosphamide have all been used in patients with TAK refractory to corticosteroids
[22-24].

Revascularization treatment in TAK is delivered by open conventional bypass grafting or
endovascular means including, percutaneous transluminal angioplasty with or without stent
insertion [25]. Advances in interventional radiology have led to more widespread use of
endovascular techniques in the management of high-risk patients. There are several patient
reports and case series of TAK in the literature describing the successful outcome of
endovascular repair of challenging stenotic and aneurysmal lesions (26-30). Comparisons of
the surgical versus endovascular management of lesions of the abdominal aorta in TAK
shows that the risk of complication especially restenosis is higher with endovascular than
surgical intervention [31, 32]. A recent multicenter retrospective study found that the risk of
complications following endovascular repair was 50% compared to 37.5% after surgical
repair [32]. Multivariate analysis showed that patients with TAK and evidence of
inflammation as defined by the indices of an erythrocyte sedimentation rate (ESR) > 30
mm/hour and C-reactive protein (CRP) level > 6 mg/L at the time of revascularization, were
7-fold more likely to develop post-procedural complications including restenosis.

Giant Cell Arteritis

Epidemiology and Classification
Giant cell arteritis (GCA) is characterized by granulomatous inflammation that

predominantly affects the thoracic aorta and its branches. It has an age of onset of greater than
50 years with an increased incidence in those 70 to 80 years of age, and a higher incidence in
Northern Europeans and North Americans of Scandinavian descent compared to other ethnic
groups [33]. The 1990 ACR criteria shown in Table 4 were intended for the classification not
the diagnosis of GCA [34].

Clinical Features
The commonest manifestation of GCA is headache followed by scalp tenderness, jaw

claudication and visual disturbances; constitutional symptoms such as fever, fatigue and
weight loss are also prominent [4, 33]. About 20% of patients with GCA develop clinical
manifestations reflecting large vessel involvement including aneurysmal and stenotic lesions
of the abdominal aorta which in turn can lead to GI sequela [33].

Gastrointestinal Involvement
Asymptomatic involvement of the liver, as manifested by elevated serum alkaline

phosphatase and transaminase levels, is reported in up to 50% of patients with GCA [35].
Symptomatic liver involvement with granulomatous inflammation on liver biopsy is rare with
only a few reported cases [36].

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