[Giant-cell arteritis and Takayasu arteritis: epidemiological, diagnostic and treatment aspects]. / Artérite à cellules géantes et maladie de Takayasu : aspects épidémiologiques, diagnostiques et thérapeutiques.

Giant-cell arteritis (GCA) and Takayasu arteritis (TAK) are primary systemic granulomatous large-vessel vasculitides. Whether both entities represent distinct phenotypic expressions of a shared etiopathogenic process remains hypothetical. GCA more commonly affects subjects of northern European background while the clinical observation that TAK might be more common in populations of Asian or African ancestry needs to be confirmed by epidemiological studies. Distinct human leukocyte antigen class II associations were identified as genetic risk factors of GCA and TAK. The increasing incidence of GCA also suggests an environmental cause. Temporal artery biopsy is the main diagnostic test for GCA, although MRI and Doppler ultrasonography of the temporal or occipital arteries may also reveal vessel wall inflammation. MRI, CT and positron emission tomography with 18F fluodeoxyglucose have progressively replaced conventional invasive imaging modalities for study of large-vessel disease. The diagnostic accuracy of these 3 imaging modalities seems equivalent, but their value in the follow-up of GCA and TAK is less clear. According to studies based on modern imaging techniques, 70-80% of patients with newly diagnosed GCA show an involvement of the aorta and/or the major branches of the aorta. Glucocorticoids are the reference therapy for GCA. Adjunctive therapy, notably with methotrexate, appears to enhance disease control and reduce glucocorticoid exposure. IL-6 blockade has hope as a new treatment option for GCA. Principles of therapy for TAK are similar to those of GCA, except that TNFα blockers, particularly infliximab, seem to show good results in TAK and revascularization procedures are an important part of the TAK therapy.

Therapeutic plasma exchange in systemic vasculitis: an update on indications and results.

PURPOSE OF REVIEW: Therapeutic plasma exchange has been long proposed as a potentially useful modality to treat several systemic vasculitis conditions. This review summarizes the available evidence for the effectiveness of plasma exchange in systemic vasculitis. RECENT FINDINGS: Therapy with plasma exchange, most often combined with immunosuppressive agents, has been found effective for antiglomerular basement membrane disease, antineutrophil cytoplasmic antibody-associated vasculitis (AAV), Henoch-Schönlein purpura, cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis nodosa and Kawasaki disease. The most common indications are life-threatening or organ function-threatening manifestations, particularly advanced renal dysfunction and disease refractory to traditional therapy. Thus, most of the available evidence favoring plasma exchange in these circumstances is from small observational studies or expert consensus. Recent advances in findings include results strengthening the notion of a small beneficial effect on preserving renal function with adjunct plasma exchange therapy in AAV with renal failure and observational data suggesting plasma exchange as a promising effective salvage option for children with Kawasaki disease not responding to standard therapy with intravenous immunoglobulins. SUMMARY: Evidence from case reports and case series and a few randomized controlled trials continues to support plasma exchange as a major rescue-treatment modality for several systemic vasculitis diseases. These studies offer some guidance for use of plasma exchange in systemic vasculitis, but additional data from controlled trials are needed for more accurate assessment of the indications, practical modalities, benefits and shortcomings of this treatment approach.