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Figure 1. Granulomatous orbital pseudo-tumor in a patient with Wegener's granulomatosis (orbital-computed tomography scan).
(Figure omitted. See article PDF.)
Figure 2. Lung-cavitated nodule in a patient with Wegener's granulomatosis (thoracic computed tomography scan 2D reconstruction).
(Figure omitted. See article PDF.)
Figure 3. Granulomatous inflammation on a lung biopsy in a patient with Wegener's granulomatosis.
(Figure omitted. See article PDF.)
Figure 4. Florid necrotizing medium-sized vessel vasculitis with eosinophil infiltration in a patient with Churg-Strauss syndrome (muscle biopsy).
(Figure omitted. See article PDF.)
Figure 5. Scalp necrosis in a patient with giant cell (temporal) arteritis.
(Figure omitted. See article PDF.)
Figure 6. Algorithm for diagnosing a patient with suspected vasculitis. ANCA: Antineutrophil cytoplasmic autoantibodies; CSS: Churg-Strauss syndrome; HBV: Hepatitis B virus; HCV: Hepatitis C virus; MPA: Microscopic polyangiitis; PAN: Polyarteritis nodosa; WG: Wegener's granulomatosis.
(Figure omitted. See article PDF.)
The spectrum of granulomatous vasculitides encompasses different diseases and/or conditions, such as primary systemic vasculitides, but also those that are secondary to autoimmune and/or systemic inflammatory diseases, lymphoproliferative disorders or infections (Box 1) [1-3]. Since the lung is one of the most frequently affected organs in granulomatous vasculitides, a classification of pulmonary granulomatous vasculitides has been proposed (Box 2) [4]. However, no classification has been devised to include all the different entities characterized by, or associated with, granulomatous vasculitis in one or several organ(s) or system(s). Therefore, we attempted to list and briefly describe each of these diseases, with particular emphasis on primary small-vessel granulomatous vasculitides, especially Wegener's granulomatosis (WG) and Churg-Strauss syndrome (CSS), with which we have conducted several therapeutic studies on behalf of the French Vasculitis Study Group over the past several years.
Primary systemic vasculitides
Definition & classification
Systemic vasculitides are characterized by inflammation within the blood vessel walls, potentially resulting in vascular obstruction and ischemia of the affected tissues or organs. They can be classified according to the criteria established in 1990 by the American College of Rheumatology (ACR) [5-10] or the Chapel Hill Nomenclature [11], which appears to be more pragmatic as it distinguishes vasculitides according to the size and type of vessels affected, the target end organs and whether vessel-wall necrosis and/or granulomatous inflammatory infiltration exists. Moreover, the nomenclature also introduces the diagnostic value of the detection in sera of...