Principales vascularites nécrosantes systémiques

Churg–Strauss Syndrome

“…CSS is defined by the combined presence of at least four of the six criteria with a sensitivity of 85% and a specificity of 99.7%. However, it should be realized that in rare cases, CSS vasculitis may precede the onset of asthma and blood eosinophilia (Fries et al 1990;Masi et al 1990;Abril et al 2003;Guillevin and Pagnoux 2006).…”

“…Many provoking factors have been suggested, such as hepatitis B vaccination, allergy desensitization therapy, corticosteroid therapy weaning, allergic bronchopulmonary candidiasis, parasitic infections such as ascaridiosis (Val-Bernal et al 2003;Guillevin and Pagnoux 2006;Lhote 2007), inhalation of particles (diesel fumes, flour or cereal dust, and pigeon droppings) (Abril et al 2003), and exposure to various medications: propylthiouracile, carbamazepine (Imai et al 1989), quinine (Mathur et al 1990) macrolides (Dietz et al 1998;Roux and Fabre 2004), and leukotriene receptor antagonists (Wechsler et al 2000;Guilpain et al 2002;Val-Bernal et al 2003;Beduneau et al 2005); • A direct role of circulating antineutrophil cytoplasmic antibodies (ANCA), immune complexes with a dominating role of IgE and cytokines (Noth et al 2003;Guillevin et al 2004); • A direct toxic effect of eosinophils, possibly explaining the ANCA-negative CSS cases Sinico and Bottero 2009).…”

“…All the arteries can be affected, but the pulmonary vessels are most often involved. The pathological confirmation is based on the presence of three characteristic histological features which coexist occasionally at the same biopsy site (Cottin and Cordier 2003;Guillevin and Pagnoux 2006;Khasnis and Langford 2009): 1. A fibrinoid necrosis of the vessel media characterizing the acute phase of vasculitis with an intraparietal and perivascular infiltration by varied polynuclear cells and rich in eosinophils.…”

“…The most prominent organs involved are the peripheral nervous system, the skin, and the cardiovascular system. The GIT, the brain, the eyes, and the kidneys are less affected (Chumbley et al 1977;Lanham et al 1984;Guillevin et al 1988;Abu-Shakra et al 1994;Haas et al 2001;Guillevin and Pagnoux 2006). Occasionally, isolated involvement of the respiratory system, the GIT, or the heart can be observed, making the diagnosis difficult (Churg et al 1995;Noth et al 2003).…”

“…Eosinophils may also be found in bronchoalveolar lavage fluid. Peripheral blood eosinophilia may regress spontaneously and quickly when CSS is treated with corticosteroids but reappears in the event of a relapse (Guillevin and Pagnoux 2006).…”