Revista de vasculitis

Primary Angiitis of the Central Nervous System (PACNS) is a rare disease that causes inflammation and destruction of the vessels within the CNS without evidence of a systemic vasculitic process. There is little known about the etiology of this disease entity but there are a limited number of case reports of CNS vasculitis being associated with biologic therapies in particular TNF antagonists. TNF antagonists have been in use for over 20 years for the treatment of numerous immunologic diseases. It is estimated that over 3 million people have been exposed to these drugs. TNF antagonists have rarely been associated with the development of vasculitis. Here we present a case of a biopsy proven CNS vascultis developing in a patient with rheumatoid arthritis while taking etanercept with a systematic review of the literature regarding TNF antagonists and subsequent development of CNS vasculitis.

Giant-cell arteritis (GCA) is a large-vessel granulomatous vasculitis in which aging, gender and genetics likely play a significant role. The association with polymorphisms in the major histocompatibility complex suggests that GCA may be an antigen-driven disease. Immunopathology studies performed with temporal artery biopsies from patients with GCA have generated relevant clues regarding to pathogenesis by indicating participation of Th1 and Th17-mediated pathways, a prominent role for macrophages in tissue injury, and the relevance of vascular response to inflammation. Vascular wall elements, especially endothelial cells and vascular smooth muscle cells are not passive bystanders. Through expression of chemokines and adhesion molecules vascular cells contribute to the continuous recruitment of inflammatory cells that are able to enter the artery wall through newly formed neovessels. Inflammatory cell products, as well as vascular injury, trigger a vascular remodelling process. This eventually leads to the development of intimal hyperplasia and vascular lumen obliteration, source of ischemic complications.