Neurological involvement in Wegener's granulomatosis

Abstract

Wegener's granulomatosis is a rare autoimmune disease associated with granulomatous inflammation and antineutrophil cytoplasmic antibody-associated vessel vasculitis. Most commonly, upper and lower respiratory tract and kidneys are involved: alveolar hemorrhage and necrotizing glomerulonephritis are hallmarks of full-blown disease; yet, a significant proportion of patients presents with peripheral nervous system (PNS) involvement due to vasculitis or with central nervous system (CNS) involvement due to infiltrating granulomatous manifestations (10-45%). The purpose of this review is to give a systematic overview on Wegener's granulomatosis manifestations of the PNS and CNS and to highlight new findings regarding manifestations, diagnosis and therapy. So far, peripheral neuropathy has been recognized as a severe and frequently occurring organ manifestation in Wegener's granulomatosis which requires early introduction of highly potent immunosuppression to induce remission. Recently, the impact of granulomatous manifestations originating from the ear-nose-throat tract and frequently affecting CNS structures has moved into the focus of attention, first, because they are not uncommon (occurring in 10-45% of patients) and, second, because they are associated with a high frequency of refractory disease courses. For both CNS and PNS involvement, rituximab and infliximab have emerged as potential treatment options for refractory disease. CNS and PNS manifestation in Wegener's granulomatosis are less frequent than classical manifestations such as lung and kidney involvement in Wegener's granulomatosis; however, neurological manifestations - not only peripheral neuropathy but also granulomatous manifestations affecting CNS structures - necessitate a fast diagnostic work-up and therapeutic intervention in order to prevent or reduce potential damage.