Neurological involvement in Wegener's granulomatosis

Purpose of review: 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. Recent findings: 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. Summary: 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.