Diagnosis and therapeutic options for peripheral vasculitic neuropathy

Abstract

Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types.

Keywords: autoantibodies; polyneuropathy; rheumatic diseases; treatment; vasculitic neuropathy.

Figure 1.

Immunohistochemistry of peripheral nerve vasculitis. Epineural vessel with extensive lymphocyte infiltration and subtotal stenosis. Staining of lymphocytes with anti-LCA (lymphocyte common antigen) antibody. (Courtesy of J. Weis, Aachen.)