Over the last two decades, increasing interest has been focused on the association between autoimmune polyneuropathies and anti-neuronal autoantibodies in immune-mediated polyneuropathy. The possible appearance of these autoantibodies in systemic diseases that are not limited to the nervous system has not been fully addressed yet.
We evaluated 32 patients with systemic lupus erythematosus, 34 patients with hepatitis C virus-associated mixed IgM-k/IgG cryoglobulinemia, 19 with small vessel ANCA-associated vasculitis, and 20 patients with Sjögren's syndrome by means of an immunoenzyme method of anti-neuronal autoantibody detection.
As compared to normals, a significant increase (p < 0.001) in plasma titers of both IgM and IgG anti-GM1 ganglioside and IgM and IgG anti-sulfatide was observed in patients with systemic lupus erythematosus, mixed cryoglobulinemia and Sjög-ren's syndrome. Idiopathic systemic vasculitis patients were found to have significantly increased levels of anti-sulfatide IgG autoantibodies (p < 0.001). Clinical and electrophysiologic studies revealed that abnormal titers of anti-neuronal antibodies were associated with evidence of neuropathy in patients with systemic lupus erythematosus and ANCA-related vasculitis (p < 0.05) as well as in patients with mixed cryoglobulinemia and Sjögren's syndrome (p < 0.001).
Anti-GM1 and anti-sulfatide antibodies are frequently found in patients with small vessel ANCA-associated vasculitis and other multi-organ immune-mediated diseases. Upon detection of these antibodies, accurate neurologic examination should be carried out due to the significant association that can be found between these serologic abnormalities and the involvement of the peripheral nervous system as also detected by electrophysiologic studies. This study supports the unexpected possibility that anti-neuronal reactivity may be a direct trigger of neurologic injury in these systemic disorders.