The studies recounted in this review have demonstrated that cutaneous vasculitis is a frequent extraglandular manifestation of primary Sjögren's syndrome. Two histopathologic types of vasculitis have been detected. One type, a leukocytoclastic angiitis, is found in association with high-titer anti-Ro(SS-A) antibodies, rheumatoid factor, hypergammaglobulinemia, and hypocomplementemia. The second type, a mononuclear inflammatory vasculopathy, in sharp contrast, is found in association with low-titer Ro(SS-A) antibodies, normocomplementemia, and absence of hypergammaglobulinemia and rheumatoid factor. Both types of vasculitis are found in association with peripheral nervous system and CNS disease. The peripheral nervous system and CNS disease involves the entire neuroaxis and preliminary data indicate that a vasculopathy is the cause of the peripheral nervous system and CNS disease. Evoked sensory response testing, CSF analysis, and MRI have proved to be very valuable techniques in investigating these patients with Sjögren's syndrome. Preliminary data suggest that high doses of prednisone or immunosuppressive agents are effective in treating these patients.