Central nervous system manifestations of rheumatologic diseases

To summarize the current literature on central nervous system manifestations of vasculitides and connective tissue diseases.

There have been advances in understanding the mechanisms behind the initiation and perpetuation of inflammatory processes in vasculitic neuropathy. Clinically relevant data have been obtained on the predictive criteria for a positive biopsy result in giant cell arteritis, the imaging characteristics of primary angiitis of the central nervous system, and Behçet disease, and the clinical and radiologic features of neuro-Behçet disease. There is more clarity about the central nervous system syndromes attributable to systemic lupus erythematosus and new insights into the central mechanisms involved in the manifestations of Sjögren syndrome and rheumatoid arthritis. Novel immunomodulatory agents, such as infliximab, have shown some benefit in rheumatoid vasculitis and Sjögren syndrome.

A better understanding of the clinical, radiographic, and serologic characteristics of various central nervous system complications of rheumatologic diseases has been gained in the past year. Recent advances in understanding the pathophysiology of peripheral nervous system complications and their treatment may affect the management of the central nervous system complications.