Intravenous immunoglobulin treatment in vasculitis and connective tissue disorders

Vasculitis syndromes and connective tissue disorders are heterogeneous and mostly rare multisystem disorders with various autoimmune mechanisms driving tissue inflammation and remodeling, ischemic and hemorrhagic tissue damage. While the nervous system can be affected by most of these diseases, the pathogenesis for neural involvement is often ambiguous and elusive for the clinician. Intravenous immunoglobulins (IVIG) have been used for the treatment of most of these disorders. However, a thorough review of the literature indicates that the role for IVIG has to be discussed for individual entities, has often only anecdotal evidence, and is particularly hard to define with respect to neurological manifestations. This review gathers the available evidence on the efficacy of IVIG in neurologically relevant rheumatic diseases, leading to recommendations for their clinical use.