Table 1.

Revised criteria for a disease to be considered autoimmune: neuromyelitis optica and multiple sclerosis

Criteria for Autoimmune Disease7	NMO	MS
Immune response to a precise autoantigen in all patients	aquaporin 422, 23	Multiple antigens have been described, not present in all patients12, 13, 17, 18, 19, 20
Lesion reproducibility after administration of autoantibody or T cells	Exacerbation of EAE model after adoptive transfer of neuromyelitis optica Abs27
Animal: induction of lesion by antigen immunization		EAE model: induced by myelin oligodendrocyte glycoprotein, proteolipid protein, myelin basic protein28 and reactivated CD4+ T cells29
Autoantibody or T cell isolation form lesion or serum	aquaporin 4 antibodies27, 30
Autoantibody titers or T-cell levels associated with disease activity	Higher antibody titers during relapse than during remission31
Autoimmune disorders or autoantigens associated with the disease	Sjogren syndrome, SLE32	No association in population-based cohort studies33
Immune absorption with purified autoantigen abrogates pathogenic autoantibody or T cell
Reduction of autoantibody or T cell associated with clinical improvement	Plasma exchange34	Plasma exchange35, 36

Abbreviations: EAE, experimental autoimmune encephalomyelitis; MBP, myelin basic protein; PLP, proteolipid protein.

Adapted from Paul WE, Schwartz RS, Datta SK. Autoimmunity and autoimmune diseases. In Fundamental immunology. New York: Raven Press; 1989. p. 819-66; and Rodriguez M, Warrington AE, Pease LR. Invited article: human natural autoantibodies in the treatment of neurologic disease. Neurology 2009;72(14):1269–76.