Devic's neuromyelitis optica: a primary autoimmune disease?

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Abstract
Sir, We read with interest the letter of Giorgi et al.[1], concerning the co‐existence of Devic's neuromyelitis optica (DNO) and systemic lupus erythematosus (SLE). The authors suggested that DNO was associated with SLE and cited reports of 21 patients with such an association from 1973 to 1997. Although many cases of DNO have been described in SLE, there have been several patients in which DNO appears to be isolated, or perhaps primary. DNO has traditionally been considered to be a subgroup of multiple sclerosis (MS) with optic neuritis (invariably causing residual visual impairment) and transverse myelitis, with no other clinical features to suggest MS. This group of isolated DNO is of particular interest and deserves special mention. We propose that isolated DNO is an autoimmune disease in its own right. The co‐existence of two autoimmune diseases is well known. In this context alone, one might expect SLE and DNO to occur together. A review of reported cases of DNO reveals many features of an autoimmune disease, including a striking female to male predominance (24:1), an association with pregnancy [2] and livedo reticularis [3]. Cuadrado et al.[4] have described a subgroup of patients with the anti‐phospholipid syndrome with neurological symptoms and magnetic resonance imaging (MRI) findings compatible with MS. In this series of 23 patients, five patients had transverse myelitis and six optic neuritis.