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To identify the presence of neuromyelitis optica spectrum disorders (NMOSD) in patients with acute myelitis and suspected connective tissue disease (CTD), and to discuss the utility of this distinction in establishing a diagnostic and therapeutic plan.

Seventeen patients with myelitis were identified from a single university-based rheumatology clinic and prospectively followed. Clinical data and serologic profile were used to determine the presence of neuromyelitis optica (NMO) or NMOSD according to established criteria. Disease course and response to treatment were recorded.

Eight of 15 (53%) patients diagnosed with a CTD and acute myelitis met criteria for NMOSD. Additionally, two patients had full-spectrum NMO without evidence of co-existent CTD. Optic neuritis was more commonly seen in Sjogren’s syndrome (SS) than in systemic lupus erythematosus (SLE) (83% vs. 0%; p=0.02), as was the serum NMO-IgG antibody (67% vs. 0%; p=0.06). Full-spectrum NMO was diagnosed in 4/6 patients with primary SS, 1/4 with Sjogren’s-associated overlap syndrome, and 0/5 with SLE alone. Among patients with follow-up data (N= 15), all six with NMO-IgG positivity suffered relapse compared to 3/9 patients that were NMO-IgG negative (p = 0.03).

NMOSD was common in our patients with CTD and myelitis. Full-spectrum NMO and presence of the NMO-IgG antibody was more common in patients with SS than SLE. The NMO-IgG antibody was associated with disease relapse. Recognition of overlap NMO/NMOSD requires a multi-disciplinary approach, and will help clinicians formulate an accurate diagnostic and therapeutic plan. ? 2011 by the American College of Rheumatology.