Diagnostic Criteria Released for Neuromyelitis Optica Spectrum Disorders

Dr. Kolfenbach

Dr. Kolfenbach also emphasized the patients with autoimmune disease are at risk for a second, or even a third, concurrent autoimmune illness. In a prior study of patients with rheumatologic disease and CNS inflammatory manifestations, he and his colleagues found a high percentage of patients with both rheumatic disease and concurrent NMOSD.³ “There tends, in NMO, to be higher rates of concurrent autoimmune disease,” he says, adding that neurologic involvement may present before, after or concurrently with a diagnosis of rheumatic disease. “We may be following a patient with lupus or Sjögren’s disease who then is unlucky enough to develop this second autoimmune disease, so rheumatologists are becoming well versed in this condition as well,” he says.

Message to Rheumatologists

“Our patients are at unique risk for this special neurologic disease,” emphasizes Dr. Kolfenbach. “It is not a condition that only neurologists think about, but it is something that rheumatologists need to be aware of in our patients because they are at higher risk.”

Dr. Kolfenbach emphasizes that rheumatologists have a role in assessing patients with potential NMO because these patients may have an unidentified rheumatic condition. “One of the red flag conditions mentioned in this paper, sarcoidosis, is a condition commonly managed by rheumatologists,” he says. “Also, many of our diseases such as lupus, Sjögren’s syndrome, Behçet’s disease, anti-phospholipid antibody syndrome and others can be associated with CNS involvement.”

As such, he encourages “neurologists to consider a concurrent disease in these patients and refer them to a rheumatologist for a full evaluation.”

Dr. Wingerchuk agrees, stating that one of the most common scenarios is neurologists referring a patient to rheumatologists to help them find a rheumatologic disease in a patient who has some positive autoimmune antibodies. “This is the recognition that sometimes you see a cluster of antibodies occur in these patients with NMO and checking out these antibodies is reasonable.”

Mary Beth Nierengarten is a writer, editor and journalist with more than 20 years of experience writing about clinical medicine and health-related issues, and lives in Minneapolis.

References

1. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177–189.
2. Wingerchuk DM, Lennon VA, Pittock SJ, et al. Revised Diagnostic Criteria for Neuromy-Elitis Optica. Neurology. 2006 May 23;66(10):1485–1489.
3. Kolfenbach JR, Horner BJ, Ferucci ED, et al. Neuromyelitis optica spectrum disorder in patients with connective tissue disease and myelitis. Arthritis Care Res (Hoboken). 2011 Aug;63(8):1203–1208.