A rare case of neuromyelitis optica spectrum disorder (NMOSD) with systemic sclerosis was recently reported in Italy. The case report, published in Neurological Sciences, also included a literature review, which found just 7 published articles on systemic sclerosis coexisting with NMOSD between 2004 and 2020.

“Accurate information about the prevalence of immune-mediated disorders in NMOSD patients is not available,” the authors wrote. “Large cohorts of NMOSD patients with a complete assessment of systemic autoimmunity are needed to evaluate the prevalence of autoimmune comorbidities and could help to compare demographic features, attack types, treatment response, and prognosis, between NMOSD patients with and without such comorbidities to select the best treatment option.”

All known combined cases have occurred in women aged between 30 and 65 years. Furthermore, in all cases, the symptoms of systemic sclerosis began years before the NMOSD, and in all but one case, aquaporin-4 (AQP4)-IgG antibodies were detected in serum.

Vascular dysfunction is common in the pathogenesis of systemic sclerosis, and it can damage the blood-brain barrier and trigger an autoimmune response due to AQP4 channel protein exposure during the presentation of antigens and T-cell activation.


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The central treatment for both NMOSD and systemic sclerosis is immunosuppressive therapy, and apheresis is an option for relapse therapy in the nearly half of cases that do not respond to steroid treatment. The present case was treated with intravenous methylprednisolone followed by apheresis treatment, which led to a poor clinical response despite improvements in her spinal magnetic resonance imaging (MRI) exam.

Cyclophosphamide, rituximab, and tocilizumab are preferred treatments for patients with systemic sclerosis with lung dysfunction, and both rituximab and tocilizumab have been shown to reduce the frequency of relapses and improve disability in NMO patients. Therefore, the authors recommend close collaboration between rheumatologists and neurologists to optimize the therapeutic approach for these patients.

Reference

Bollo L, Santoro C, Libro G, et al. Neuromyelitis optica spectrum disorders associated with systemic sclerosis: a case report and literature review. Neurol Sci. Published online March 15, 2022. doi: 10.1007/s10072-022-05984-2