A case of neuromyelitis optica spectrum disorder with coexisting systemic lupus erythematosus

Abstract

Neuromyelitis Optica or Devic disease is changed to Neuromyelitis Optica spectrum disorder to include more diverse neurological and autoimmune manifestations. This is a severe relapsing autoimmune demyelinating disorder commonly affecting the optic nerve and spinal cord. It has been reported as either the first manifestation of SLE or as a coexisting condition with other autoimmune disorders commonly included but not limited to SLE and SS. We discussed a case of a 49-year-old female patient who was initially presented with a left-sided weakness that rapidly progressed to quadriparesis and bladder dysfunction within a few days. She had positive autoimmune serology tests for SLE posing a diagnostic challenge as SLE is associated with neurological manifestations. Due to a lack of definitive diagnostic criteria for SLE, presence of AQP-4 antibodies in CSF, and evidence of longitudinal extensive transverse myelitis in MRI cervical spine, we conclude that she has Neuromyelitis Optica spectrum disorder with probable SLE. It is possible that she may develop more signs and symptoms of SLE with time and will need close follow up. Timely diagnosis and prompt treatment are vital to decrease morbidity and mortality, as done in our case. The patient was started on high-dose steroids with significant improvement in her symptoms. These patients may need early treatment with plasmapheresis and long-term follow-up with immunotherapy to prevent relapse. There are few case reports in the literature, and more information is needed to understand and better diagnose NMO with coexisting SLE.

Neuromyelitis optica spectrum disorder co‐existing with antiphospholipid syndrome: A case report

“…2,24,25 About 20%-30% of NMOSD cases have a variety of symptoms that resemble those of other autoimmune disorders. 12,23 Other autoimmune diseases that have been reported to coexist with NMOSD include myasthenia gravis, thyroid disease, ankylosing spondylitis, pernicious anemia, ulcerative colitis, systemic lupus erythematosus, and thrombotic thrombocytopenic purpura. [26][27][28] The development of autoantibodies as a result of complement pathways, activation of interleukins, particularly IL-6, and molecular mimicry of autoreactive B cells represent the hallmarks of the co-occurrence of disease.…”

“…The course of NMOSD is progressive over weeks to years as compared to other demyelinating diseases and can be monophasic or relapsing–remitting patterns 4,7 . In 2015, the international revised consensus diagnostic criteria were recommended for the diagnosis of NMOSD 3,6,23 . These include any of the core clinical characteristics; optic neuritis, longitudinally extensive transverse myelitis of ≥3 spine segments, area postrema syndrome, symptomatic narcolepsy, or acute brainstem syndrome without cognitive impairment and positive serum AQP‐4 autoantibodies 6,7,11 .…”

Combination of Neuromyelitis Optica Spectrum Disorder with Myasthenia Gravis, Systemic Lupus Erythematosus and Hashimoto's Thyroiditis: A Case of Multiple Autoimmune Syndrome

“…5 Sjogren's syndrome and SLE are the most frequently reported comorbid systemic autoimmune diseases and MG is the most reported neurological disease accompanying NMOSD. [5][6][7] The pathological mechanism of the SLE and MG association is not clearly known. SLE may develop after thymectomy in MG patients, but there was no thymectomy history in our case since there was no thymoma.…”

Systemic Lupus Erythematous and Neuromyelitis Optica Causing Hypercapnic Respiratory Failure

“…NMO-IgG antibodies bind to the water channel aquaporin 4 (AQP4) and are specific to patients with NMO [ 2 ]. AQP4 is the most expressed channel in the brain, spinal cord, and optic nerves [ 4 ].…”

“…NMO is more common in women, has a prevalence of 0.5 to 4.4 per 100 000 people, and the age of onset is typically between 35 and 42 years [ 2 ]. NMO is associated with other auto-immune diseases [ 1 ] and can occur as a primary autoimmune disorder in patients with existing systemic lupus erythematosus (SLE) [ 3 ].…”