Background: Transverse myelitis (TM) is an infrequent but serious manifestation of Systemic Lupus Erythematosus (SLE) with a reported incidence of 1–2 % and significant morbidity and mortality. TM complicating SLE has been variously reported to be associated with optic neuritis, anti-phospholipid antibodies, and antibodies to Ro/SSA and more recently with antibodies to aquaporin 4.
Neuromyelitis optica (NMO) is a relapsing, autoimmune, demyelinating condition characterized by TM and ON & at least two of the following: 1)Brain MRI, non-diagnostic for multiple sclerosis; 2) Spinal cord lesion extending over >=3 vertebral segments 3) NMO Ig-G antibody.
NMO spectrum disorders refer to inflammatory conditions of spinal cord & optic nerve, associated with anti-NMO Ig-G that do not fulfill the classic definition for NMO.
We present the largest study of TM in SLE including testing for anti-NMO antibodies in patients who had stored sera from the time of onset of neurologic symptoms. We also try to investigate the relationship of TM in lupus with NMO/NMO spectrum disorders.
Method: We conducted a retrospective study of all the patients satisfying 4 ACR criteria for SLE within the Michigan Lupus Cohort (MLC) & evaluated the clinical, laboratory & Imaging features in 23 patients with a history of TM. Available stored sera from the time of onset of TM were tested for IgG NMO antibodies.
Results: 2.7% (23/ 856) of patients in the MLC had TM of whom 9.5 % ( 2/ 23 ) had optic neuritis. Only 81.8% of all the pts demonstrated ANA positivity. At the time of the event, serologic markers for lupus activity were normal in 50 %of the patients. We did not observe the previously reported increased frequency of anti-phospholipid antibodies ( 8/23, 34.7%)%) or anti Ro/SSA antibodies (39.13%) compared to the general lupus population.
Sensory involvement was the commonest manifestation (93.7%) followed by motor (62.5%) and bladder involvement (50%). Paraplegia occurred in 21.05% & 17.6% were catheter dependent. All the pts received steroids & 81.25% received Cytoxan. Overall recovery was good mostly due to aggressive immunotherapy.
2/23 (9.5) % of pts with TM had optic neuritis and fulfilled criteria for NMO. 30% of pts with available serum samples demonstrated NMO Ig-G . Overall 36.3% were categorized as NMO disease (inclusive of NMO and NMO spectrum). Contrary to our expectation, none of the pts in NMO category had a severe involvement such as paraplegia or catheter dependency. There were no statistically significant differences between the two groups in imaging or serological characteristics. Recurrences were seen in 75%of NMO group vs none in non-NMO group (P<0.024).
Conclusion: In a large prospective cohort, TM occurred in 2.7% of patients & was not associated with antibodies to phospholipids or Ro/SSA. Majority of patients recovered after aggressive immunosuppression. There were no significant differences between the NMO and NMO groups. Recurrences of TM, but not severity of onset, were associated with NMO spectrum disorders.
Disclosure: M. Venkatram, None; P. C. Cagnoli, None; D. Gomez-Hassan, None; W. J. McCune, None.