SLE-Related Longitudinal Myelitis with Favorable Outcome

Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ involvement, defined by its clinical features and the presence in the blood of antibodies against one or more components of cell nuclei 1 .Neurological and psychiatric complications of SLE include seizures, psychosis, cerebrovascular disease, cognitive dysfunction 2 and headache 3 , described in up to 75% of patients 4,5 .In general terms, any part of the nervous system 6,7 may be involved, however, central nervous system (CNS) complications are of relevance because can significantly affect prognosis and mortality.Different pathogenetic mechanisms have been claimed to play a relevant role in CNS complications of SLE such as vasculitis, antibodies against brain tissues, deposition of immunocomplex, overproduction of cytokines, thrombosis, and hemorrhage 8 .Myelopathy is one of 19 possible syndromes described by the American College of Rheumatology 9 , with transverse myelitis (TM) being a rare but serious neurologic complication.It presents as a rapidly progressive motor, sensory and autonomic dysfunction [10][11][12] , with uncertain pathophysiology, whose prevalence seems to be in the region of 1-2% among SLE patients 10,[13][14][15][16] .Since the initial description of the first nine cases in 1968 17 , SLE-related TM is now a well-known clinical entity and a number of authors pointed out the close relationships with antiphospholipid antibodies (aPL) syndrome.In fact, from 55% to 64% of SLE-related TM present high levels of aPL, which is higher than that reported in the general SLE population (30-50%) [18][19] .Different authors, therefore, speculated that the pathogenesis of TM may be related to a vascular thrombosis or to a direct interaction between aPL and membrane phospholipids at the spinal cord level 14 .A rare variant of the SLE-related myelopathy is longitudinal myelitis (LM), which is defined by the continuous involvement of more than four spinal cord segments [20][21][22][23] .In fact, one to four spinal segments are generally involved in SLE-related TM 20,23 .Longitudinal myelitis needs to be promptly diagnosed and recognized because it is characterized by an acute catastrophic onset and an unfavorable prognosis, and a closer association with aPL syndrome has been suggested when compared to TM 20 .Here, we describe a patient presenting with LM and peripheral neuropathy as early manifestations of SLE, with negative aPL and a favorable outcome.Clinical, electrophysiological and radiological features at the onset and during the follow-up are discussed. Laboratory work-upRoutine blood tests including inflammation indexes, electrolytes, renal, liver and thyroid function, creatine kinase, glucose, vitamin B12, folic acid, hemoglobin level, platelet count, white blood cell count, prothrombin time and activated partial prothrombin time were all within the normal range.Rheumatologic blood tests showed highly positive antinuclear antibodies (4.91 IU/mL, normal range 0-1 IU/mL) speckled pattern; high levels of anti-dsDNA antibodies (69.4 IU/mL, normal range 0-30 IU/mL); dRVTT screening 38.7 sec (normal

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