36º Congresso Brasileiro de Reumatologia

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Title

LONGITUDINAL EXTENSIVE TRANSVERSE MYELITIS IN A PREGNANT WITH ADULT-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT

Background

Transverse myelitis is an uncommon neurological manifestation in systemic lupus erythematosus (SLE), affecting 1-2% of patients. Scarce reports describe the myelitis transverse in pregnant with long-standing SLE disease. However, to the best of our knowledge, there is no report of extensive transverse myelitis in pregnant patients with adult-onset SLE.

Case report

A 26-year-old Caucasian female with SLE for 18 months (cutaneous, joint involvement, homogeneous nuclear antinuclear factor with high title, anti-dsDNA and anti-Ro autoantibody positive). After the induction period, she had only used hydroxychloroquine sulfate and prednisone 0.5 mg/kg/day. Pregnancy discovered in February 2019 with discontinuation of the therapy by the patient. After one month, she had headache, paraplegia and loss of reflexes in lower limbs (motor sensory level in T5) and urinary retention. Laboratory, she had proteinuria and reduction of serum level of complement. Magnetic resonance imaging showed an extensive longitudinal myelitis (C5-T5), cerebrospinal fluid with increased cells, proteins and glucose reduction, negative infectious panel (viral - enterovirus, herpesvirus 6 and 7, adenovirus, cytomegalovirus, Epstein-Barr virus, varicella zoster, parvovirus B19 and herpes simplex I/II); negative to cultures: aerobic, anaerobic, fungal and mycobacterial). The SLE-related myelitis hypothesis was established and methylprednisolone pulse therapy (1 g/day, for 7 days) was introduced, followed by prednisone 1 mg/kg/day, and plasmapheresis (10 sessions). However, because of the refractoriness to these treatments, intravenous human immunoglobulin 2 g/kg and then rituximab 2 g were also introduced. Until the present date (June 2019), she has discharged without neurological improvement or other manifestations related to SLE, but only with improvement of the serum level of complement. No complications have been reported in relation to the pregnancy and the fetus.

Conclusion

Transverse myelitis is uncommon and with poor prognosis. To date, there are few reports of pregnant women with long-standing SLE disease. Herein, we reported a case of extensive transverse myelitis, particularly in the adult-onset SLE. Moreover, because of the refractoriness, the patient also received intravenous human immunoglobulin and rituximab, but with dubious prognosis.

Área

Systemic Lupus Erythematosus

Autores

Douglas Amaral Moreira, Sergio Luiz Oliveira Nunes, Lorena Elizabeth Betancourt, Felipe Freire da Silva, Diogo Souza Domiciano, Eduardo Ferreira Borba Neto, Samuel Katsuyuki Shinjo