Clinical, Radiologic, and Therapeutic Analysis of 14 Patients with Transverse Myelitis Associated with Antiphospholipid Syndrome: Report of 4 Cases and Review of the Literature

Objective

To analyze the clinical, radiologic, therapeutic, and developmental characteristics of transverse myelitis (TM) and antiphospholipid syndrome (APS).

Methods

We systematically searched English, Spanish, and Japanese articles on the subjects of TM and APS that had English abstracts in PubMed from 1966 to 2010. In addition, we reported on 4 patients with APS and TM that were treated by the Rheumatology Division of the Hospital das Clínicas da Faculdade de Medicina da Universidade in São Paulo, Brazil.

Results

Fourteen cases of patients with APS and TM were reviewed. The age of these patients ranged from 8 to 83, and cases of TM predominantly occurred among patients with primary APS (9/14). The clinical presentation of TM was characterized by effects on the thoracic spinal cord (9/14) that were associated with sphincter disturbances (8/14). The onset of symptoms was sudden in 8/14 cases, and the symptoms of myelitis were recurring in 3 cases. One case resulted in death. In most cases, treatment was based on corticosteroid pulse therapy (12/14), but some patients were treated with pulse cyclophosphamide (5/14), plasmapheresis (3/14), or rituximab (1/14). Generally, the therapeutic response was satisfactory, and complete improvement was seen in 9/14 patients.

Conclusion

In light of the severe clinical presentation of TM and its morbidity and mortality, early diagnosis and aggressive treatment are vital for therapeutic success. We can verify the excellent therapeutic response, as we saw a complete improvement in 64% of patients.

Keywords: antiphospholipid syndrome, transverse myelitis, systemic lupus erythematosus