Myelitis in systemic lupus erythematosus frequently manifests as longitudinal and sometimes occurs at low disease activity.

Background Transverse myelitis (TM) is a relatively infrequent but severe complication in systemic lupus erythematosus (SLE). Owing to its rarity and unfavorable outcome, we investigated its general features on MRI and incidence in the context of lupus activity, to facilitate early recognition and treatment. Methods We report a case of a young man with clinically inactive lupus nephritis but who presented with a sudden attack of myelitis. We performed systematic literature search in Medline to study the clinical features of SLE-related TM. Results From 1960 to April 2013, a total of 72 articles containing 194 cases of lupus myelitis were found. Among acquired articles, 93 patients fulfilled the inclusion criteria. The majority of the cases (88.8%) were female. Longitudinal myelitis was the predominant imaging finding on MRI (71.4%, 45/63). Nearly two-thirds (61/94) of lupus myelitis occurred in association with active lupus, and one-third (33/94) occurred in low disease activity. Conclusions Upon literature review, we found myelitis in SLE more frequently manifested as longitudinal on MRI. Although lupus myelitis often presented at an active phase of SLE, one-third of events happened in the presence of low disease activity. Early initiation of effective immunosuppressive therapy facilitated recovery.