Acute longitudinal myelitis as the first presentation in child with systemic lupus erythematosus concurrent with positive antiphospholipid antibody

Abstract

Systemic lupus erythematosus (SLE) is a multi-system auto-immune disorder that is characterized by widespread immune deregulation, formation of auto–antibodies, and immune complexes, resulting in inflammation and potential damage to variety of organs. 25-95% it is complicated by neurological or neuropsychiatric symptoms, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contain both central and peripheral nervous systems, which includes transverse myelitis. We report our experience of concurrent manifestation of transverse myelitis as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 7-year-old girl with SLE who first presented with features of TM. The patient developed ascending weakness starting from low extremities, experienced difficulty in voiding. An initial diagnosis of TM was made on the basis of clinical findings and MRI spine, which displayed T2 weighted high signal intensities at thoracic level. She partially respond to intravenous immunoglobulin therapy, and serological analysis revealed the presence of anti-dsDNA, anti nuclear antibody with decreased level of complements. The diagnosis was revised to acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. This was followed by oral prednisolone and cyclophosphamide pulse therapy. The crossreactivity of auto-antibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Systemic Lupus Erytheromyitis should consider as an etiology of transverse myelitis. Aggressive treatment may alter the course and lead to a better outcome.