A case of systemic lupus erythematosus associated with longitudinal extensive transverse myelitis, cerebral neutrophilic vasculitis, and cerebritis
Modjinou, D.; Gurin, L.; Chhabra, A.; Mikolaenko, I.; Lydon, E.; Smiles, S.
Bulletin of the Hospital for Joint Disease 72(4): 294-300
2014
ISSN/ISBN: 2328-5273 PMID: 25986355 Document Number: 672320
Systemic Lupus erythematosus (SLE) is an autoimmune disease with multiple clinical presentations and manifestations. Here, we report an intriguing case of a 30-year-old female with full-blown SLE, associated with longitudinal extensive transverse myelitis (LETM) on Magnetic Resonance Imaging (MRI) manifested by lower extremity weakness, neurogenic bladder and bowel, and central nervous system (CNS) lupus clinically manifested by changes in mood and behavior as well as neutrophilic vasculitis and cerebritis on pathology. LETM is a rare complication of SLE; however, what makes this case even more intriguing is that it additionally had cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations subsequently followed by a rash as well as intractable fevers of unknown etiology- much later attributed to her lupus. Although she had a most concerning and dramatic presentation, she, so far, had responded very well to therapy including pulse dose steroids, plasmapheresis, intravenous immunoglobulins (IVIG), cyclophosphamide, and related medications.