Association of severe and therapy-refractory systemic lupus erythematosus and neuromyelitis optica: a management challenge
Autor: | Guiomar Pinheiro, Inês Furtado, Ana Campar, Teresa Mendonça |
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Rok vydání: | 2018 |
Předmět: |
medicine.medical_specialty
Neurology Adolescent medicine.medical_treatment Brain Edema Unusual Association of Diseases/Symptoms Urinary incontinence Transverse myelitis 03 medical and health sciences Fatal Outcome 0302 clinical medicine Refractory medicine Humans Lupus Erythematosus Systemic Coma skin and connective tissue diseases 030203 arthritis & rheumatology Neuromyelitis optica business.industry Neuromyelitis Optica Brain Disease Management Plasmapheresis General Medicine medicine.disease Magnetic Resonance Imaging Dermatology Spinal Cord Respiratory failure Female medicine.symptom Respiratory Insufficiency Tomography X-Ray Computed business Immunosuppressive Agents 030217 neurology & neurosurgery |
Zdroj: | BMJ Case Rep |
ISSN: | 1757-790X |
Popis: | Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. Neuromyelitis optica (NMO) is an infrequent neuroinflammatory disorder, whose association with SLE remains rare. The authors report the case of an 18-year-old woman, with SLE refractory to multiple immunosuppressive therapies and novel biological agents. Under immunosuppressive therapy, the patient presented with transverse myelitis with contiguous spinal cord lesions and urinary incontinence, having been diagnosed with seropositive NMO, which was also proven to be refractory to common treatments. Partial recovery of the neurological deficits occurred with plasmapheresis, although not averting the brain involvement by NMO that ensued. The patient was listed nationally for allogeneic bone marrow transplant, but, unfortunately, no match was found and the patient died of severe cerebral NMO flare with coma due to brain swelling and consequent respiratory failure. Although the association of SLE and NMO is very rare, early diagnosis is crucial to facilitate initiation of immunosuppressive therapy. |
Databáze: | OpenAIRE |
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