A case of systemic lupus erythematosus which developed the central nerve lesion that was possibly caused by cerebral vasculitis

Abstract

    A 27-year old female, who had been diagnosed as mixed connective tissue disease (MCTD) due to Raynaud’s phenomenon, swollen hands, polyarthritis, myositis, generalized erythema and elevation of anti-U1 RNP antibody titer, was successfully treated with oral prednisolone, presented exacerbations with fever, lymphadenopathy, polyarthralgia and myalgia. In March 2006, she was admitted to our hospital because of fever, headache and syncope. Physical examination on admission revealed facial erythema, complete right hemiplegia and disturbance of consciousness (Glasgow Coma Scale 11). Laboratory examinations showed decreased serum complement level and elevation of serum anti-DNA antibody level. Cerebrospinal fluid (CSF) interleukin (IL)-6 was also markedly elevated. MRI scans revealed high intensity areas along the left ventricle on diffusion weighted images and FLAIR images. Two courses of methylprednisolone pulse therapy followed by 100 mg/day of prednisolone for 4 weeks improved all of her symptoms and laboratory findings along with the disappearance of high intensity areas on MRI. The cerebral reversible lesions in this patient might possibly be caused by vasculitis due to SLE.