Among a variety of central nervous system (CNS) manifestations of Behçet's disease, the most serious one is progressive dementia. However, there has been no effective treatment to prevent the progression of dementia. The present studies have examined the efficacy of low-dose weekly methotrexate therapy for the progressive dementia in Behçet's disease. Six patients with the progressive type of neuro-Behçet's disease were treated with low-dose weekly methotrexate (7.515mg/week). Neurological findings, psychological findings, brain MRI and IL-6 in cerebrospinal fluid were evaluated at the start and one year after the initation of the therapy. All the 6 patients were refractory to the conventional therapy for neuro-Behçet's disease. The progression of neuropsychological abnormalities such as dementia was not recognized after one year from the initiation of methotrexate therapy. Consistently, IL-6 levels in cerebrospinal fluid were significantly decreased after one year from the initiation of therapy. Six months after the discontinuation of low-dose weekly methotrexate, progression of neuropsychological abnormalities such as dementia was recognized, and IL-6 levels in cerebrospinal fluid were significantly increased. These findings may suggest the suppression of the ongoing inflammatory process in the intracranial lesions by methotrexate. These results suggest that low-dose weekly methotrexate therapy may be a new promising method for the treatment of the progressive type of neuro-Behçet's disease.

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A 19-year-old man was admitted to our hospital due to low grade fever and polyarthralgia with history of photosensitivity. Laboratory findings showed the elevated transaminases, leukocytopenia and thrombocytopenia. Examinations in regard to autoimmune diseases revealed the positive antinuclear antibody and positive LE test. The computed tomography (CT) showed the multiple low density spots in liver. CT guided liver biopsy specimens regarding to these low density spots showed the hepatic lesions with mild inflammatory cell infiltration and subtle piecemeal necrosis. The patient was diagnosed as systemic lupus erythematosus (SLE) with hepatic lesions based on the clinical symptoms and the laboratory data. Rheumatoid arthritis was excluded because of intact joints X-ray findings. Autoimmune hepatitis was also denied because of the fulfillment of definite diagnostic criteria of SLE and minimal histological changes of liver. Prednisolone therapy improved his clinical manifestations and normalized both the laboratory data of liver function and the immunological abnormalities in SLE, so the multiple spots in CT of liver also disappeared.

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A 31-year-old woman presented 1993 with fever, painful swelling of cartilaginous portions of the ears and the bridge of nose, polyarthralgia including costochondroral pains, and episcleritis. She has been taking propylthiouracil since 1991 when she was diagnosed as Graves' disease. Laboratory evaluations revealed an elevated erythrocyte sedimentation rate (ESR) of 133mm/h, a high CRP level of 13.2mg/dl and positive antinuclear antibodies and anti-type II collagen antibodies. Histopathological findings of the biopsy specimen from the auricular cartilage included chondrocyte degeneration, matrix destruction and inflammatory cell infiltration. She was diagnosed as RP and treatment with 30mg/day of prednisolone dramatically improved all symptoms and signs, accompanied by a fall in ESR, CRP and autoantibodies. When prednisolone was tapered to 5mg/day, a clinical relapse occurred. After discontinuation of propylthiouracil, she has been well without prednisolone.
Propylthiouracil-induced SLE-like syndrome or antineutrophil cytoplasmic antibodies (ANCA) related angitis has been reported previously. In addition, recent studies demonstrated that about 20% of sera from patients with relapsing polychondritis are P-ANCA positive. This is the first report suggesting a possible association between the development of relapsing polychondritis and propylthiouracil.

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The management of disseminated intravascular coagulation (DIC) in a 22-year-old female patient with antiphospholipid syndrome is reported. Gabexate mesilate was given by continuous drip infusion at 1.5g/day. No effect was seen, therefore Dalteparin sodium (DS) was administered by continuous drip infusion at 70U/kg/day. The DIC score improved gradually during the first 4 days to normalization by 10 days. However, convulsive seizure was developed. Computed tomographic scan of brain demonstrated brain abscess at It-basal ganglia. Continuous drainage was performed while administered continuous drip infusion of DS. Follow-up CT after operation showed reduction of low density area which means brain abscess. Finding in this case suggest that DS may play a role in the management of DIC accompanying intracranial infection.

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