A case of polymyositis with a significantly high level of KL-6 associated with pancreatic cancer.

A 53-year-old man was diagnosed with polymyositis (PM) in 1997 and treated with prednisolone. The subjective symptoms of pneumonitis were poor. However, the KL-6 values were elevated to 2230 IU/l in March 2001. Abdominal computer tomography findings revealed a pancreatic-tail tumor and multiple liver nodules, diagnosed as primary pancreatic adenocarcinoma with multiple liver metastasis. The stage of the pancreatic cancer was IV, and curative surgery of the tumor was not indicated. Chemotherapy and radiotherapy were administered for the liver metastasis. However, these therapies were ineffective against the tumors. The patient died on 12 September 2001. If a high level of KL-6 is found without the increasing activity of lung disease containing interstitial pneumonia in PM patients, examination for the internal malignancies including pancreatic cancer should be performed, although cases of PM with a significantly high level of KL-6 associated with pancreatic cancer are rare.

The efficacy of vitamin E against oxidative damage and autoantibody production in systemic lupus erythematosus: a preliminary study.

The hypothesis that reactive oxygen species (ROS) modification of DNA is involved in the development of autoantibodies in systemic lupus erythematosus (SLE) is supported by the enhanced reactivity of anti-DNA antibodies to ROS-denatured DNA. We studied the efficacy of vitamin E against both oxidative DNA damage and autoantibody production in SLE. Urinary 8-hydroxydeoxyguanosine (8-OHdG), an indicator of oxidative DNA damage, and the anti-double-stranded DNA (anti-ds DNA) antibody, a predictor of disease activity, were assayed twice, first during the season with the most intense sunlight and then later in the year. Twelve women among 36 outpatients received vitamin E (150 to 300 mg/day) together with prednisolone (PSL). No significant age or daily dose of PSL differences were evident between patient groups. Urinary 8-OHdG in the PSL with vitamin E group (15.0 +/- 10.2 ng/mg during the period of intense sunlight and 11.7 +/- 8.7 ng/mg during the remainder of the year) did not differ significantly from that in the PSL without vitamin E group (20.0 +/- 23.2 and 11.0 +/- 5.9 ng/mg, at these respective times), but the anti-ds DNA antibody titer in the PSL with vitamin E group (17.9 +/- 20.3 IU/l during the period of intense sunlight and 16.3 +/- 19.4 IU/l during the remainder of the year) was significantly lower than that in the PSL without vitamin E group for both sunlight-defined periods (66.3 +/- 76.8 and 55.8 +/- 59.0 IU/l, at these respective times; P < 0.05). The present study suggests that vitamin E can suppress autoantibody production via a mechanism independent of antioxidant activity.

A case of systemic lupus erythematosus expressing intractable thrombocytopenia remedied effectively by intermittent and continuous administrations of a small amount of immune globulin.

We describe a case where intermittent and continuous administrations of a small amount of immune globulin were effective in the treatment of refractory chronic immune thrombocytopenic purpura by systemic lupus erythematosus (SLE). Steroid pulse therapy and cyclophosphamide pulse therapy were considered for thrombopenia. However, the patient had compressed fracture of the lumbar vertebrae due to osteoporosis and right external malleolus ulcer with complications of infection. Therefore, high-dose intravenous immune globulin (IVIG) therapy (400 mg/kg daily for 5 consecutive days) was administered. Then, as a maintenance therapy, a small amount of 400 mg/kg for 1 day (400 mg/kg monthly) was given in an intermittent and continuous manner, which resulted in improvement of thrombocytopenia and reduction of the amount of steroid administered.

STAT6 deficiency inhibits tubulointerstitial fibrosis in obstructive nephropathy.

To elucidate the contribution of signal transducer and activator of transcription (STAT) 6 to the pathophysiology of chronic renal injury, STAT6-/- mice were subjected to unilateral ureteral ligation together with wild-type control mice. STAT6-/- kidneys had more apoptotic cells and a greater influx of F4/80-positive cells than wild-type kidneys following ureteral obstruction. There was a much larger alpha-smooth muscle actin-positive area in STAT6-/- kidneys than in wild-type kidneys after ureteral ligation. However, renal fibrosis, as quantified by Masson-Trichrome staining, was not significantly exaggerated in STAT6-/- kidneys compared with wild-type kidneys. The accumulation of collagen I was significantly less in STAT6-/- kidneys than in wild-type kidneys. These observations indicate that the STAT6 signal transduction pathway exerts a protective role on renal cell apoptosis in chronic obstructive uropathy. Our findings also suggest that the STAT6 pathway may have a promotive effect on renal fibrosis by activating collagen synthesis following ureteral obstruction.