We conducted an epidemiological study of systemic sclerosis in Japan using the records of patients who had been registered to receive public financial aid. A total of 10, 956 patients were registered as having systemic sclerosis in 1999. We statistically analyzed the data on the patients, including sex, age, major symptoms, and laboratory findings. We also made contingency tables in order to evaluate the correlations of the data.
Our study estimated that the male/female ratio was 1:7.3. The mean ages of male and female patients were 58.8 and 58.5 years old, respectively.
The major symptoms were as follows: Raynaud's phenomenon 92.4%, skin sclerosis 94.7%, dyspnea 29.9%, and dysphagia 32.2%. With respect to specific antibodies, antinuclear antibody was present in 92.2%, antitopoisomerase I antibody in 27.5%, anticentromere antibody in 37.7%, and antiribonucleoprotein antibody in 19.9%.
Making contingency tables, we could elucidate the association of antitopoisomerase I with lung fibrosis.

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Objective: To investigate the autoimmune responses against SS-A/B antigens by double immunodiffusion (DID) and western blotting (WB) in primary and secondary Sjögren's syndrome (SS).
Patients: Forty-nine patients with primary SS (PSS), 28 patients with secondary SS (SSS) and control group that couldn't be diagnosed as SS were included in this study.
Results: In DID analysis, Anti-SS-A antibody was detected in 69% of PSS and 86% of SSS, and anti-SS-B antibody was found in 22% of PSS and 39% of SSS. No significant difference could be demonstrated between PSS and SSS concerning anti-SS-A/B antibodies. Conversely, WB studies disclosed evidences that 18% of PSS and no SSS reacted only with the 52 kD protein, and there was significantly increased in PSS. Sera reacting with the 60 kD antigen were found in 37% of PSS, 71% of SSS, and 75% of SSS with SLE, 63% of SSS with RA. The ratio of SSS, and SSS with SLE were particularly significantly higher than PSS.
Conclusion: Our results revealed data that there are the difference of reactivity against SS-A/B antigens in WB between PSS and SSS.

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A 16-year-old girl was admitted to our hospital because of high fever, abdominal pain, and jaundice. Abnormal lymphocytes and hemophagocytic cells had infiltrated the bone marrow. Laboratory data revealed a severe type of hemophagocytic syndrome accompanied by an initial Epstein-Barr virus (EBV) infection. Persistent EBV infection was identified by polymerase chain reaction (PCR) detection of EBV-DNA in peripheral blood and bone marrow mononuclear cells. The limited efficacy of initial treatment with high-dose γ-globulin, plasmapheresis, and high-dose methylprednisolone prompted us to administration of T-COP-E (VP-16). Two courses of T-COP-E improved the patient's clinical symptoms and laboratory data; however, marked splenomegaly remained. In addition, fever and serum increase of lactate dehydrogenase (LDH) and cytokines such as γ-interferon recurred shortly after chemotherapy. On day 53 after diagnosis, the patient underwent laparoscopic splenectomy. The resected spleen weighted 420g and abnormal lymphocytes in the spleen were positive for CD 8 and negative for CD 56. In situ hybridization revealed EBV-encoded small RNAs (EBERs) in the abnormal lymphocytes. Clinical symptoms including high fever disappeared shortly after the splnectomy, and laboratory data returned to normal. Lymphocytosis after the splenectomy was not observed. We continued out patient monitoring of the case, and 16 months after diagnosis, EBV-DNA in peripheral blood mononuclear cells was not detected, even by PCR.

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