Neonatal lupus syndrome is a passively acquired autoimmune syndrome in which pathogenic autoantibodies (anti-SSA/Ro, anti-SSB/La, and both, or rarely anti-U₁RNP antibodies) are transmitted from a mother to her fetus through the placenta. The major clinical manifestations in the infants are cardiac (congenital heart block), dermatologic (skin lesion), hepatic (elevated hepatic enzymes), and hematologic (cytopenia). Congenital complete heart block (CCHB) is irreversible, while noncardiac manifestations are transient, resolving by one-year-old of age without specific treatments. Two prospective studies show that the prevalence of CCHB in children from a woman previously known to have anti-SSA/Ro antibodies is approximately 2%. However, when the previous pregnancy is complicated by CCHB and skin lesion, the recurrence rates of these symptoms go much higher to 10.5% and 26%, respectively, in the subsequent pregnancy.

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  IFNγ plays an important role to induce several functional molecules on salivary epithelial cells, including class II MHC, Fas and CD40 in salivary glands from patients with Sjögren’s syndrome (SS). IFNγ also contributes to the development of lymphocytic infiltrates by inducing T cell attracting chemokines in SS salivary epithelial cells, such as IP-10 (CXCL10), Mig (CXCL9), and I-TAC (CXCL11). IFNγ dysregulation in SS salivary gland may attribute to the decreased production of TGFβ from salivary epithelial cells in some patients. Expression of Fas and CD40 was significantly higher in SS salivary epithelial cells than in normal cells after IFNγ stimulation. Although neither anti-Fas (CH11) nor anti-CD40 mAb alone could induce typical apoptosis, the two together and preincubation with IFNγ efficiently induced apoptosis in SS salivary epithelial cells. This apoptosis was almost completely blocked by neutralizing anti-Fas mAb (ZB4). c-FLIP, an important inhibitory molecule in the Fas death pathway, was strongly expressed in SS salivary epithelial cells, but its expression was downregulated, at the protein level, by anti-CD40 mAb. CD40 signals promote Fas-dependent death of SS salivary epithelial cells by downregulating c-FLIP expression. The presence of c-FLIP in these cells may explain their resistance to undergo apoptosis in response to either anti-Fas or anti-CD40 mAb, despite their surface expression of both proteins. These findings suggest that SS salivary epithelial cell death requires the cooperation of Fas and CD40.

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  The activation of naïve T cells requires two signals from the antigen presenting cells (APC). Firstly, an antigen specific signal which is triggered by the binding of the T cell receptor (TCR) to the peptide-MHC complex, and secondly, antigen nonspecific signals initiated through a set of co-signalling receptors. Co-signalling molecules are cell-surface glycoproteins that play essential roles for the communication of a T cell with virtually all other host cells by modulating and fine-tuning TCR signals. On the basis of their functional outcome, co-signalling molecules can be divided into co-stimulators and co-inhibitors, which promote or suppress T-cell activation, respectively. By expression at the appropriate time and location, co-signalling molecules positively and negatively control the priming, growth, differentiation and functional maturation of a T-cell response. In this article, I overview property of co-signaling molecules in the CD28- and TNFR family and discuss their potential functional relationships with each other. In addition, role of these co-signalling molecules in various diseases, such as autoimmune diseases, graft rejection, allergy, inflammatory bowel disease, and cancer, and the therapeutic potential of targeting these molecules to enhance or curtail an ongoing immune response in these diseases are discussed.

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  Aim : The aim of the study was to describe the frequency of anti-phosphatidylserine-prothorombin antibody(aPS/PTAb) in patients with recurrent abortion and preeclampsia, and to study the relationship between the presence of aPS/PTAb and clinical finding. Eighty six cases of recurrent abortion and 82 cases of preeclampsia were studied. A aPS/PTAb was measured by an enzyme linked immunosorbent assay(ELISA).
  Results : In patients with recurrent abortion, 3 out of 86 cases(3.4%) were positive in IgG antibody measurements and 5 out of 86(5.8%) were positive in IgM antibody measurements. In patients with preeclampsia, 2 out of 82 cases(2.5%) were positive in IgG antibody measurements and 13 out of 82(16%) were positive in IgM antibody measurements. The positive rates of aPS/PTAb in severe hypertension-positive cases is greater than in hypertension-negative cases(p=0.045). The positive rates of aPS/PTAb is higher tendency with in severe type than in mild type(p=0.117). The positive rates of aPS/PTAb is higher tendency with proteinuria and/or hypertension than without proteinuria(p=0.098) or hypertension(p=0.096).
  Conclusion : We found that aPS/PTAb appears in some cases of patients with recurrent abortion and preeclampsia. Our data suggest that aPS/PTAb might be a risk factor in patients with recurrent abortion, and may relate to clinical finding in preeclampsia.

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  Because the prognosis of systemic lupus erythematosus (SLE) has been much improved by recent progress in the treatment of this disease, improvement of quality of life (QOL) will be required more and more. However, QOL in SLE has not been well studied in comparison to that in rheumatoid arthritis. Fifty-four patients with systemic lupus erythematosus were asked about healthy feeling, acceptance of disease and the extent of satisfaction with their life. The percentage of patients who gave affirmative answers to healthy feeling, acceptance, and satisfaction was 64, 87, and 50, respectively. These three parameters were correlated with the following factors; 1. physical activity, especially that for daily living, 2. understanding in the family and workplace, and 3. depression and anxiety, whereas acceptance was not correlated with disease activity. Due to having a chronic disease, there are depression and anxiety derived from loss of existence in the family or workplace in their minds. In order to resolve these issues, education and explanation about the disease is needed for the family and society as well as for the patients. Although compliance of the patients in answering the questionnaire was easily obtained, the reliability and reproducibility, and the relationship between the items and the low-ranking factors should be investigated using a larger number of patients.

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  Here we described an autopsy case of intestinal Behçet’s disease with sacroiliitis associated with myelodysplastic syndrome (RAEB-t, 8+). Over twenty cases of Behçet’s disease associated with myelodysplastic syndrome have been reported in preliterature so far. The majority of them are incomplete type of Behçet’s disease having intestinal ulceration. Of those, trisomy 8 is the most common chromosomal abnormality. We reviewed similar cases reported and investigated the association of intestinal Behçet’s disease with trisomy 8. The association of sacroiliitis with Behçet’s disease is also studied.

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