Genetic analysis of systemic lupus erythematosus: association with a T cell receptor restriction fragment length polymorphism in Japanese patients.

Many studies have suggested the involvement of multiple genetic loci in the development of systemic lupus erythematosus (SLE). We have analyzed the correlation between various genetic markers and the susceptibility to SLE. In this study, the association of SLE and restriction fragment length polymorphism (RFLP) of T cell receptor gene was evaluated. The cDNA for constant regions of alpha, beta and gamma chain were used as probes and RFLPs were analyzed after digestion with Eco RI, Bam HI, Pst I, Pvu II, Hind III and Bgl II. Among them, polymorphisms were detected using Bgl II- and Hind III-digested DNA and C beta as a probe. Association with SLE in Japanese patients was found only after digestion with Hind III. The absence of the 13 kb polymorphic band appeared to be correlated with the development of SLE (relative risk = 4.78).

[The effect of beraprost sodium on the Raynaud's phenomenon].

We investigated the effect of beraprost sodium (BPS) on the Raynaud's phenomenon on 15 patients with systemic sclerosis, 3 with mixed connective tissue disease and 1 with Raynaud's disease, respectively. After 12 weeks of administration of 60 micrograms/d BPS, the duration and the incidence of the Raynaud's symptom were significantly reduced and the dermal temperature on the fingers was found to be elevated. Of the parameters which are known to reflect vascular endothelial damages such as tissue plasminogen activator (t-PA), von Willebrand's factor (vWF) and endothelin, the plasma level of t-PA was significantly reduced by BPS. Furthermore, the capillary loop in the nail bed of the fingers seemed to increase in one patient by the treatment with BPS. These results suggest that BPS has a capacity to repair peripheral vascular damages resulting in the improvement of Raynaud's phenomenon.

[Efficacy and safety of percutaneous endoscopic gastrostomy in the elderly].

Percutaneous endoscopic gastrostomy (PEG) was performed in 32 patients with nasogastric feeding (NGF) or total parenteral nutrition (TPN), who were unable to swallow. Our cases of PEG included 10 with dementia, 8 with cerebral infarction, 8 with cerebral bleeding, 3 with gastric cancer, and others. TPN was performed after PEG for a short time. Because of the combination of TPN and PEG, there was no mortality or major complication related to the procedure. Minor complications included subcutaneous abscess and TPN catheter fever. PEG can be safely and rapidly performed. Furthermore, painless life, better cosmetic features and physical condition can be obtained with this procedure. The majority of the patients are able to return home after PEG. PEG can improve the quality of life of the patients who cannot swallow but have an intact gut.

[Rheumatic pain].

RA is a chronic inflammatory arthropathy that characterized by joint destruction with invasive proliferation of synovial cells in articular cartilage. Inflammatory cytokines like TNF-alpha, IL-1 and IL-6 are overproduced and play an important role in the process. In order to relief patient's chronic pain and to prevent of joint destruction, NSAIDs, steroids and DMARDs are common. Recently, anti-cytokine therapy develop and neutralizing antibody to TNF-alpha (infliximab) and TNF-alpha receptor/IgG fusion protein(etanercept) are already used in U.S.A. and in Europe. In Japan, these medicines are now on clinical trial and will be available in a few years. Other anti-cytokine therapy like IL-1 receptor antagonist and anti-IL-6 receptor antibody is also on clinical trial.

Familial Pelger-Huet anomaly accompanied by tuberculosis and complicated by acute polyarthritis.

A patient with familial Pelger-Huet (PH) anomaly, which accompanied tuberculosis, and acute polyarthritis is described. Investigations surrounding this case suggests that the immunological abnormalities may be associated with the PH anomaly and the tuberculosis, and that the complications may be related to the development of the acute polyarthritis.

Sequence analysis of a cDNA clone of a gene encoding a component of a putative phosphorylcholine-specific T suppressor factor and functional property of its gene product.

We have isolated a putative phosphorylcholine (PC)-T cell suppressor factor (TsF) cDNA clone, p6-5, from a cDNA library of a T hybridoma which constitutively secretes a PC-TsF in vitro [8]. In the present study, we determined the nucleotide sequence of the p6-5 gene and found that the p6-5 sequence is 86% homologous to rat preproelastase 1 gene, one of the serine protease genes. An oligopeptide (14 mer, TsF14) deduced from the p6-5 sequence was synthesized and antisera against TsF14 were prepared in rabbits. Anti-TsF14-conjugated Sepharose 4B specifically absorbed the PC-TsF activity from the culture supernatant of PC-TsF-secreting T hybridomas. In contrast, the binding molecule eluted from the anti-TsF14-conjugated Sepharose suppressed the antibody response PC specifically. These results indicated that the p6-5 polypeptide is a component of the PC-TsF molecule.

[A case of malignant rheumatoid arthritis with long-term improvement by regular use of cryofiltration plasmapheresis].

A Japanese man, 25 years old, suffered from stiffness, swelling, and pain of his joints in May 1983. He was diagnosed as rheumatoid arthritis and was given steroid unsuccessfully. Then, he was admitted to our hospital in February 1984. Because of the presence of high fever and rash on admission, differential diagnosis of RA from adult Stills disease was difficult. However, skin biopsy disclosed apparent vasculitis, leading to the definite diagnosis of malignant RA (MRA). We could not induce any remission by large dose of steroid including pulse therapy, various immuno-suppressants, and anti-rheumatic agents, because of lack of effectiveness and side effects of the drugs. Double filtration plasmapheresis (PA) was performed, but its beneficial effect soon disappeared. On the other hand, cryofiltration PA caused more beneficial and prolonged effect, resulting in improvement. Thereafter, he was successfully followed on regular use of PA for about 6 years. His condition depended on the interval between PA and next PA and on the volume of filtrated plasma. He died of septicemia on March, 1991. We report here the case of MRA with long time improvement by regular use of cryofiltration PA.

Adult Still's disease: review of 228 cases from the literature.

To clarify the clinical pictures of adult Still's disease, 228 cases reported in the past 15 years since Bywaters' first description were reviewed. These included our 9 new cases and an additional 25 cases from the Japanese literature, none of which had been described in previous English reviews. Most of the patients with long followup showed frequent recurrences. About one third developed deforming arthritis with ankylosis. There were 6 deaths. Of interest was the remarkably elevated levels of serum ferritin and prostaglandin E1 in some patients.