A 68-year-old male with a 2-year history of rheumatoid arthritis was hospitalized due to severe polyarthritis. Since level of rheumatoid factor was high, and subcutaneous nodules and cutaneous ulcers were present, the case was diagnosed as malignant rheumatoid arthritis (rheumatoid vasculitis). On 10th day after admission, severe dyspnea developed due to acute heart failure, followed by severe melaena. The patient did not respond to various treatments including steroid pulse therapy, and died 3 days later. Autopsy revealed widespread hemorrhagic infarction that was extended from the colon to the rectum. Proliferative endarteritis was recognized in mesenteric vessels, however neither necrosis nor inflammatory changes were observed. The melaena was caused by the interstinal infarction. The intestinal infarction was thought to be responsible for the circulatory disturbances due to heart failure in obstructive vessel lesion of mesenteric vessels.

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To elucidate the long-term renal prognosis in patients with lupus nephritis treated with pulse methylprednisolone, the renal outcome and the risk factors associated with poor renal prognosis were studied in 14 female lupus nephritis patients treated with pulse methylprednisolone. The mean age when the pulse methylprednisolone was started was 28.1 years old and their renal pathology were 3 of WHO III, 10 of WHO IV and 1 of WHO V. Four of the patients fell into renal insufficiency at 5.7 (mean, 1.310) years after pulse methylprednisolone, however residual 10 patients kept their renal function for 11.5 (mean, 3.519.6) years observation. The deterioration of renal function significantly (p=0.033) correlated with glomerular sclerosing index before pulse methylprednisolone, but did not correlate with sustained immunological activity, hypoproteinemia or hypertension. Because glomerular sclerosing index was thought to be one of the risk factors for poor renal prognosis, the indication for pulse methylprednisolone in lupus nephritis was the patients with low glomerular sclerosing index.

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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 60μg/d BPS, the duration and the incidence of the Raynaud's symptom were significantly reduced and the dermal temparature 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.

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The enzymatic amplification of specific DNA sequences by the polymerase chain reaction (PCR) has provided a new approach to genetic typing of HLA-class II region specificities. However, we found the existing PCR protocols to be adequate and/or time consuming, especially when large numbers of samples need to be processed. We have devised a simplified method which alleviates these problems. Genomic DNA prepared from peripheral blood nucleated cells was facilitated using the punched-out filter paper absorbed with the peripheral blood. Second exon HLA-DRB segments of the genomic DNA were amplified by the polymerase chain reaction (PCR) (30 cycles: denaturing at 94 C for 1min: annealing at 55 for 1min, extension at 72 C for 2min) using HLA-DRB primer DRB 5'-1 (ACCGGTCGTTCITGTCCCCICAGCA) and DRB 3'-1 (CTCGCCICTGCACIGTIAAGC) designed in our laboratory. The presence of specific alleles in a PCR-amplified sample was determined by dot-blot hybridization with 32 P-labeled sequence-specific-oligonucleotides (SSOs). Quantitation of the producer is being evaluated using an automated scanner.
These newly designed primers allowed amplification of the all known expressed alleles of DRB 1, B 3, B 4 and B 5 loci. This type of reproducible and precise assay is very useful in the HLA-class II typing of large number of patients.

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CD 46, membrane cofactor protein, is a membrane protein which shows different expression and phenotypes with the organ or cell in the same individual. Previously, we reported that gastric CD 46 was expressed strongly in the mucosal epithelium, mucosa and endothelial cells of vessels in the submucosal layer. Western blot analysis revealed that gastric CD 46 was expressed as one broad band with a molecular weight ranging from 60 kDa to 69 kDa, which was different from that of lymphocytes. In this study, we investigated the differences in expression and characterization of CD 46 in gastric cancer obtained by surgery as compared with non-cancerous mucosa. Expression of CD 46 was greater in gastric cancer than in non-cancerous mucosa. In some cases, the phenotypes of CD 46 in gastric cancer were different from those in the non-cancerous tissue.

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A 56-year-old Japanese woman had a 8-year history of systemic lupus erythematosus (SLE) with recurrent flares. When she was 48 years old, she was diagnosed as having SLE on the basis of fever, polyarthritis, oral ulcers, leukopenia, and positive anti-DNA antibody. Three years later she developed pericarditis and pleuritis, that were improved with treatment with 30mg of prednisone a day. With tapering of prednisone dose to 9mg a day in January 1993, she was admitted due to shortness of breath on exertion. Chest radiograph revealed bilateral elevated diaphragms and sluggish movement with clear lung field. Pulmonary function tests showed restrictive defect with a vital capacity 38% of predicted value. A diagnosis of “shrinking lung syndrome” was made. Simultaneously, blood test revealed leukopenia, elevated red cell sedimentation rate, and elevated anti-DNA antibody titer. Therefore, we suspected this pulmonary involvement was related to lupus flare-up. Treatment with 20mg of prednisone a day resulted in resolution of the patient's dyspnea and in improvement of her vital capacity. Corticosteroid therapy for acute “shrinking lung syndrome” in active SLE can improve symptoms and pulmonary function.

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A 52-year-old female was admitted to our hospital in August 1988, for evaluation of purpura and gingival bleeding. Hematologic examination showed mild leukocytosis (12, 400/μl) and severe thrombocytopenia (1, 000/μl). On bone marrow examination, megakaryocyte count was normal and the number of myeloblasts was increased (7.2%). Serological examination was positive for anti-nuclear antibody and anti-DNA antibody. She was diagnosed as having idiopathic or autoimmune thrombocytopenia, and received thrombocyte transfusion and γ-globulin administration. Hematologic values improved temporarily, but leukocytosis and thrombocytopenia recurred. On the 22nd hospital day, leukocytes increased to 49, 300/μl and thrombocytes decreased to 10, 000/μl. Bone marrow myeloblasts were also increased to 18.8%, and she was suspected of having myelodysplastic syndrome. Then, hematologic values improved simultaneously, and she was discharged in November 1988. After the discharge, leukocyte count ranged from 6, 000 to 16, 500/μl, but the number of bone marrow myeloblasts was normal. However, transient thrombocytopenia appeared in association with decrease or absence of bone marrow megakaryocytes and rise of platelet associated-IgG (PA-IgG) to 99.6ng/10⁷ cells. From September to December 1989, she complained of fever, morning stiffness, multiple arthralgia, and oral ulcer. On serological findings, she was positive for LE cell. Therefore, she was diagnosed as having systemic lupus erythematosus (SLE). In January 1990, she had a high grade fever and dyspnea. Bilateral pleuritis and interstitial pneumonitis were shown on the chest roentgenogram. She received γ-globulin administration, methylprednisolone pulse therapy, and mechanical ventilation. However, hypoxia developed rapidly, and she died of respiratory failure. Autopsy revealed severe interstitial pneumonitis, fibrinous pleuritis, fibrinous pericarditis, and vasculitis in the arcuate artery of the kidney.
This is the first report of SLE complicating thrombocytopenia associated with decrease of megakaryocytes and rise of the PA-IgG, and severe leukocytosis associated with increased bone marrow myeloblasts.

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The association of systemic lupus erythematosus (SLE) and solid tumors is infrequent. Furthermore, there has been no previous description of multiple occurrence of carcinoma in a single patient with SLE. We describe clinical picture of the patient who had dual occurrence of endometrial and gastric adenocarcinomas in addition to clinically active SLE and organ system involvement.

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A 65-year old man presented with complaints of sclerosis of skin and numbness in the extremities. During last 10 year, he had developed monoclonal gammopathy, Raynaud's phenomenon, ischemic heart disease, sigmoid colon cancer, hyperkalemia, polyneuropathy and scleroderma-like skin changes. Laboratory examinations revealed a monoclonal protein (IgA-λ) and an elevated serum level of IL-6. Subsequently a diagnosis of POEMS syndrome was made based on the clinical features and laboratory findings which were characteristic of this syndrome. Further examinations showed the presence of glomerulonephritis and brain tumor. These various complications are of great interest in understanding the pathogenesis of POEMS syndrome.

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We described a 10-year-old girl with SLE copmplicated with anticardiolipin antibody, who showed bleeding tendency since infant. Her platelet was 6×10³/μl. The APTT was remarkably prolonged. The platelet count was normalized after steroid treatment. A false positive Venereal Disease Research Laboratory (VDRL) test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. Her symtoms and the laboratory data fulfilled the criteria of antiphospholipid syndrome. Renal biopsy was performed because of proteinuria, hematuria and the positive of anti-nuclear antibody. The pathology of kidney showed that of lupus nephritis (diffuse membranous glomerulonephritis), and she was diagnosed SLE. She showed no laboratory findings of hyperfibrinolysis and no symtoms of thrombosis under the steroid treatment for 3 years.

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A case of acquired immunodeficiency syndrome (AIDS) developed cryptococcosis which was successfully treated with amphotericin B (AMPH) and fluconazole (FLCZ) is reported. A 52-year-old man was admitted because of pyrexia and oral candidiasis. He had a history of multiple sexual exposures to persons at risk for AIDS in Thailand. On admission, serologic tests for human immunodeficiency virus (HIV)-1 were positive on both EIA and Western blot analysis for anti-HIV-1 antibody. Furthermore, test for cryptococcal antigen and fungal cultures from blood and cerebrospinal fluid revealed that he was suffering from cryptococcemia and cryptococcal meningitis. In spite of identification of Cryptococcus neoformans in his blood and cerebrospinal fluid, the finding of cerebrospinal fluid had a minimal inflammatory response with mild elevation of protein. He was initially treated with intravenous AMPH, 10 to 30mg a day, for 7 weeks, and then was given oral FLCZ, 400mg a day, for the suppressive therapy. His fever subsided three weeks after the start of AMPH therapy. He was eventually discharged 9 weeks after the start of therapy without any symptoms, and continued to receive oral FLCZ as an out-patient. Thus, attention should be paid to diagnosis and treatment for cryptococcal meningitis in AIDS patients.

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