The comparative studies of patients with primary Sjögren's syndrome between in the presence of renal tubular acidosis and in the absence of renal tubular acidosis were performed.
Nine out of 30 patients with primary Sjögren's syndrome had renal tubular acidosis.
HLA-DRW9 was significantly increased in frequency in the patients with primary Sjögren's syndrome with renal tubular acidosis (6/9=66.7%), as compared to 26.2%(115/439) among control Japanese and 14.3%(3/21) among patients with primary Sjögren's syndrome without renal tubular acidosis.
The possibility that primary Sjögren's syndrome with renal tubular acidosis is genetically different from the patients with primary Sjögren's syndrome without renal tubular acidosis is discussed.

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The subsets and function of Leu-7⁺ cells in the peripheral blood were investigated in 26 patients with myeloma and 20 age-matched normal controls to elucidate the pathogenesis of humoral immunodeficiency in myeloma.
The subsets of Leu-7⁺ cells were analyzed by two-color immunofluorescence assays using mouse monoclonal antibodies. The NK cell activity of lymphocytes was measured against ⁵¹Cr-labeled cell line K562 (E/T: 25). The isolated Leu-7⁺ cells were co-cultured with the mononuclear cells from healthy subjects in the presence of pokeweed mitogen for 7 days and the secreted IgG, IgA and IgM into medium from the lymphocytes were measured by ELISA to investigate the effect of Leu-7⁺ cells on Ig synthesis by B cells.
The proportion of Leu-7⁺ cells in the peripheral blood was higher in myeloma patients (33.4±11.0%, mean±SD) than in healthy subjects (20.5±4.9%). The percentage of cells co-expressing Leu-2a marker in Leu-7⁺ cells was increased in myeloma patients compared with healthy subjects (52.6±13.1% vs 29.5±12.3%) and showed a positive correlation with monoclonal Ig levels in the serum of the patients with myeloma. Peripheral blood lymphocytes from myeloma patients and healthy subjects had similar NK cell activity (48.6±22.9%, 53.1±15.0%, respectively). The Leu-7⁺ cells from myeloma patients suppressed IgG, IgA, and IgM synthesis by B cells more strongly than those from healthy subjects.
These results indicate that the Leu-7⁺ Leu-2a⁺ cells are significantly increased in myeloma patients, having low NK cell activity and suppress the production of polyclonal Ig by B cells. In addition, it is suggested that the Leu-7⁺ Leu-2a⁺ cells in myeloma are induced by myeloma cells. Accordingly, it is considered that the Leu-7⁺ Leu-2a⁺ cells may have a role of immunosuppression in humoral immunodeficiency in myeloma.

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Thirteen patients with 15 pregnancies occurring during the course of systemic lupus erythematosus (SLE) were reviewed. The effects of the disease activity and the therapy on the outcome of the pregnancy were evaluated.
Six patients became pregnant when the disease was active, including three patients who were first diagnosed as SLE after the conception. Three of them were treated with sufficient dose of steroids, and rescued. But the others lost their lives after delivery because of renal insufficiency. All of the seven patients (9 deliveries) who conceived during inactive phase of the disease delivered healthy babies at full term, although some of them increased the activity of their disease a little during the pregnancy. Eleven patients (13 pregnancies) were treated with a high dose of steroid after their deliveries, and their clinical courses were fine. However, two patients who did not take enough doses of steroid drugs died in postpartum period.
The data indicate that successful outcome of pregnancy may be expected in patients who do not have severe renal disease and conceive during the inactive phase of the disease. Administrations of enough doses of steroids to suppress the disease activity is advised even during pregnancy, and high doses of the drug seem to limit the incidence of exacerbation following delivery.

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Serum immunoglobulin (IgG, A, M) levels were measured in 123 children with Type I diabetes in order to clarify the relationship between immunoglobulin levels and islet cell antibodies (ICA) or the duration of illness and to investigate the incidence of selective IgA deficiency in children with Type I diabetes in Japan.
IgA levels in Type I childhood diabetics were significantly higher than in normal children; however, IgG and IgM levels in Type I childhood diabetics were similar to those in normal children. There was no correlation between immunoglobulin (IgG, A, M) levels and the duration of illness. Immunoglobulin levels in patients with ICA were about the same in those without ICA. IgM levels in ICA-positive patients with a duration of less than one year were significantly higher in ICA-negative patients. Two out of 123 children (1.6%) with Type I diabetes had selective IgA deficiency.
From these results, it was suggested that high IgM levels in ICA-positive diabetic children with short duration might show recent viral infection and that the incidence of IgA deficiency in Japanese children with Type I diabetes was slightly lower than in Caucasians.

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We previously reported the presence of precipitating antibodies specific to one or more of three mitochondrial antigens (M-A, M-B, M-C) in sera from patients with primary biliary cirrhosis (PBC). It was also confirmed that antibodies to M-A and M-B are useful markers for the diagnosis of PBC. These mitochondrial antigens reactive with PBC sera were further characterized and purified in the present study.
When a crude mitochondrial preparation was run on a DEAE Sephacel column using a 0.1-0.3M NaCl gradient, satisfactory resolution was not obtained among M-A, M-B, and M-C. However, when the same antigen preparation was eluted from a Sepharose 6 B column, peaks were obtained at molecular weight of 310kd, 240kd, and 155kd. These were found to correspond to M-C, M-A, and M-B, respectively, by counterimmunoelectrophoresis. Western blot analysis using a pool of the three Sepharose 6 B peaks as antigen showed that under reducing conditions, M-A antisera reacted with a 41kd band, M-B antisera with a 50kd band, and M-C antisera with a 31kd band. The pooled preparation was then purified and concentrated 1000× on a CNBr-activated Sepharose 4 B affinity column using anti-M-A and M-B IgG. Presently, additional characterization experiments are being conducted on these antigens in our laboratory.

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A 58-year-old female, who suffered from general fatigue and faint attack since 1983, was admitted to our hospital because of further examination for hypogammaglobulinemia in August 17, 1984. On admission increased erythrocyte sedimentation rate and rouleaux formation were demonstrated, but total protein had diminished to 6.3 g/dl with 8.2% γ-globulin. Fundic examination demonstrated sausage-like change in vein and she was diagnosed as hyperviscosity syndrome. Whether thermoprotein existed or not was examined, and cryogelglobulin was detected, although she didn's have Raynaud's symptom. Cryogelglobulin was 60% in cryocrit. Her serum was separated into supernatant serum and gel fraction. Monoclonal peak on γ-region was observed in the whole serum and its gel fractions, and IgM was 2, 700 mg/dl in the whole serum and 3, 360 mg/dl in the gel fraction, while hypogammaglobulinemia was recognized in supernatant serum. Serum protein study revealed that cryogelglobulin consisted of monoclonal IgM, _k and polyclonal IgG. Bone marrow aspiration revealed that the frequencies of plasma cells was 5.2% and that of lymphocytoid cells 1.6%.
Thus, she was diagnosed as primary macroglobulinemia with mixed cryogelglobulin. After administration of procarbazine, she remained asymptomatic and the cryocrit diminished from 60% to 31%.

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Monoclonal antibody Sa 3 was obtained by fusion of spleen cells from the BALB/c mice immunized with Epstein-Barr virus (EBV) transformed human B lymphoblastoid cell line, EBV-SA (HLA-A 24, B 7, C-, DR 1, DQ 1, DP 4) to mouse myeloma P 3-NSI/1-Ag 4-I (NS-1) cells. In the microcytotoxicity test, enzyme linked immunosorbant assay (ELISA) and immunofluorescence technique, this antibody reacted with EBV-transformed B lymphoblastoid cell line, Burkitt's lymphomas, Pre-B leukemia and peripheral B cells, but not with T cell leukemias, Myeloid leukemia or peripheral T cells. The immunoglobulin class of this antibody, which was designated as Sa 3, was IgG₃. Two-dimensional gel electrophoresis analysis showed that Sa 3 could detect the HLA class II antigens including DR, DQ and DP on the cell surface of EBV-transformed B lymphoblastoid cell lines. In reactivity of Sa 3 against transfected cells, it became clear that Sa 3 could recognize the HLA-DQ and DP antigens expressed on T cell leukemia (CEM) transfected with the cloned DQα and DQβ or DPα and DPβ genes. On the other hand, it was demonstrated that Sa 3 have inhibitory effects on the mixed lymphocyte reaction (MLR) and Pokeweed mitogen (PWM)-induced immunoglobulin synthesis.
In our experiment, since Sa3 could detect the HLA class II (DR, DQ and DP) antigens, this antibody will be quite useful to analyze human immune regulations at the cellular level.

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Recent reports say that it is possible to remove MHC class I from the surface of cell membrane specifically with short termed acid treatment, as a result, LAK activity increases significantly against acid treated cells. For the purpose of introducing the effect to in vivo, the short termed acid treatment was performed to the murine peritonitis carcinomatosa with Meth A fibrosarcoma.
In vitro, ⁵¹Cr release assay revealed that LAK activity increased against acid treated Meth A cells significantly. And in vivo, it was safe that tumor-bearing mice were treated with our technique under anesthesia, so that there were no mortal cases.
On the other hand, the effect of LAK cells and recombinant IL-2 on peritonitis carcinomatosa models in mice was studied with % survival. There was no effect for survival between control group and only LAK i. p. group, but treatment with LAK cells plus acid treatment lengthened median survival significantly. Treatment with IL-2 administration and with LAK cells plus IL-2 cured these mice partially, but in these group plus acid treatment, the therapeutic effect was more evident. With the results, it is prospective that acid treatment before adoptive immunotherapy could enhance the antitumor effect for local therapy.

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We have experienced 37-year-old woman with dermatomyositis associated with recurrent spontaneous mediastinal emphysema during prednisolone therapy. She also had intractable ulcers of her finger-tips and elbows. She received moderate dose of prednisolone and intravenous methotrexate without definite clinical improvement. Mediastinal emphysema was progressive and the patient died of cardiac and respiratory failure.
There have been only one paper reporting recurrent mediastinal emphysema in patient with PM/DM. According to the literature, relationship between mediastinal emphysema and dermatomyositis in discuss in this paper.

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We investigated the subclass and allotype specificity of anti-IgA antibody in a 7 year-old boy with selective IgA deficiency. The antibodies in this patient were found to be class specific anti-α, subclass specific anti-α₂ and allotype specific anti A 2m (1). He has neither received blood transfusion nor γ-globulin injection, but he has been fed by his mother's breast milk. Serum IgA in his mother was not deficient and its allotype (A 2m (1)+, (2)-) was matched with that of anti-IgA antibody in the patient. Accordingly, it is likely that he was sensitized by IgA come from his mother, however the possibilities remain that these are antibodies against himself or something else which passed through the gut as a result of deficient secretory IgA.
On the other hand, he had diminished ratio of T4/T8, and further, the production of IL-2 and IFN-γ was found to be impaired. The IFN-γ production was restored by adding recombinant IL-2. These findings are interesting as selective IgA deficiency, in which a T-cell defect is suggested in the regulation of B-cell differentiation, as it is reported that IL-2 and IFN-γ are required for B-cell differentiation.

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An autopsy case of systemic lupus erythematosus (SLE) with progressive liver dysfunction is reported. A 12-year-old female was admitted in October 1982 because of eruption on cheeks, hemolytic anemia and proteinuria. On admission, her laboratory examination revealed an elevated anti-single-stranded (ss) DNA antibody, severe coagulopathy, hypoalbuminemia and hypocomplementemia. After the administration of prednisolone, 40 mg/day, she looked clinically well. However, coagulopathy, hypocomplementemia and disturbance of ICG excretion (31.6%) were not improved. Laparoscopy, that was carried out under the supplement of coagulant factors, indicated that her hepatic injury was due to chronic active hepatitis. Therefore, azathiopurine was administered in combination with prednisolone. The titers of anti-ss and double stranded (ds) DNA antibodies, however, were gradually elevated. After the pulse therapy with methylprednisolone, 1 g/day, anti-ss and dsDNA antibody tests became negative, but she finally died in June 1983 because of DIC, followed by hepatic failure. An autosy revealed that the liver was in an early stage of liver cirrhosis showing massive necrosis, fibrosis, marked regeneration of bile stasis.
It has been assessed that the liver involvement in SLE is mild and unworthy of attention for the management of the disease. However, her hepatic injury was closely related to the disease activity and progressive, indicating the association of hepatic damage with SLE. The liver involvement may be important as one of the prognostic indicators in some SLE patients.

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We have experienced a case of systemic lupus erythematosus (SLE) with cardiac tamponade caused by staphylococcus aureus infection. The patient had been treated as SLE for three years at our patient clinic. After the administration of antibiotics for her cervical lymphoadenopathy following dental therapy, ulnar and palmar erythema were aggravated.
Steroid pulse therapy was performed for her exacerbation. Three days after the pulse therapy, low blood pressure, tachycardia, paradoxical pulse and friction rub sound were appeared and echocardiogram demonstrated a large quantity of pericardial effusion, from which staphylococcus aureus was detected.
After the drainage and administration of effective antibiotics for that bacteria, her symptomes were remarkably improved. Pericarditis in SLE is the most common cardiac manifestation. But infectious pericarditis and cardiac tamponade is rare. In this case we suspect the focus of this infection was digital ulcer or cervical lymphonode.

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In this report, we described a patient of overlap syndrome of rheumatoid arthritis (RA) with sjögren's syndrome and systemic lupus erythematosus (SLE).
She had been treated for definite RA for ten years until she was made a diagnosis of SLE according to an oral ulcer, pleuritis, hemolytic anemia, lymphocytopenia, positive reaction of LE test, and existence of various anto-antibodies.
Also she was suggested to be accompanied with sjögren's syndrome by dry mouth, dry eye, and keratoconjunctivitis sicca.
After the treatment with prednisolone, marked improvement of clinical features and laboratorial data were observed.
Up to the present, the case report of overlap syndrome of RA and SLE is rare. This case has many suggestive data about classification of collagen diseases.

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A 64-year old man was admitted to Toyama Medical and Pharmaceutical University Hospital in July 1983 because of systemic lymphadenopathy. On physical examination, enlarged inguinal and axillary lymph nodes were palpable; they were matted together, elastic hard, and not tender. Laboratory findings were as follows: The erythrocyte sedimentation rate was 39mm/hr; The total serum protein was 7.6 g/dl with hyper γ-globulinemia (43%). Immunological studies showed elevated immunoglobulin M (IgM) level of 3, 650 mg/dl. Immunoelectrophoresis demonstrated M protein identified as IgM-_κ.Bone marrow aspiration revealed an increase in abnormal lymphoid cells. Histologic and immunohistochemical studies of the biopsied inguinal lymph node revealed follicular lymphoma characterized by the tumor cells containing Ig M and _κ-light chain in the cytoplasma. A diagnosis of primary macroglobulinemia was established with clinical features and lymph node biopsy. He had a good response with the chemotherapy of prednisolone and melphalan. Prior to discharge in October 1983, his serum Ig M level had decrease to 1, 150 mg/dl. However, he developed left peripheral facial paralysis in November 1983, and paralysis of right vagus nerve and right glossopharyngeal nerve in June 1984. He was readmitted for further examination in July 1984. The cerebrospinal fluid (CSF) indicated increased cellularity (993/3), total protein 116mg/dl, and glucose 119mg/dl. The cells in CSF looked lymphocytoid, and resembled tumor cells infiltrating into other organs as state before. In addition, a computed tomographic scan of his head showed multiple high density areas enhanced by contrast medium. The tumor cells invasion into central nervous system was diagnosed. While in the hospital, round July 1984, he developed neck stiffness, meningeal sign and systemic convulsion. The treatment with prednisolone was unsuccessful, and he was semiconscious, apathic, incoherent. On September 17th 1984, he expired apparently due to CNS involvement.
To our knowledge, tumor cells invasion into the cerebrospinal fluid in primary macroglobulinemia seems to be exceedingly rare.

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Anti-tumor effect of neutrophil migrating into peritoneal or pleural cavity was demonstrated in patients with carcinomatous peritonitis or pleuritis by the intraperitoneal or intrapleural injection of OK-432. Complement-derived chemotactic factor such as C 5a might play a role to attract neutrophil into the cavity, when patients received i. p. injection of OK 432 with fresh frozen plasma as a sourse of complement. Increased levels of thromboxane B₂ and leukotriene B₄ as well as C 5a were found before neutrophil attracted in the fluids. These results suggested that arachidonic acid metabolites participated in the migration of neutrophil.

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