Fifteen cases with severe systemic lupus erythematosus (SLE) were treated with bolus administration of methylprednisolone and 10 of them had marked improvement of subjective symptoms.
They were grouped into central nerveous system involvement (CNS-lupus) group with neurologic signs, CNS-lupus group with psychiatric illness, a group with lupus nephritis, a patient with vasculitis and the others. 6 cases of the 9 CNS-lupus patients with neurologic signs, 2 cases of the 3 lupus nephritis patients, a patient with vasculitis were involved in “good response group”: Psychiatric illness of 3 CNS-lupus cases on the other hand, did not show any clinical improvement.
The mean pheripheral white blood cell (WBC) count rose from 4, 200/mm³ at the start of therapy to 7, 050/mm³ within 4 weeks after pulse therapy (p<0.005) and WBC count of all cases recovered to normal range. Antinuclear antibody (ANA) titer decreased in only 2 (15.4%) of the 13 ANA positive cases. Low serum complement activity (CH50) became normal range after treatment only in 15.4% of the cases. The improvement of the ANA titer and the value of CH50 did not correlate with that of clinical symptoms in each case. In many cases, the improvement of ANA titer and the value of CH50 appeared within 16 weeks after pulse therapy.
Three of 15 cases complicated severe infection as adverse effects of methylprednisolone, and one of them had diabetes mellitus and hypertension.

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We attempted to clarify the effect of age on the clinical manifestations and complications of systemic lupus erythematosus (SLE). One hundred and forty two patients with SLE were classified into 3 groups according to their ages at the time of diagnosis.
The number of patients of the 3 groups, younger onset group (less than 19 years old), common onset group (between 20 and 39 years old), and older onset group (more than 40 years old) was 27, 91 and 24, respectively.
There was a higher incidence of malar rash, oral ulcers, fever elevation and renal disorder, especially nephrotic syndrome, in the younger onset group than in the other two groups. Serositis, particularly pericarditis, hypocomplementemia and anti-DNA antibody were also more frequent in the younger onset group compared with the other two groups.
As for complications, although avascular bone.necrosis of femoral head is more frequent in the younger onset group, there was no significant difference in the incidences of herpes
zoster and vascular thrombosis between the three groups. The response to the corticosteroid therapy and the prognosis were generally poorer in the younger onset group than in the other
two groups.
These data suggest that clinical manifestations and complications in younger patients with SLE differ from those in older patients.

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We investigated cell surface phenotypes of anti-DNA antibody-producing B cells from NZB×NZW (B/W) F 1 mice, using several monoclonal antibodies (mAb) to B cells includ. ing Ly-1 (CD 5) and our newly established Lp-3 which defines a unique B cell differentiation antigen. The 2-mo-old B/W F 1 mice spontaneously produced IgM-class but not IgG-class anti-DNA antibodies, while the 7- to 8-mo-old B/W F 1 mice produced mainly IgG and less amounts of IgM anti-DNA antibodies. In in vitro studies using FACS-sorted B cells, we found that surface phenotypes of the IgM anti-DNA antibody-producing B cells are Ly-1⁺, Lp-3^-, sIgM⁺ and CD 45 R⁺, whereas those of IgG antibody-producing B cells are Ly-1^-, Lp-3⁺, sIgM^-, sIgG⁺ and CD45R⁺. The majority of peritoneal B cells from either 2- or 7-mo-old B/ W F 1 mice was Ly-1⁺, Lp-3⁺, sIgM⁺ and CD45R⁺, and produced neither, if at all, IgM not IgG anti-DNA antibodies. Whether such phenotypical distinction represents the differences in the lineage or the changes during differentiation of B cells is discussed.

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Lymphokine-activated killer (LAK) cells have been shown to have anti-tumor.effect in vitro and in vuvo. Prompted by our recent finding that LAK cells mediate both veto and natural suppression, we tested the ability of adoptively transferred LAK cells to block lethal graft-versus-host disease.
Lethal GVHD was induced in sublethally irradiated (5.5 Gy)Balb/C mice by injecting C 57 BL/6 spleen cells intraperitoneally. Nearly all mice receiving this dose of irradiation alone survived whereas irradiated mice injected with C 57 BL/6 spleen cells alone all died of lethal GVH by day 12. By contrast, similarly treated mice all survived when they were coinjected on the same day or one day later with lymphokine-activated recipient-type Balb/C spleen cells.
These findings provide a substantial background of experience which could be readilly applicable to future trials of adoptive immunotherapy with LAK cells to prevent GVHD.

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In an attempt to identify prognostic indicator of patients with systemic lupus erythematosus (SLE) complicated by infection, we compared laboratory findings between those with fatal outcome (the fatal group) and those whose infections had been successfully controlled (the recovery group). From among 121 SLE patients hospitalized at University of Tsukuba Hospital between 1976 and 1986, 26 episodes of bacterial, fungal or protozoal infections were recorded. Infections contracted while SLE was in remission and viral infections were excluded from the study. The fatal group patients acquired more infections classifiable as opportunistic infections including sepsis and pneumonia. They were associated with hypocomplementemia and leukopenia more frequently than the recovery group patients. Since hypocomplementemia and leukopenia are common findings in patients with both active SLE and severe infection, it is difficult to accurately evaluate the disease activity of SLE complicated by infection. But the comparison of serum complement values before and following the infection was considered to serve as a useful prognostic indicator.

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The purpose of this paper is to determine what types of circulating immune complex (CIC) assays are the most suitable for monitoring rheumatoid arthritis (RA) patients treated with plasmapheresis (PP).
PP using the double filtration method was carried out in 11 patients with RA. In each PP procedure, 2, 000mll of plasma was passed through the filtration column. Each procedure was performed once a week for 4 weeks. CIC was measured by three different methods, namely Raji cell assay (RJC), anti-C 3 assay (AC 3) and C 1 q solid-phase assay (C 1 q SA). The clinical activities of RA were evaluated using the Lansbury indices.
CIC titers significantly decreased in 4 weeks after PP treatment for 3 patients measured by RJC and for 6 patients by AC 3. A significant decrease of CIC measured by C 1 q SA was also observed for 3 patients in 1 week after treatment.
The decrease of CIC titers measured by AC 3 was associated with a significant improvement of Lansbury index during 8 weeks after treatment. However, there was no relationship between the decrease of CIC titers by RJC or C 1 q SA, and the changes in Lansbury index after PP treatment.
In conclusion, AC 3 assay for CIC in RA patients treated with PP was thought to be best among the 3 different methods.

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The cytotoxic activities of polymorphonuclear leukocytes (PMN) induced by intraperitoneal (i. p.) administration of OK-432 or Nocardia rubra cell wall skeleton (N-CWS) were examined by a ⁵¹Cr release assay. Male C3H/He mice, 8_??_10 weeks old, and syngeneic MM46 mammary adenocarcinoma cell line were used in this experiment. PMN accumulated in the peritoneal cavity 6 hrs after i. p. injection of 1 KE of OK-432 or 200μg of N-CWS showed no cytotoxicity. Very strong cytolysis, more than 80%, was obtained when PMN was cultured with N-CWS in the concentrations from 10μg/mll to 100μg/ml, while the addition of OK-432 in vitro did not enhance the cytotoxicity. This enhanced cytotoxicity by the addition of N-CWS was observed at effector: target ratios from 0.7 to 50. It is concluded that combined use of different kinds of biological response modifiers such as OK-432 and N-CWS enhances cytotoxicity of PMN.

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An enzyme-linked immunosorbent assay (ELISA) was developed to detect subtypic determinants (d, y, w and r) of hepatitis B surface antigen (HBsAg) with monoclonal antibodies in a total of 182 sera of 91 HBsAg positive asymptomatic carriers (ASC, mean age; 18 years old) and 91 HBsAg positive patients with chronic liver disease (CH, mean age; 33 years old) in Japan. In 91 ASC, the number of adr was 68 (74.7%), that of adw was 18 (19.8%), that of ayw was 2 (2.2%), and that of adwr was 3 (3.3%). In 91 CH, the number of adr was 88 (96.7%), that of adw was 1 (1.1%), that of adyr was 1 (1.1%), and that of adwr was 1 (1.1%). Thirty-six (52.9%) in 68 ASC of adr were positive anti-HBe. In contrast, 14 (77.8%) in 18 ASC of adw were positive anti-HBe.
In five (5.7%) in 88 CH of adr, the subtype of adr changed to adwr in acute exacerbation. The quantitive level of subtypic determinant w reached its peak before the peak of serum transaminase, parallel to the quantitive level of HBsAg and binding activity of polymerized human serum albumin receptor on hepatitis B virus particles and DNA-polymerase activity. However, the number of adwr in 91 ASC was 3 (3.3%) with normal level of the serum transaminase.
These results suggest that HBsAg positive carriers of adw seem to become positive anti-HBe earlier than HBsAg positive carriers of adr, and that the compound subtype of adwr doesn't always seem to be associated with liver damage.

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A 4-year-old boy with neutropenia associated with agammaglobulinemia was treated with recombinant human granulocyte colony-stimulating factor (G-CSF). The patient had a history of pneumocystis carinii pneumonia at 6 months of age and diagnosed as common variable immunodeficiency (IgG; 58 mg/dl, IgA; 41 mg/dl, IgM; 109 mg/dl, IgE<10 IU/ml) associated with neutropenia (<5%). Although he was placed on a regimen of gamma-globulin administration as a monthly intravenous injection, and no episodes of recurrent infections was observed, he was seen at 4 years of age with a perianal abscess. Neutrophil count was ranged less than 5 percent. Chemotaxic and phagocytic function and oxidative product formation of pheripheral neutrophils were conserved. Neutrophilic cells in bone marrow was decreased with a ratio of myeloid to erythroid of 0.75: 1, and levels of metamyelocytes (1%) and band form (4%) and segmented (1%) neutrophils were much decreased. Bone marrow levels of CFU-GM and CFU-E were slightly increased (488/10⁵ cells) and decreased (2.7/5×10⁴ cells), respectively. Peripheral-blood level of neutrophils was increased by 10 fold by provacation test of prednisolone, but not epinephrine.
As he was unsuccessfully treated with antibiotics and sulfamethoxazole-trimethoprim, the daily administration of G-CSF (2μg per kg of body weight) was performed for seven days. Treatment with this growth factor has been very well tolerated. There has been no local reaction at the perianal sites, rash and edema. Although no any change was observed in level of peripheral neutrophils and that of bone marrow CFU-GM and CFU-E, percent of neutrophilic cells was much increased (32.8%) with two peaks of myelocytes (10.2%) and band formed cells (11.0%). Taken as a whole, neutropenia of this patient was probably due to maturation disturbance at the level of myelocytes in bone marrow and treatment with G-CSF was efficient for therapy of this disorder.

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Ge-132, 1, 500mg per day, was administered to 47 y-o house wife who suffered from rheumatoid arthritis associated with Sjögren's syndrome and microcytic hypochromic anemia since 1983. Her clinical stage of rheumatoid arthritis was Stage II and class was 1. Polyarth-ralgia and joint swelling were getting worse even though she was administered both non-steroidal antiinflammatory drug and small dose of prednisolone. Treatment with Ge-132 brought her in a remission state of rheumatoid arthritis and MCV and MCH as well as Hb were improved within 5 months. Two-color flow cytometry of peripheral lymphocytes demonstrated an increase of lymphocyte, CD3^-, CD21⁺ (B cell), CD3⁺, CD21^- (T cell), CD4^-, CD8⁺ (suppressor T cell), CD4⁺, CD8^- (helper T cell), CD16⁺, DR^- (NK cell) and all of double negative cells such as CD3^-, CD21^- cell, CD4^-, C8^- cell, and CD16^-, DR^-cell, parallel to clinical status.
These data surely indicated that Ge-132 is effective to this patient.

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Polymyositis associated with asymptomatic primary biliary cirrhosis, with complex of creatine kinase (CK) and immunoglobulin A-λ is reported. A 44-year-old female was seen to this hospital in December 1988. She complained of proximal muscle weakness and liver dysfunction. CK and aldolase levels were high and EMG examination revealed myogenic changes. She was admitted in March 1989. Anti mitochondria antibody level was very high and the liver biopsy showed chronic nonsuppurative destructive cholangitis. She had no history of icterus and pruritus. She was diagnosed as polymyositis associated with asymptomatic primary biliary cirrhosis. CK isoenzyme electrophoresis revealed an extraband between MB and MM. Further studies showed the existence of complex of CK and immunoglobulin A-λ. She was treated with prednisolone 50 mg/day. Muscle weak-ness gradually improved and she was able to stand up by herself after a month. The extraband on CK isoenzyme electrophoresis also disappeared. Polymyositis associated with asymptomatic primary biliary cirrhosis is rare and is regarded as an interesting case.

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A patient with polymyositis (PM) and the one with Sjögren's syndrome (SjS) who both developed thrombotic thrombocytopenic purpura (TTP) were reported. Case 1, a 41-yearold female who had been suffered from PM for five years and interstitial pneumonitis for three years, had been treated with low doses of prednisolone (PSL) and methotrexate. She was hospitalized because of the exacerbation of the pneumonitis. On admission, blood analysis showed prominent thrombocytopenia. Soon after she developed microangiopathic anemia, renal damage and repeated transient ischemic attack of the brain, and diagnosis of TTP was established. The initial treatment with the increased dose of PSL was not effective, but she responded well to the subsequent treatment with the infusion of fresh plasma and antiplatelet agents. Case 2 was a 35-years-old female with SjS who had been treated with a low dose of methylprednisolone for arthritis, fever and digital ulcers. Mild thrombocytopenia was initially recognized three months prior to the admission and gradually progressed. On admission, she was accompanied by renal damage, microangiopathic anemia and hypesthesia on the left leg and diagnosed as TTP. Corticosteroid therapy was not again successful in this case and the combination of fresh plasma and anti-platelet agents were found to be effective.

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