Aortitis syndrome (AoS) associated with pulmonary hypertension (PH) is rare. Patients of AoS associated with PH are milder than those of mixed connective tissue disease (MCTD) with PH.
We measured the anticardiolipin antibody (aCL) in the plasma of 10 patients with AoS and 17 patients with mixed connective tissue disease (MCTD), and tried to find out whether it correlates with PH. All two patients with AoS and PH were negative for aCL, on one hand all three patients with MCTD and PH were positive for it. Two cases of AoS complicated with PH were reported. In case 1, PH appeared 14 years after the diagnosis of AoS and the patient has been followed up as an outpatient for 6 years. In case 2, PH appeared 6 years after the diagnosis of AoS and the patient died of cardiac failure by the complication of PH after 6 years. The survival periods in patients with AoS and PH, and MCTD and PH were over five and 0.8 years, respectively.
We consider that the mechanism of PH between AoS and MCTD are different and the difference of it has influence on their prognosis.

View full abstract

Chronic idiopathic thrombocytopenic purpura (c-ITP) is an autoimmune disorder, but the abnormalities in immune mechanism have remained only vaguely defined. In this study, we examined the correlation among the platelet counts, platelet associated immunoglobulin G (PAIgG), platelet associated immunoglobulin M (PAIgM), platelet volume, various immunological functions and severity of illness at onset. In c-ITP patients an inverse relation ship was found between the platelet counts and PAIgG (r=-0.3648, p<0.05). The correlation was found between PAIgG and PAIgM (r=0.6055, p<0.001). And in c-ITP patients an inverse relationship was also found between the platelet counts and PAIgM (r=-0.3456, p<0.05). In c-ITP the proportion of CD 3 and CD 8 were significantly decreased (p<0.05) compared with controls. But there were no difference in proportion of CD 4 and CD 4/8 ratio. There was no difference in lymphocytic blastogenic response to phytohemagglutinin (PHA). Stimulation index of concanavalin A for patients' lymphocytes were significantly decreased compared with controls' lymphocytes. The proportion of T-cells was no different than those of control. But the proportion of B-cell was a significantly increased (p<0.05) compared with controls. There were no correlation among clinical severity of illness at onset and immunological functions, platelet antibodies and platelet volumes. These data suggested that the abnormalities of T-cell subset and the proportion of B-cell in peripheral blood may play an important role in outbreak of c-ITP in childhood.

View full abstract

Farr's assay using ¹²⁵I labelled double-stranded DNA (dsDNA) obtained from E. coli plasmid by recombinant technique is the most sensitive and specific method for the detection of anti ds-DNA antibodies in patients with clinically active systemic lupus erythematosus (SLE). Because of insufficient dsDNA antigen, however, final antibody levels were hardly determined when the sera contained antibodies more than 100U/ml. A new Farr's assay has been developed by increasing dsDNA antigen, which enlarged calibration range from 0100U/ml up to 0300IU/ml. The final antibody levels of thirty two out of thirty nine SLE sera (82.1%) with antibodies more than 100U/ml by current Farr's assay have been measurable by the new Farr's assay. An excellent correlation has been observed between two assays in sera showing antibody less than 100U/ml by current Farr's assay (r=0.9346, p<0.01).
The application of newly developed Farr's assay, thus, is a useful tool for the evaluation of clinical characteristics and course of SLE patients who had high levels of anti dsDNA antibodies.

View full abstract

We have established a simple, sensitive and reproducible sandwich enzyme-linked immunosorbent assay (ELISA) for quantitation of immunoglobulins of λ VI subgroup (Ig λ VI) which has been of special interest by its amyloidogenicity. It used murine monoclonal antibodies specific for λ VI subgroup of variable region (V_L) and for λ type of constant region (C_L) of human immunoglobulin light chains. The λ VI protein level and the percent distribution of λ VI subgroup among total Igλ proteins (λ VI/λ ratio) in serum were determined. Igλ VI proteins were contained in each of serum specimens tested. In serum specimens obtained from 44 normal subjects and 14 patients with polyclonal hypergammaglobulinemia and six patients with hypogammaglobulinemia, the concentration (mean±SD) of Igλ VI proteins was 78 to 432 (197±81), 218 to 1875 (648±474) and 49 to 115 (85±25) μg/ml and that of total Igλ proteins was 2, 640 to 8, 300 (4, 712±1, 454), 8, 197 to 30, 000 (14, 837±7, 029) and 890 to 1, 800 (1312±340) μg/ml, and the λ VI/λ ratio (mean±SD) was 1.2 to 9.4 (4.3±1.7), 2.1 to 6.4 (4.2±1.7) and 4.6 to 9.2 (6.5±1.6)%, respectively. The λ VI/λ ratio was strikingly higher than 10% in serum specimens from three patients with λ VI-related amyloidosis, which implied clinical and diagnostic availability of this ratio. In patients with rheumatoid arthritis, systemic lupus erythematosus and liver cirrhosis presenting polyclonal hypergammaglobulinemia, λ VI proteins were contained at high concentration in serum, but no amyloid deposition was found in biopsied specimens. However, further study on more cases with polyclonal Igλ VI protein overproduction is required to elucidate amyloidogenicity of non-malignant Igλ VI proteins. The assays described here will facilitate the study on monoclonal and polyclonal nature of this unique λ VI subgroup in various disease conditions.

View full abstract

15 patients were diagnosed as having cytomegalovirus (CMV) infection based on the positive reaction to antiCMV-IgM, and -IgG and clinical symptomes. The expression of CD 4, CD 8, HLA-DR, IL 2 Rβ, CD 7, CD 45 RA and CD 45 RO in peripheral blood mononuclear cells were examined on flow cytometrical analysis.
The mean (±SD) proportion and number of CD 8 positive (CD 8⁺) cells were 36.7± 9.0%, and 4, 762±1, 162 cells/μl for the infants with CMV infection and 20.9±5.0%, and 1, 414±127 cells/μl for healthy infants: the ratio of CD 4⁺ cells to CD 8⁺ cells was significantly decreased and the number of CD 8⁺ cells was significantly increased compared with those in healthy infants. The expression of HLA-DR, IL 2 Rβ and CD 45 RO on CD 8⁺ cells were increased. The marked increase of CD 45 RO expression and the decrease of CD 7⁺ cells, as observed in infectious mononucleosis caused by EB virus were not observed in CMV infection.

View full abstract

Peripheral blood lymphocytes (PBL) possess lectin-dependent cellular cytotoxicity (LDCC) in the presence of various lectins. In this study we showed more potent LDCC could be induced in lymphokine-activated killer (LAK) cells. Among several lectins investigated in this study pokeweed mitogen (PWM) could give LAK cells most potent and broad spectrum LDCC when LAK cells were initially stimulated by PWM. Analysis of effector cells revealed that either PBL, CD 3⁺ CD 4⁺, CD 3⁺ CD 8⁺ cells or CD 3^- CD 56⁺ cells expressed manifest cytotoxicity in the presence of lectins. Both anti-HLA class I and anti-HLA class II monoclonal antibodies did not inhibit LDCC at any concentrations applied. Taken together with the former result, HLA, CD 3, CD 8 or CD 56 molecules were not relevant to LDCC. Quality and quantity of binding sites were shown to be different between effector cells and target tumor cells revealed by FACS analysis using fluorescence conjugated PWM. Mechanism of LDCC partially associated with apoptotic cell death but without NO production by effector cells. These facts suggest that more potent immunotherapy of malignant diseases might be applicable using mechanism of LDCC.

View full abstract

Autoimmune hepatitis type 2 (AIH type 2), which has anti-liver-kidney-microsome type 1 (LKM 1) antibody, is a poor prognostic disease, although the administration of corticosteroid is sometimes remarkably effective. Most of the patients are found in young female and get worse so as to progress to liver cirrhosis. A 20 years-old woman with the rare combination of AIH type 2 and autoimmune hemolytic anemia (AHA) was reported. In 1982, she presented jaundice and anemia. In 1983, the level of transaminase increased. After that, hemolytic anemia and liver dysfunction had been presented repeatedly. In September 1990, again she presented liver dysfunction. The level of bililubin and transaminase increased remarkably, and she presented striking jaundice and splenomegaly. Anti-LKM 1 antibody was presented in both sera at 12 years old and at 20 years old. Following the steroid pulse therapy, the condition of a disease has improved remarkably. In laparoscopy, her liver showed liver cirrhosis. The findings confirmed the diagnosis of AIH type 2 with AHA. This report is the first case of AIH type 2 which began from childhood in Japan.

View full abstract

We report a case of protein-losing gastroenteropathy in association with immune deposits. A 27-year-old woman noticed edema of the face and both extremities since February 1992. Laboratory studies revealed severe hypoalbuminemia, positive antinuclear antibody, and low complement levels. She was admitted to our hospital in April 1992 for further examination. Renal and liver function tests were normal and urinary protein was negative. A diagnosis of protein-losing gastroenteropathy was made by increased α₁-antitrypsin clearance and ^99mTc-labeled human serum albumin scintigram showing abnormal radioactivity in gastrointestinal tract. Subjective symptoms suggesting collagen diseases were not complained except for mild oral thirsty. Although endoscopic intestinal biopsies revealed non-specific inflammation with a small number of mononuclear cell infiltration, immunofluorescent studies demonstrated deposits of IgG, IgM, and C 3. Administration of prednisolone improved hypoproteinemia and low complement level. These findings suggest that autoim-munological mechanism may cause proteinlosing gastroenteropathy.

View full abstract

A 59-year-old woman who had a high fever of more than 39°C for two days and suffered from general fatigue and generalized nodular rash without itching for one month was admitted to our hospital at the end of April 1991. Laboratory findings on admission showed leukocytopenia (3, 900/μl) and liver dysfunction (GOT 324 KU, GPT 186 KU). Her laboratory data and body temperature became normal with medication. However, subsequently her body temperature rose again and rash was exacerbated. With lymph node swelling in the neck and axillae, myalgia, polyarthralgia and abdominal pain, numbness in hands and Raynaud's phenomenon occurred. Laboratory findings at the 15th day were as follows: the erythrocyte sedimentation rate was 7mm/hour, CRP 9.07mg/dl, white blood cell 27, 000/ml, blood urea nitrogen 74mg/dl, serum creatinine 3.86mg/ml, IgG 571mg/dl, IgA 63mg/ml, IgM 106mg/ml, antinuclear antibody positive (80×), and CD 4/CD 8 ratio 0.54. From these findings, polyarteritis nodosa was highly suspected. Histological findings from biopsied lymphnodes, however, revealed IBL-like T cell lymphoma with surface markers of MT-1 and leu 3 a. Lymphoma cell infiltration was also observed in the biopsied skin. This is a rare case of IBL-like T cell lymphoma accompanied by hypogammaglobulinemia.

View full abstract

This paper reports a case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL) resembling systemic lupus erythematosus (SLE). A 48-year-old lady was hospitalized with complaints of abdominal pain and high fever. She was found to have a pleural effusion in the right side, hepatosplenomegaly, general lymphadenopathy, and Raynaud's phenomenon. Laboratory examination revealed the followings: ESR 125mm/hr, Hb 9.5g/dl, WBC 5, 400/μl, platelet 1.6×10⁴/μl, creatinine 3.0mg/dl, amylase 622 IU/l, γ-globulin 4.19g/dl, CRP 15.2mg/dl, CH 50 less than 12.0U/ml, rheumatoid factor 148.9IU/ml, negative tests for LE cells or antinuclear antibody. A biopsy of the cervical lymphnodes revealed a marked increase of plasma cells with preserved follicular structures. She was diagnosed as IPL, and successfully treated with the combination chemotherapy (vincristine 1.5mg, cyclophosphamide 500mg and prednisolone). IPL is a distinct clinical entity, characterized by systemic lymphadenopathy and marked polyclonal hyperimmunoglobulinemia. The disorder must be distinguished from other diseases, in the spectrum of lymphadenopathies from angioimmunoblastic lymphadenopathy-related disorder to autoimmune-associated lymphadenopathy, most notably SLE.

View full abstract

Selective IgA deficiency associated with systemic autoimmune diseases has been reported by many groups. However, there have been few reports concerning the observation that systemic autoimmune disease broke out in the course of IgA deficiency.
A 45-years-old woman with selective IgA deficiency induced by anticonvulsant medication has been followed for a few years. She developed polyarthritis with positive rheumatoid factor and was diagnosed as rheumatoid arthritis.
After 6 month, systemic lupus erythematosus with nephrotic syndrome has been developed. Lack of secretary IgA and decreased level of serum IgG 2 which might induce recurrent infection was observed. Although the origin of the association between selective IgA deficiency and occurrence of autoimmune diseases is hypothetical, we discussed the relations among selective IgA deficiency, recurrent infection, long term anticonvulsant medication and occurrence of SLE.

View full abstract