General consideration of primary immunodeficiency diseases (PID) was discussed on the classification of the diseases presented by WHO scientific group. PID were categorized according to the presumed level of the cellular defect.
Thymus had the important role in the ontogeny of T cells. T precursor cells were differentiated under the influence of thymus hormones.
Four different kind of putative thymus hormones were presented briefly. Using the rosette inhibition assay, serum activity of facteur thymique sérique (FTS) was determined for various PID. Patients with DiGeorge syndrome always had low FTS levels. Twenty one out of 23 patients with severe combined immunodeficiency showed low levels but only two had FTS activity within the normal range. Determination of FTS activity might provide a new parameter in the aspect of thymus function in vivo.
Cellular engineering was the specific treatment for PID. Bone marrow transplantation (BMT) was the best method and it could cure many PID permanently as far as a matched donor was present. Effort must be done to find a good method for allogeneic BMT from an unrelated donor or to search the alternative way, for example using fetal organs.
Immunological substitution was also mentioned.

View full abstract

In the present paper, we reported the induction of idiotypic antiserum against anti-thyroglobulin antibody from the serum of a patient with chronic thyroiditis. Antisera were raised against IgG F (ab')₂ of anti-thyroglobulin antibodies obtained from 5 patients with chronic thyroditis. After adequate absorptions, only one antiserum was rendered idiotype-specific, as assayed by solid phase radioimmunoassay.
Specificity of the idiotypic antiserum was examined by the following ways.
1) The idiotypic antiserum reacted with F (ab')₂ of anti-thyroglobulin antibody from patient NAG, but neither F (ab')₂ without anti-thyroglobulin antibody from NAG nor F (ab')₂ of antithyroglobulin antibody obtained from other 4 patients reacted with the idiotypic antiserum.
2) Binding of the idiotypic antiserum to NAG F (ab')₂ of anti-thyroglobulin antibody was inhibited by NAG IgG and NAG IgG F (ab')₂ with anti-thyroglobulin antibody but not by NAG F (ab')₂ without anti-thyroglobulin antibody and pooled human IgG.
The reaction between anti-thyroglobulin antibody and idiotypic antiserum was inhibited by thyroglobulin till 45 percent of control. No cross reaction was detected between the idiotypic antiserum and different IgG obtained from 9 patients with chronic thyroiditis.

View full abstract

The significantly reduced natural killer (NK) cell activity was demonstrated in the peripheral blood lymphocytes (PBL) from 20 female patients with systemic lupus erythematosus (SLE) when compared with NK activity in the age- and sex-matched controls. The reduced NK activity did not correlate with clinical parameters including daily prednisolone doses, serum CH50, antinuclear antibody titers, anti DNA activities, circulating immune complex levels and cytotoxic activities of antilymphocyte antibodies (ALA). The effects of prednisolone and aggre-gated human IgG on NK activity were only slightly suppessive in the in vitro studies. When normal PBL were pretreated with rabbit complement and SLE sera containing ALA, the NK activity of the surving cells was markedly decreased. The decrease was specific and did not seem to be due to physical hindrance of the dead cells. Other heterologous ALA of rabbit origin did not exert a suppressive effect on NK activity. These results suggest that the suppressed NK activity in SLE may possibly be ascribed to an anti NK cell specific antibody in lupus sera although the participation of circulating immune complexes was not completely excluded.

View full abstract

The cholestatic activity which induces a marked reduction of bile flow was detected in the activated lymphocyte culture fluid when the peripheral blood lymphocytes from patients with pulmonary tuberculosis were stimulated with PPD in vitro. This factor (or factors) was fractionated by Sephadex G-75 and DEAE-cellulose column chromatography and was shown to have a similar property as guinea pig cholestatic factor.

View full abstract

The effect of the steptoccocal preparation OK-432 on the in vitro immune responses by the human periperal blood lymphocytes was studied.
The results obtained were as follows.
1) OK-432 did not enhance natural killer (NK) cell activity.
2) OK-432 enhanced cultured spontaneous cell mediated cytotoxicity (CSCMC). The most enhancing effect is at a concentration of around 10^-3 KE/ml and is recognized as statistically significant at the cultured duration of 3, 5 and 7 days.
The activity of CSCMC was most remarkable in the cell fraction which was not adherent to nylon wool and not rosetted with En.
However, some of En-rosette-forming cells also had a little activity.
3) OK-432 induced much more Ig production by B cell than did PWM.
4) OK-432 stimulated DNA synthesis of lymphocytes.

View full abstract

This paper discribed the effect of ATG on heme synthesis of bone marrow erythroblasts from patients with RA by means of Krantz's method. A million bone marrow erythroblasts treated with ATG for 60 min. prior the bone marrow cells culture were cultured with 1.0 U of erythropoietin, 1 μCi of radioiron in 1.0ml of pH 7.4 NCTC 109 containing 20% human heated AB plasma at 37°C, containing 5% CO₂ in air for 72 hrs. After incubation, radioiron activity incorporated into heme extracted by Teale's method was measured by a well type gamma-scintil-lator.
Results were as follows:
1) Heme synthesis of bone marrow erythroblast from patients with RA were lower than that of normal and iron deficient patients.
2) RA patient's plasma did not affect on the heme synthesis of bone marrow erythroblasts from normal subjects.
3) Heme synthesis of bone marrow erythroblasts from patients with RA obviously improved by the treatment with ATG.
These results suggest that the bone marrow lymphocytes of RA patients may be participating in the pathogenesis of anemia seen in RA patients.

View full abstract

The effect of SLE serum on Con A induced suppressor T-cell was studied in in vitro Ig synthesis. Active SLE sera containing IgG type of Tγ specific antilymphocyte antibody (ALA) markedly inhibited the supressor T-cell activity, whereas either IgM Tγ specific ALA or any Ig classes of Tγ (-) specific ALA did not significantly affect the suppressor T-cell activity when compared with IgG Tγ specific ALA.
Other factors possibly present in SLE sera affecting the suppressor T-cell activity were also examined inin vitro Ig synthesis. Aggregated human IgG as a substitute of immune complexes inhibited Con A induced suppression of IgG synthesis in the dose dependent manner. However, their inhibitory rates were far less from those with active SLE sera containing IgG Tγ specific ALA. Corticosteroid also strongly inhibited Con A induced suppressor T-cell activity, while gel-filtration study of lupus serum revealed that the most inhibitory activity was present in IgG (7S) fraction and the removal of IgG from the sera by anti human IgG coated beads resulted in the loss of the inhibition against Con A induced suppressor T-cell activity. These results suggested that the main inhibitory factors contained in active SLE sera for Con A induced suppressor T-cell activity will be IgG type of Tγ specific ALA and other type of ALA may not contribute significantly. Circulating immune complexes and corticosteroid, if they were contained in SLE sera, seemed to play a minor role for the inhibition of suppressor T-cell activity.

View full abstract

Captopril (SQ 14225), the first orally active inhibitor of angiotensin-coverting enzyme, is a promising drug for the treatment of arterial hypertension. Side-effects include rashes, fever, transient loss of taste and proteinuria, and 4 cases of agranulocytosis in the patients with renal impairment have been reported in Caucasians. However, in Japan there are no reports on captopril-associated agranulocytosis due to lack of clinical usage. We have seen a case of agranulocytosis in a patient with lupus nephritis being treated with captopril.
This 30-year-old woman was known to have had systemic lupus erythematosus (SLE) since 1968, the diagnosis being based on clinical findings (skin lesion, arthritis, and glomerulonephritis) and laboratory investigations (antinuclear antibodies, LE cell, anti-DNA antibodies, and reduced level of serum complement). She was treated with high doses of steroid hormone, immunosuppressive drugs, and dipyridamole. Nevertheless, her renal function deteriorated and hypertension with fluid overload became a major problem in May, 1979. Treatment of frusemide, propranolol, α-methyldopa, and hydralazine was unsuccessful in controlling hypertension, as was regular hemodialysis on May 18, 1979. Cephalothin was given for treatment of urinary tract infection, then drug eruption and agranulocytosis appeared. Cephalothin was withdrawn, and over the next 3 days leukocyte-counts rapidly returned to normal. Hypertension in this patient was refractory to conventional therapy. In July, 1979, captopril was given, all the other antihypertensive therapy being discontinued. A prompt and persistent fall in blood pressure ensued. Nearly 2 weeks after the start of the captopril treatment she had transiently reduced leukocyte-count and a pruritic and pustular rash. Twenty-four days later, when on a maintenance dose of 250mg captopril, agranulocytosis was seen again. Bone-marrow cytology showed a slightly increased number of granulocytic cells and a marked left shift. Anti-leukocyte agglutinating antibodies could not be demonstrated. Since no clinical or laboratory evidence for increased SLE activities was found, drug-induced agranulocytosis was suspected. Captopril was withdrawn and prednisolone was transiently increased. Over the next 9 days Ieukocytecounts gradually returned to normal.
Captopril effectively controlled the hypertension in our patient, who had been resistant to conventional management. The agranulocytosis was probably related to captopril because of its clinical course. Agranulocytosis has not been described in any other patients taking captopril treatment in Japan. The underlying disease, renal insufficiency and hypersensitivities to drugs, were relevant in this case.

View full abstract

Two patients with lupus nephropathy developed cavitary lesion of the lung while receiving a high-dose prednisolone therapy. In the first patient, Bacillus cereus and Nocardia asteroides were isolated from materials aspirated by percuaneous needle biopsy of the lung. She was successfully treated by the antimicrobial regimen based on sensitivity studies. In the second patient, the use of trimethoprim-sulfamethoxazole proved to be highly effective, suggesting that the cavitary lesion in this case was also of infectious origin.
Cavitary lesion developing as a complication in patients with systemic lupus erythematosus is a diagnostic as well as a therapeutic challenge. The importance of definitive diagnostic measures such as lung biopsy was stressed and reports of opportunistic infections as a cause of cavitary lesion were reviewed.

View full abstract