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.

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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.

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自己免疫疾患患者のNK活性につき広く,検討された報告はない. 20名の女性SLE患者の末梢リンパ球によるNK活性を観察したところ(ステロイド未治療群13.0±3.2%,ステロイド治療群13.6±10.2%),性,年齢を合致させた対照群(34.0±7.5%)に比べ,有意に(P<0.01)低値を示した.健常人リンパ球を,抗リンパ球抗体を含むSLE血清とウサギ補体で低温処理し,生き残ったリンパ球のNK活性を観察すると,顕著な低下を招いた.また,熱変性IgGをNK活性測定系に加えた際にも,著しいNK活性の抑制を認めた.
こうした点から, SLE患者のNK活性の低下の一因として,血清中の抗リンパ球抗体ならびに,免疫複合体などが関与していることが推定された.

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活性化Tリンパ球から産生される一種のリンホカイン,催胆汁うっ滞因子をヒトから得るため,肺結核症患者の末梢血リンパ球をin vitroで精製ツベルクリ(PPD)で刺激し,その細胞培養上清を分離して催胆汁うっ滞因子の存在を検討した.その結果,本因子の存在が確認されたので,細胞培養上清をSephadex G-75およびDEAE-celluloseを用いるカラムクロマトグラフィーで分画し,催胆汁うっ滞因子がモルモットリンパ節細胞由来の因子と同じ分画に認められることを見出した.
これらの結果から,ヒトにおいてもリンホカインの一種,催胆汁うっ滞因子が形成されること,その因子が肝内胆汁うっ滞をひきおこす可能性があると考えられる.

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ヒトリンパ球のin vitro免疫能に及ぼす溶連菌製剤(OK-432)の影響について, 1) natural killer (NK)細胞活性, 2) cultured spontaneous cell mediated cytotoxicity (CSCMC)活性, 3)免疫グロブリン産生能, 4) DNA合成,に及ぼす影響を検討した.その結果, OK-432は培養リンパ球による細胞障害活性(CSCMC)を高めることがわかった. CSCMCのエフェクター細胞はナイロンウール非付着性でEn(-)のリンパ球にその主たる活性がみられた.またOK-432をヒトリンパ球培養に添加すると, IgG, IgA, IgMの産生増強をおこした.この免疫グロブリン産生はT細胞依存性であり,そのT細胞はヒト組織適合抗原を越えて作用した.さらに, OK-432はPWMに匹敵するほどのリンパ球増殖能を有することがわかった.

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Krantzの方法を用いて, RA患者骨髄赤芽球のヘム合成に及ぼすanti-thymocyte globulin (ATG)の影響を検討した.
骨髄細胞をATGで60分間前処置したのち,骨髄赤芽球1×10⁶個を非働化した20%ヒトAB型血漿pH7.4のNCTC 109 1.0ml中に,エリトロポエチン1単位と放射性鉄1μCiとともに, 37°C 5% CO₂で72時間の培養を行った,その後,ヘム中に取り込まれた放射性鉄をTealeの方法で抽出し,γシンチレーターで測定した.結果:
1) RA患者骨髄赤芽球のヘム合成は,正常ヒトや鉄欠乏性貧血患者に比べ低下していた.
2) RA患者血漿は,正常ヒト骨髄赤芽球のヘム合成に影響をあたえなかった.
3) 骨髄細胞をATGで前処置したRA患者骨髄赤芽球のヘム合成は明らかに改善した.
以上の結果は, RA患者骨髄リンパ球は, RAの貧血の原因に関与している可能性を示唆するものと思われた.

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SLE血清のCon Aにより誘導したsuppressor T-cell機能に及ぼす影響をin vitroの免疫グロブリン合成系を用いて調べた. Tγに特異的なIgGの抗リンパ球抗体(antilymphocyte antibody, ALA)は,著明にCon A誘導suppressor T-cell機能を抑制したが,一方, IgM Tγ specific ALAならびにTγ (-) specific ALAのどの免疫グロブリンクラスもIgG Tγ specific ALAに比して, Con A誘導suppressor T-cell機能を抑制する活性は弱かった.
抗リンパ球抗体以外のSLEの血清中に存在する因子で, suppressor機能に影響を与えうる因子についても検討を加えた.変性ヒトIgGをimmune complexの代用として使用すると, Con A誘導suppressor T-cell機能はdose dependentに抑制されたが,その程度はIgG Tγ specific ALAに比べるとはるかに弱かった.副腎皮質ホルモンも強力にCon A誘導suppressor T-cell活性を抑えたが, Sephadex G 200カラムによるゲル濾過では,阻止活性はIgG (7S)分画にみられ,また,抗ヒトIgGのaffinity columnによりIgGを血清中より除去すると阻止活性が失われた.これらの結果から,活動期のSLEの血清中に含まれるCon A誘導suppressor T-cell阻止活性は主としてIgG型のTγにspecificな抗リンパ球抗体のためであると考えられた.

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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.

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ループス腎炎に対するprednisolone大量投与中に,空洞形成性病変を合併した全身性エリテマトーデスの2症例を報告した.うち1例では,経皮肺針生検によりNocardia asteroidesとBacillus cereusの2種を分離・同定し,その感受性検査成績に基づいた抗菌剤投与により治療に成功した.他の1例も,第1例における経験を生かし, trimethoprimsulfamethoxazoleの単独使用により治癒した.全身性エリテマトーデスに合併する空洞形成性肺病変の鑑別診断およびopportunistic infectionについて考察した.

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