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広範な解離性胸腹部大動脈瘤の発症をみたMarfan症候群の1例を発端者とし,常染色体優性遺伝により伝播したMarfan症候群の多発家系を発見した.発端者は35才,女性で,解離性大動脈瘤,水晶体脱臼, arachnodactylyをはじめとする様々な骨格異常を有していた.さらに家系調査により一卵性双生児例や,幼児のMarfan症候群例をも発見しえた.本症候群患者の家系調査は症状の顕性化していない患者を発見する可能性があり,合併症に対する早期の対策の上からも重要と思われる.また,本症候群の一卵性双生児例は本邦第1例目と思われ,文献的考察を加え報告する.

A 57-year-old female suffered from Hashimoto's thyroiditis since 1959. When she was hospitalized in Nov. 1979 for acute bronchitis, her home doctor pointed out her sera showed marked hypergammaglobulinemia. She was referred to our hospital for further examination. Her case was diagnosed as multiple myeloma according to the findings; increasement of IgG1, k monoclonal gammaglobulin, increasement of immature plasmacytes in bone marrow and multiple osteolytic lesions in the skull. Her sera also showed anti-microsome antibody activity at a dilution of 1: 25, 600.The monoclonal protein was purified from her sera to testify the presence of anti-microsome antibody activity. The results were as follows.1) Patient's purified IgG, F (ab')2 and light chain kappa F (ab')2 showed anti-microsome antibody activity at a dilution of 1: 102, 400, 1: 102, 400 and 1: 25, 600 respectively. However, light chain lambda F (ab')2 did not show this antibody activity.2) Anti-idiotypic antybody was prepared by immunizing rabbit with F (ab')2 fragment of patient's IgG followed by absorption with IgG of Cohn's fraction II. This anti-idiotypic antibody binded only F (ab')2 of patient's IgG but not IgG of Cohn's fraction II. This anti-idiotypic antibody inhibited the binding between the monoclonal protein and microsome antigen.These results suggested that patient's monoclonal protein was the antibody to microsome.This is the first case reported in the literature whose serum monoclonal protein has been proved to be anti-microsome antibody.

A seventy-two-year old female had chest pain since October 1981. In July 1982, she was admitted for congestive heart failure. In October 1982, compression fractures of vertebrae on Th12 and L1, and Bence Jones protein in the urine were found. She was transferred to our hospital for further examination about multiple myeloma in November 1982. Her laboratory data included mild normocytic normochromic anemia, leukocytes 9, 600/cmm, plasma cells 34% in peripheral blood, total protein 4.5g/dl, decreased all classes of immunoglobulins, BUN 99mg/dl, and creatinine 6.9mg/dl. Her serum and urine showed M-bow only against anti-λ antiserum in immunoelectrophoresis. She was diagnosed as Bence Jones λ type plasma cell leukemia associated with renal failure. Her renal function was gradually improved after chemotherapy and plasmapheresis were started. After a month her renal function became worse, but the response to further treatments including chemotherapy and plasmapheresis could not be obtained. She was died of renal failure on 19th December 1982. In autopsy specimen, many tubular casts and Bence Jones λ type proteins in tubular epithelial cells were found. Bence Jones proteins were purified from patient's urine by ammonium sulfate precipitation method followed by DEAE column chromatography. This purified λ type Bence Jones proteins were used as a standard marker in the measurement of λ type free light chains in the patient's serum. Patient's serum free light chains were serially measured during her clinical course, and were markedly decreased after initial treatment. We discussed about the negative role of Bence Jones proteins on renal function in multiple myeloma. Plasmapheresis treatment along with chemotherapy shows beneficial effect on renal function mainly by removing free light chains from the serum in the patient with Bence Jones type multiple myeloma.

自己免疫性溶血性貧血(AIHA)とBasedow病との合併例を経験したので報告する.患者は28才の女性で,高熱,動悸,全身倦怠感,軽度の黄痩のため入院した.クームス試験陽性.ハプトグロビン消失,赤血球形態正常であるためAIHAと診断した.また,甲状腺機能亢進症状があり.123I摂取率高値, TRHテストにてTSHが無反応, T3抑制試験にて1231摂取率抑制されず,さらにTSH受容体抗体(TBII)を認め, Basedow病と診断した.赤血球自己抗体は,温式の血液型特異性をもたないIgGκ型の抗体が検出されたほか,冷式抗体の存在も疑われた. AIHAとBasedow病の確実な合併例は文献上本例が3例目で,両疾患の免疫学的な関係について考察を加えた。

SLEの発症をみた二組の一卵性双生児例を経験した.一組は20才の姉妹例で,両者ともSLEを発症している.両者の間では発症時期の違いの他,免疫学的検査等の一部に不一致を認めたが,両者の間の環境因子に特に違いはなく,日光曝露が発症時期に影響を与えたと考えられた.もう一組は22才の兄弟例で,兄はSLEであつたが,弟はBFP以外は特記すべきものがなかつた.この両者も環境因子は同等であつた.以上の二組の血縁者調査では,姉妹例では,他に免疫学的異常を認める者はなかつたが,兄弟例では,他に抗核抗体弱陽性者を認め,父方のHLAハプロタイプが一致していた.この両組について, MLC,免疫グロブリン産生能, ALAについて検討した.

当科126例のSLE群と, 70例の非SLEリウマチ疾患群においてARA新旧両基準について検討した. SLE群の臨床症状では関節炎90%,蝶形紅斑62%が多く, Tanらの報告と比較すると新基準では漿膜炎が低頻度である以外に有意差はなかつた. ARA新基準に加えられた項目では,抗核抗体99%,抗DNA抗体83%と高かつた. Sm抗体は陽性率26%であるがLE細胞やBFPと同様に特異度はきわめて高かつた. ARA基準の平均陽性項目数はSLE群では旧基準5.40項目,新基準5.61項目であり, 4項目以上陽性症例の割合はそれぞれ88.1%, 96.8%と有意に増加が認められた.対照群の特異度は98.6%から95.7%と減少したが有意差はなく,新基準は感度に勝ることが示された.

目的:超音波パルスドプラー法(以下PDE)を用い,当科全身性エリテマトーデス(以下SLE)患者を対象に肺高血圧症(PH)の検索を行い,臨床検査所見について比較検討した.方法:超音波断層法とPDEの複合システムを用い,右室流出路又は肺動脈弁口部での血流速度パターンを表示しPH patternを検討した.結果: SLE 42例中7例にPH patternが認められ,この群ではRaynaud現象,抗RNP抗体,血小板減少が高頻度に認められた.