嗄声で発症した心サルコイドーシスの1剖検例

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1 嗄声で発症した心サルコイドーシスの 1 剖検例 1) 2) 1) 1) 1) 1) 1) 3) 4) 1) 要旨 Thallium キーワード : An Autopsy Case of Cardiac Sarcoidosis Presenting with Hoarseness Takako Kawai 1), Kiyoshi Katoh 2), Haruhiro Saito 1), Takeshi Takamura 1), Akiko Okouchi 1), Koichi Yamanaka 1), Masataka Shoda 1), Eriko Matsumura 3), Takaaki Ito 4), Masao Ishii 1) ABSTRACT A 54-year-old Japanese woman, who had been diagnosed as sarcoidosis, was referred to our hospital in July She noticed hoarseness in March Laryngeal biopsy failed to clarify the cause of hoarseness. Diagnosis of sarcoidosis was made in September by mediastinal lymph node biopsy. She developed chest pain in August 1990 and cardiovascular examinations were performed. However, there were no abnormalities in electrocardiography and echocardiography. In March 1992, corticosteroid therapy was started because of the enlargement of cardiac silhoutte. She developed frequent premature ventricular contractions and chronic heart failure and was hospitalized in July Severe left ventricular hypokinesia, marked decrease in LVEF and defect in 201 Thallium myocardial scintigraphy were shown. She was discharged in December after the iniation of digitalis, diuretic drugs, and antiarrhythmic drugs. She was hospitalized in July 1999 for severe heart failure and died in August. The autopsy revealed noncaseating epithelioid cell granulomas in mediastinal lymph nodes. In the heart muscle, fibrosis and granulomatous changes were observed. keywords ; Cardiac sarcoidosis, Heart failure, Hoarseness 1) 横浜船員保険病院内科 2) 横浜市立大学医学部附属病院医療情報部 3) 同中央検査部 4) 同第 1 病理学教室著者連絡先 : 川井孝子横浜船員保険病院横浜市保土ヶ谷区釜台町 43-1 TEL FAX ) Yokohama Seamen s Insurance Hospital, Department of Internal Medicine 2) Yokohama City University Hospital, Department of Medical Informatics 3) Yokohama City University Hospital, Department of Laboratory Medicine 4) Yokohama City University Hospital, First Department of Pathology 45

日サ会誌 2000,20(1) はじめに 1) 2) 3) 症例呈示患者 54 主訴職業既往歴家族歴飲酒歴喫煙歴現病歴 3 50 4 X CTR 45% BHL 5 9 ACE 21.

0 110/58mmHg 72/ GOT 53U/l GPT 57U/l LDH 666U/l CRP ACE 6.9U/l T CD4/CD8 3.

2 日サ会誌 2000,20(1) はじめに 1) 2) 3) 症例呈示患者 54 主訴職業既往歴家族歴飲酒歴喫煙歴現病歴 X CTR 45% BHL 5 9 ACE 21.2U/ l CTR 56% CTR 61% 40mg/ CTR PVC 7 入院時現症 153cm 49.5Kg /58mmHg 72/ GOT 53U/l GPT 57U/l LDH 666U/l CRP ACE 6.9U/l T CD4/CD X Figure 1 CTR 62% BHL PVC LVDd 70mm LVDs 63mm LVEF 27% 201 Thallium 201 Tl Figure 2 Figure 1 Chest X ray film on admission in July Cardiothoracic ratio is 62%. Figure 2 201Tl-myocardial scintigraphy revealed reduced uptake in anteroseptal and apex area. 46

経過 PVC 0.125mg/ 4 PVC PVC 0.0625mg/ 40mg/ 20mg/ CTR 57 15mg/ 12 16 5 3 Th12 Th4 Th5 L2 7.5mg/ CTR 11 7 7 31 再入院時現症 35.

0U/l 450pg/ml 40 X Figure 3 CTR 72% (Figure 4) V 5 V 6 q PVC LVDd 84 mm LVDs 74mm LVEF 25% Figure 3 Chest X ray film on admission

3 経過 PVC 0.125mg/ 4 PVC PVC mg/ 40mg/ 20mg/ CTR 57 15mg/ Th12 Th4 Th5 L2 7.5mg/ CTR 再入院時現症 /70mmHg 92/ III IV 1 再入院時検査所見 CRP 2.4mg/dl ACE 12.0U/l 450pg/ml 40 X Figure 3 CTR 72% (Figure 4) V 5 V 6 q PVC LVDd 84 mm LVDs 74mm LVEF 25% Figure 3 Chest X ray film on admission in July 1999, showing marked cardiomegaly and pulmonary congestion. Figure 4 Electrocardiogram on admission in July 1999, showing right bundle branch block, left axis deviation, and abnormal q waves in leads V5, V6. 47

日サ会誌 2000,20(1) Figure 5 Clinical course of the patient.

剖検所見両肺門傍気管頚部および後腹膜にリンパ節の腫脹を認め類上皮細胞肉芽腫を認めた Figure 6 心筋は主に側壁から後壁にかけて線維化が拡がっており Figure 7

Photomicrograph of autopsy specimen from the heart, showing myocardial fibrosis (hematoxylin and eosin

Figure 6 Photomicrograph of autopsy specimen from mediastinal lymph node, showing granulomatous

4 日サ会誌 2000,20(1) Figure 5 Clinical course of the patient. 再入院後経過利尿剤の静脈内投与にも反応せず 8月2 日より心室性頻拍 VT が出現塩酸リドカインの持続点滴静注を行い VTは消失したが 8月 12日より血圧が低下し 14日死亡された Figure 5 剖検所見両肺門傍気管頚部および後腹膜にリンパ節の腫脹を認め類上皮細胞肉芽腫を認めた Figure 6 心筋は主に側壁から後壁にかけて線維化が拡がっており Figure 7 一部不完全ながら肉芽腫と思われる部位を認めた Figure 8 肺肝にはうっ血性の変化を認めた冠動脈には軽度の粥状硬化性の変化が認められた喉頭に腫瘤を認めなかった Figure 7 Photomicrograph of autopsy specimen from the heart, showing myocardial fibrosis (hematoxylin and eosin stain; original magnification 10). Figure 6 Photomicrograph of autopsy specimen from mediastinal lymph node, showing granulomatous lymphadenitis (hematoxylin and eosin stain; original magnification 50). 48 Figure 8 Photomicrograph of autopsy specimen from the heart, showing myocardial fibrosis and granulomatous change (arrow) (hematoxylin and eosin stain; original magnification 50).

5 考察 Lown Tl 4) ) 7 5 DCM 5) 6) 1) 7) ACE DCM 8) 3) ADL 4 CTR 4) 結論引用文献 1) 矢崎善一, 関口守衛 : 心サ症重症化の要因と心不全治療の問題点. サルコイドーシス / 肉芽腫性疾患 1999; 19: ) Iwai K., Sekiguti M, Hoshoda Y et al: racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis 1994; 11: ) 関口守衛, 三井富士夫 : 各種疾患病態にみられる心血管血圧異常心臓サルコイドーシス. 別冊日本臨牀循環器症候群 IV 1996; ) 平賀洋明, 廣江道昭, 岩井和郎, 他 : 心臓サルコイドーシス診断の手引き作成の経過について. 厚生省特定疾患びまん性肺疾患調査研究班平成 4 年度報告集 1993; ) Bower JS, Belen JE, Weg JG et al: Manifestations and treatment of laryngeal sarcoidosis. Am Rev Respir Dis 1980; 122: ) 小川ゆかり, 樋口英一, 古賀英之, 他 : 舌咽迷走神経不全麻痺により, 嗄声, 嚥下障害を認めたサルコイドーシスの1 症例. 日胸疾会誌 1994; 32: ) 加藤晴通, 森下宗彦, 沖良生他 : ステロイドに反応せずメソトレキセート追加投与により改善した心サ症の一例. サルコイドーシス / 肉芽腫性疾患 1999; 19: ) Waagstein F, Caidahl K, Wallentin I et al: Long term beta blockade in dilated cardiomyopathy. Effects of short and long term metoprolol treatment followed by withdrawal and readministration of metoprolol. Circulation 1989; 80:

8 The Bulletin of Meiji University of Integrative Medicine API II 61 ASO X 11 7 X-4 6 X m 5 X-2 4 X 3 9 X 11 7 API 0.84 ASO X 1 1 MR-angio

8 The Bulletin of Meiji University of Integrative Medicine API II 61 ASO X 11 7 X-4 6 X m 5 X-2 4 X 3 9 X 11 7 API 0.84 ASO X 1 1 MR-angio 7-14 2010 1 1 1 2 1 1 1 2 Fontaine II ASO61 3 API ASO ASO ASO API API KKKKKKKKKK ASO Fontaine II API Received April 14, 2009; Accepted July 16, 2009 I arteriosclerosis obliterans: ASO ASO 50 70 1,2 Fontaine