森本 紳一郎

Questionnaires were sent to 46 hospitals of all over Japan in order to obtain the clinical data on sarcoidosis patients who were treated with oral corticosteroids. The number of female patients was greater than that of male patients (1.5:1), and the average age was 44.9 +/- 16.5 with peaks at 20 and at 50 to 60. The markers of disease activity were high in serum or bronchoalveolar lavage fluids (BALF): specifically, the serum angiotensin-converting enzyme (sACE) was 27.9 +/- 31.9 IU/ml (n.v. < 21.4), and the CD4/CD8 lymphocyte ratio was 6.5 +/- 5.7. Eye involvement was the most common reason for systemic steroid therapy, followed in order by lung and heart involvement. The main reasons for steroid therapy were the exacerbation of ocular symptoms, visual disturbance, respiratory symptoms, such as cough or exertional dyspnea, progression of chest radiographic findings, heart failure and severe arrhythmia, such as AV block. The initial corticosteroid dose was usually 30 mg of predinisolone per day, but for some refractory cases, a 40-60 mg per day was used. Immunosuppressive drugs, such as methotrexate, were also used in the small number of patients who responded poorly to the steroid. Overall, a good clinical response to the drug was found in 70-80% of the steroid treated patients, but in those with cardiac disease, the response rate was only 48%.

In many cases, the diagnosis of eosinophilic myocarditis is suggested by an elevated peripheral blood eosinophil count. However, no detailed studies have been performed on the sequential changes in the initial peripheral blood eosinophil count over the course of the disease. We measured the peripheral blood eosinophil count at the time of presentation in eight patients with eosinophilic myocarditis proven by endomyocardial biopsy and intermittently thereafter. The eosinophil count at the time of onset was <500/mm(3) in four patients, >500/mm(3) but <1000/mm(3) in three patients, and greater than or equal to1000/mm(3) in one patient. In three of the four patients with an initial eosinophil count of <500/mm(3), an increase to greater than or equal to500/mm(3) occurred 7-12 days after the onset. The remaining patient did not develop peripheral eosinophilia. In conclusion, in the early stage of eosinophilic myocarditis, peripheral hypereosinophilia is not present initially in some patients, and may not develop during the course of the illness in a subset of these patients.

In many cases, the diagnosis of eosinophilic myocarditis is suggested by an elevated peripheral blood eosinophil count. However, no detailed studies have been performed on the sequential changes in the initial peripheral blood eosinophil count over the course of the disease. We measured the peripheral blood eosinophil count at the time of presentation in eight patients with eosinophilic myocarditis proven by endomyocardial biopsy and intermittently thereafter. The eosinophil count at the time of onset was <500/mm(3) in four patients, >500/mm(3) but <1000/mm(3) in three patients, and greater than or equal to1000/mm(3) in one patient. In three of the four patients with an initial eosinophil count of <500/mm(3), an increase to greater than or equal to500/mm(3) occurred 7-12 days after the onset. The remaining patient did not develop peripheral eosinophilia. In conclusion, in the early stage of eosinophilic myocarditis, peripheral hypereosinophilia is not present initially in some patients, and may not develop during the course of the illness in a subset of these patients.

Background: The usefulness of corticosteroid therapy for cardiac sarcoidosis has not yet been fully clarified. Methods: Of 40 patients diagnosed with cardiac sarcoidosis, twenty patients complicated by atrioventricular block but normal cardiac function (left ventricular ejection fraction greater than or equal to50%) were divided retrospectively into one group (n = 7) receiving corticosteroids and another (n=13) not receiving these agents. Over a mean observation period of 79.4 +/- 39.9 months, long-term outcome and laboratory findings were compared between the two groups and side effects also were noted. Results: There were no deaths in the corticosteroid-treated group. In the untreated group, 2 patients died (15.4%). Atrioventricular block resolved in 4 of the 7 patients in the treated group (57.1%), but did not resolve or improve in any of the untreated patients (p < 0.05). Left ventricular ejection fraction did not differ significantly between the treated and untreated groups at the time of initial evaluation (66.7 +/- 6.5% vs. 60.5 +/- 6.4%). In the follow-up period, a marked decline in the ejection fraction had occurred in the untreated group (37.6 +/- 17.3%), but not in the treated group (62.1 +/- 4.4%; p < 0.005). Ventricular tachycardia was not present at the initial assessment in any patient in either group. In the follow-up period, ventricular tachycardia occurred in only 1 of 7 treated patients (14.3%), but was present in 8 of 13 untreated patients (61.5%; p<0.05). However, side effects of corticosteroid therapy were noted in 6 of the 7 treated patients (85.7%). Conclusion: Our findings suggest that corticosteroids are useful in the treatment of cardiac sarcoidosis complicated by atrioventricular block but with normal cardiac function. However, these agents must be used with caution, with the maintenance dose kept as low as possible.

Background: The usefulness of corticosteroid therapy for cardiac sarcoidosis has not yet been fully clarified. Methods: Of 40 patients diagnosed with cardiac sarcoidosis, twenty patients complicated by atrioventricular block but normal cardiac function (left ventricular ejection fraction greater than or equal to50%) were divided retrospectively into one group (n = 7) receiving corticosteroids and another (n=13) not receiving these agents. Over a mean observation period of 79.4 +/- 39.9 months, long-term outcome and laboratory findings were compared between the two groups and side effects also were noted. Results: There were no deaths in the corticosteroid-treated group. In the untreated group, 2 patients died (15.4%). Atrioventricular block resolved in 4 of the 7 patients in the treated group (57.1%), but did not resolve or improve in any of the untreated patients (p < 0.05). Left ventricular ejection fraction did not differ significantly between the treated and untreated groups at the time of initial evaluation (66.7 +/- 6.5% vs. 60.5 +/- 6.4%). In the follow-up period, a marked decline in the ejection fraction had occurred in the untreated group (37.6 +/- 17.3%), but not in the treated group (62.1 +/- 4.4%; p < 0.005). Ventricular tachycardia was not present at the initial assessment in any patient in either group. In the follow-up period, ventricular tachycardia occurred in only 1 of 7 treated patients (14.3%), but was present in 8 of 13 untreated patients (61.5%; p<0.05). However, side effects of corticosteroid therapy were noted in 6 of the 7 treated patients (85.7%). Conclusion: Our findings suggest that corticosteroids are useful in the treatment of cardiac sarcoidosis complicated by atrioventricular block but with normal cardiac function. However, these agents must be used with caution, with the maintenance dose kept as low as possible.

副腎皮質ステロイド剤による重大な副作用のために, メトトレキサート療法への変更を行った心臓サルコイドーシスの4例について, 本剤の有用性を検討した. メトトレキサートによる副作用で再生不良性貧血が1例, 肝機能障害が2例で生じた. 再生不良性貧血については, 本剤を中止し顆粒球コロニー形成刺激因子 (G-CSF; granulocyte-colony stimulating factor) filgrastimを用いたところ, 速やかに改善した. 肝障害の2例については本剤を減量せざるを得なかった. 残り1例については, 明らかな副作用は生じなかったものの, 本剤への変更によりサルコイドーシスの活動性が再燃し, 再び副腎皮質ステロイド剤に変更せざるを得なかった. 心サルコイドーシスにおけるメトトレキサート療法の経験の蓄積が待たれるところであるが, 少なくとも, 今回経験した4例のうち3例に関して, 本治療は有用とは言えなかった.

急性心筋炎では,心原性ショックにおちいる症例が存在する.しかし,一体いかなる時期にいかなる症例が,心原性ショックにおちいるのかという点については明らかにされていない.今回,前述の問題点を明らかにしえたので報告する.<BR>心筋生検により,組織学的に診断された急性リンパ球性心筋炎47例中,入院時にすでに心原性ショックにおちいっていた8例を除く39例を対象とした.39例を,入院後にショックにおちいり第2～6病日に大動脈内バルーンパンピングあるいは経皮的心肺補助の補助循環を必要とした劇症群12例(30.8%)と,補助循環を必要としなかった非劇症群27例(69.2%)に分け,両群間における入院直後の臨床検査所見を比較検討した.また,劇症群12例において,ショックの発生時期とその増悪因子についてもあわせて検討した.<BR>劇症群は非劇症群に比較して年齢が高く(57.8±11.6,40.7±17.3歳,p<0.01),脈拍(110.9±29.0,86.5±26.9歳,p<0.05),C反応性蛋白(7.0±7.0,2.3±2.2mg/dl,p<0.01)およびクレアチンキナーゼ(1,147.1±876.2,594.6±568.7IU/l,p<0.05)も高値を示した.心電図では,劇症群において心室内伝導障害の合併(9/12,7/27例,p<0.01)が高頻度で,心エコー図では劇症群の左室駆出率(40.7±13.9,50.1±10.6%,p<0.05)が低下していた.また,ショックの発生時期は入院後1.8±1.2日であった.劇症群12例中7例(58.3%)では,心室頻拍あるいは完全房室ブロックなどの不整脈が誘因となってショックにおちいったことが明らかとなった.<BR>急性リンパ球性心筋炎では,入院後に心原性ショックにおちいる症例が約30%存在する.入院時に頻脈あるいはC反応性蛋白,クレアチンキナーゼの高値,心室内伝導障害合併,左室駆出率低下を示す症例が劇症化しやすいことが明らかとなった.また,その発症時期は入院後平均1.8日であり,増悪因子の一つとして不整脈の関与が示された.