Sugo A, Seyama K, Yaguchi T, Noto K, Kira S, Yamaguchi H
Department of Cardiology, Juntendo University, School of Medicine, Tokyo, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1995 Oct;33(10):1111-8.
In 1992, a 49-year-old woman was admitted to the hospital because of exertional dyspnea. Three years earlier sarcoidosis had been diagnosed, and the patient was found to have bilateral hilar lymphadenopathy. The eye, skin, and knee joint were also involved. During the second hospital stay, atrial flutter with advanced A-V nodal block, scattered defects on a 201T1 scintigram, and marked cardiomegaly on chest roentgenogram led to the diagnosis of cardiac sarcoidosis. Signs and symptoms of cardiac failure subsided after placement of an artificial cardiac pacemaker, but the patient still complained of mild muscle weakness in the lower extremities on exertion. 67Ga scintigraphy revealed marked accumulation in the lower extremities, and muscle biopsy of the left gastrocnemius revealed numerous epithelioid cell granulomas with muscle fiber degeneration. Oral corticosteroid therapy was effective. A review of the 24 cases of sarcoid myopathy reported in Japan indicated that the male-to-female ratio is 1:3.8. As compared to patients in whom myopathy led to the diagnosis of sarcoidosis, those in whom myopathy developed after sarcoidosis was diagnosed were (1) relatively older, (2) more likely to have multiple organ involvement, and (3) more likely to have cardiac sarcoidosis. Corticosteroids were beneficial in about three quarters of these 16 cases, who received corticosteroid therapy.
1992年,一名49岁女性因劳力性呼吸困难入院。三年前被诊断为结节病,患者存在双侧肺门淋巴结肿大,眼、皮肤和膝关节也受累。在第二次住院期间,出现心房扑动伴高度房室传导阻滞、201Tl闪烁扫描有散在缺损以及胸部X线片显示明显心脏扩大,从而诊断为心脏结节病。植入人工心脏起搏器后心力衰竭的症状和体征消退,但患者仍主诉劳力时下肢轻度肌无力。67Ga闪烁扫描显示下肢有明显放射性浓聚,左腓肠肌肌肉活检发现大量上皮样细胞肉芽肿伴肌纤维变性。口服糖皮质激素治疗有效。对日本报道的24例结节病性肌病病例的回顾表明,男女比例为1:3.8。与因肌病而诊断为结节病的患者相比,在结节病诊断后发生肌病的患者(1)年龄相对较大,(2)更易有多器官受累,(3)更易有心脏结节病。在接受糖皮质激素治疗的这16例患者中,约四分之三的患者使用糖皮质激素有益。