Sugisaki Katsunori, Yamaguchi Tetsuo, Nagai Sonoko, Ohmiti Mitsuhide, Takenaka Sinobu, Morimoto Sin'ichiro, Ishihara Mami, Tachibana Teruo, Tsuda Tomiyasu
Division of Pulmonary Disease, Department of Allergy and Immunology, Oita Medical University, Hazama-machi, Japan.
Sarcoidosis Vasc Diffuse Lung Dis. 2003 Oct;20(3):222-6.
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%.
为获取接受口服糖皮质激素治疗的结节病患者的临床数据，向日本各地的46家医院发放了调查问卷。女性患者数量多于男性患者（比例为1.5:1），平均年龄为44.9±16.5岁，发病高峰在20岁以及50至60岁。疾病活动标志物在血清或支气管肺泡灌洗液（BALF）中水平较高：具体而言，血清血管紧张素转换酶（sACE）为27.9±31.9 IU/ml（正常范围<21.4），CD4/CD8淋巴细胞比值为6.5±5.7。眼部受累是全身使用类固醇治疗最常见的原因，其次依次为肺部和心脏受累。类固醇治疗的主要原因是眼部症状加重、视力障碍、呼吸道症状（如咳嗽或劳力性呼吸困难）、胸部X线表现进展、心力衰竭以及严重心律失常（如房室传导阻滞）。初始糖皮质激素剂量通常为每日30 mg泼尼松龙，但对于一些难治性病例，使用的剂量为每日40 - 60 mg。少数对类固醇反应不佳的患者还使用了免疫抑制药物，如甲氨蝶呤。总体而言，70 - 80%接受类固醇治疗的患者对药物有良好的临床反应，但患有心脏疾病的患者，反应率仅为48%。
