Hsieh Chia-Wei, Chen Der-Yuan, Lan Joung-Liang
Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taichung Veterans General Hospital and National Yang-Ming University, Taipei, Taiwan.
J Formos Med Assoc. 2006 Apr;105(4):269-76. doi: 10.1016/S0929-6646(09)60117-0.
Sarcoidosis is still considered a rare multisystem disorder in Taiwan, and data on the disease course and outcome are limited. We analyzed the clinical manifestations, disease course and complications in Taiwanese patients with sarcoidosis.
A retrospective cohort design was used. Fifty-six patients with sarcoidosis diagnosed between 1985 and 2004 were included. Their clinical features, laboratory findings at initial presentation, disease course, and complications were analyzed.
Forty-three patients (76.8%) were female. The mean age at symptom onset was 47 years. The most common clinical symptoms were pulmonary (82.1%), cutaneous (23.2%), ophthalmic (19.6%), and articular (17.8%). Only two patients presented with Löfgren's syndrome. There was a seasonal variation in disease onset, with higher incidence in winter and early spring. No advanced pulmonary involvement was noted. Elevated levels of serum angiotensin converting enzyme (sACE) were found in 72.5% (29/40) of patients with active sarcoidosis, and significantly higher levels of sACE were found in patients with lung involvement (27.98+/-1.71 IU/L vs. 18.2+/-2.76 IU/L; p<0.01). In 50% (20/40) of patients, sACE levels declined significantly in parallel with clinical remission (24.75+/-1.53 IU/L vs. 16.33+/-1.21 IU/L; p<0.05). Spontaneous complete remission was found in 20.7% of patients, whereas 39.6% of patients with multiple extrapulmonary involvement responded poorly to intensive corticosteroids plus various immunosuppressants.
In this series, the mean age of disease onset was in middle age (mean, 47 years old), there was a low incidence of Löfgren's syndrome (3.6%), and no patients had advanced pulmonary syndrome. The results of this study also suggest that sACE might be a marker of pulmonary involvement that is also useful in monitoring disease activity.
结节病在台湾仍被视为一种罕见的多系统疾病，关于该病病程及转归的数据有限。我们分析了台湾结节病患者的临床表现、病程及并发症。
采用回顾性队列研究设计。纳入1985年至2004年间确诊的56例结节病患者。分析其临床特征、初次就诊时的实验室检查结果、病程及并发症。
43例患者（76.8%）为女性。症状出现时的平均年龄为47岁。最常见的临床症状为肺部症状（82.1%）、皮肤症状（23.2%）、眼部症状（19.6%）及关节症状（17.8%）。仅2例患者出现 Löfgren 综合征。疾病发病存在季节性变化，冬季和早春发病率较高。未发现晚期肺部受累情况。72.5%（29/40）的活动期结节病患者血清血管紧张素转换酶（sACE）水平升高，肺部受累患者的 sACE 水平显著更高（27.98±1.71 IU/L 对 18.2±2.76 IU/L；p<0.01）。50%（20/40）的患者中，sACE 水平随临床缓解而显著下降（24.75±1.53 IU/L 对 16.33±1.21 IU/L；p<0.05）。20.7%的患者出现自发完全缓解，而39.6%的多系统肺外受累患者对强化糖皮质激素加多种免疫抑制剂反应不佳。
在本系列研究中，疾病发病的平均年龄为中年（平均47岁），Löfgren 综合征发病率较低（3.6%），且无患者出现晚期肺部综合征。本研究结果还表明，sACE 可能是肺部受累的标志物，也有助于监测疾病活动。
