结节病59例治疗及随访分析

Analysis of the treatment and prognosis of 59 patients with sarcoidosis

目的探讨结节病的治疗、影响预后的因素及判断病情和预后的指标。方法回顾性分析1984年1月-2003年1月北京协和医院住院治疗且随访≥2年的59例结节病临床资料。结果随访时间(61.9±49.4)个月。(1)0期患者7例,均在确诊后即口服糖皮质激素(以下简称激素)治疗,其中4例完全缓解。Ⅰ期患者22例,确诊后未予治疗的5例患者中1例进展,3例自愈,1例部分缓解;确诊后即予激素治疗的17例患者中,9例完全缓解,1例进展至Ⅱ期。Ⅱ期患者23例,1例在未予治疗3个月加重后及22例在确诊后开始激素治疗,13例完全缓解,2例进展至Ⅲ期。Ⅲ期5例患者均在确诊后即予激素治疗,1例治愈,2例进展Ⅳ期。Ⅳ期患者2例,尽管长期激素治疗,病情仍在缓慢进展。(2)Ⅰ期、Ⅱ期患者发病时的支气管肺泡灌洗液(BALF)中细胞总数和分类、CD_4/CD_8比值及血清血管紧张素转换酶(sACE)水平差异无统计学意义。(3)共有24例患者复发,其中胸内结节病20例,14例有肺外受累;未复发者中10例有肺外受累;有肺外受累与无肺外受累比较,P=0.006。(4)完全缓解者发病时 BALF 中中性粒细胞数低于尚未缓解者(P<0.001)。结论结节病病程有一定的自愈性。对Ⅰ期患者可先观察。有肺外病变的胸内结节病患者更易复发。发病时BALF 中中性粒细胞数可能作为一个预测预后的指标。

Objective To investigate the relationship between clinical characteristics, treatment and prognosis of sarcoidosis, and to analyze the factors for disease severity and prognosis. Method Retrospective analysis of 59 patients with sarcoidosis admitted to Peking Union Medical Collage Hospital from January 1984 to January 2003, with a follow-up for at least 24 months. Results The mean follow-up period was （61.9 ±49. 4）months. All of the 7 patients with stage 0 accepted glucocorticoid therapy and responded well, and 4 of them completely remitted. Among 22 cases with stage Ⅰ disease, 4 underwent spontaneous remission while another one who had extra-pulmonary sarcoidosis at the onset progressed after follow-up for 6 months. Seventeen cases were treated with glucocorticoids after the diagnosis was confirmed. Before this study, 9 patients had experienced complete remission, and 7 had been stable disease respectively, while one had progressed to stage Ⅱ. Twenty-two of the 23 stage Ⅱ patients received glucocorticoids initially and one was treated after an observation period of 3 months before deterioration. Thirteen patients of stage Ⅱ were completely cured, and 2 were still progressing under the drug therapy. All of the 5 patients in stage Ⅲ accepted glucocorticoids after diagnosis. One of the 5 patients has resolved completely and 2 had progressed into stage Ⅳ. The interstitial changes of the lung in 2 stage Ⅳ patients had been progressing even though the treatment lasted for years. There were no significant differences in the serum angiotensin converting enzyme （ACE） level, total broncho-alveolar fluid （BALF） cell count, percentage of alveolar lymphoeytes, neutrophils and lymphocyte CD4/CD8 ratio at the onset between stage Ⅰ and stage Ⅱ diseases. Age, gender, presence of respiratory symptom , serum ACE level, total BALF cell count, percentage of alveolar lymphoeytes ,neutrophils or lymphocyte CD4/CD8 ratio at the onset were not associated with relapse. 14 cases relapsing pulmonary sareoidosis had extra-pulmonary lesions, and there was a dose correlation between the relapse and extra-pulmonary lesions （P = 0. 006 ）. A significant increase in the percentage of BALF neutrophils （8. 37 ± 3.55 ）% at the time of diagnosis was observed in patients who had not completely resolved as compare with the patients who had recovered [ （ 3. 52 ± 2. 37 ） %, P 〈 0. 001 ]. Conclusion The outcome of sareoidosis are generally good. Patients with extra-pulmonary sareoidosis are more likely to experience relapse. Stage I disease should receive observation before glucocorticoid therapy. Increased percentage of neutrophils in BALF at the onset is possibly associated with a progressive or persistent disease.