胸内结节病与全身多脏器结节病的临床对比分析

A comparative clinical analysis of thoracic sarcoidosis and multi-organ sarcoidosis

目的探讨胸内结节病和全身多脏器结节病的临床特点。方法回顾性分析1995—2010年北京同仁医院经组织病理学确诊的24例胸内结节病（胸内结节病组）和29例全身多脏器结节病（全身多脏器结节病组）的人口统计学资料、临床表现与诊断情况、器官受累状况、血清ACE水平、肺功能结果和支气管肺泡灌洗液的细胞学特点。结果（1）胸内结节病组和全身多脏器结节病组的发病年龄分别为（49±12）岁和（48±11）岁，差异无统计学意义（t=0．114，P〉0．05）。与胸内结节病相比，全身多脏器结节病以女性多见（分别为13／24和24／29，〈=5．094，P〈0．05），且40岁以上女性占72．4％（21／29）。（2）胸内结节病主要首诊于呼吸科和胸外科，多以肺部受累症状就诊；全身多脏器结节病临床表现多样，可首诊于各个临床科室；而以少见累及器官为首发表现的结节病容易误诊和漏诊。与胸内结节病相比，全身多脏器结节病全身症状多见（分别为25．0％和58．6％，）（2=6．043，P〈0．05），确诊时间长[分别为1．75（0．625～3．000）个月和6（O～40）个月，Z=-3．377，P〈0．01]。（3）两组患者的血清血管紧张素转化酶（sACE）水平分别为（72±33）U／L和（75±59）U／L，差异无统计学意义（t=-0．193，P〉0．05）。与胸内结节病比较，DLCO占预计值％在全身多脏器结节病下降更为明显[分别为（84±8）％和（69±21）％，t=2．674，P〈0．05]，而FEV1／FVC、FEV1占预计值％、FVC占预计值％及肺总量占预计值％在两组间差异无统计学意义（均P〉0．05）。两组患者BALF的细胞总数、淋巴细胞比例和CD。／CD。比值差异无统计学意义（均P〉0．05），但多脏器结节病患者BALF的中性粒细胞比例显著高于胸内结节病[分别为（10．9±4．9）％和（5．1±2．1）％，t=-4．187，P〈0．01]。结论与胸内结节病相比，全身多脏器结节病以女性多见，病情较重，易发生误诊和漏诊。BALF的中性粒细胞比例可能作为发生全身多脏器结节病的提示指标。

Objective To investigate the clinical characteristics of thoracic sarcoidosis compared with multi-organ sarcoidosis. Methods The clinical data of 24 patients with thoracic sarcoidosis and 29 patients with multi-organ sarcoidosis histologically diagnosed at Beijing Tongren Hospital from 1995 to 2010 were retrospectively analyzed. The demographic data, clinical manifestations, diagnostic procedures, involved organs, serum angiotensin converting enzyme （ACE） levels, lung functions, and cellular characteristics of bronchoalveolar lavage fluid（BALF） were compared. Results No difference was found in the age of onset between the 2 groups [ （49 ± 12）, （48 ±11） years old; t = 0. 114, P 〉 0.05 ]. Multi- organ sarcoidosis was more frequent in females compared with thoracic sarcoidosis （ 13/24, 24/29 ; χ2 = 5. 094 ,P 〈0. 05）, and 72. 41% of the patients with multi-organ disease were females above 40 years old. The patients with thoracic sarcoidosis mostly presented first to respiratory physicians or chest surgeons, often with the symptoms of lung involvement. The manifestations of multi-organ sarcoidosis varied considerably and the patients might present to any clinical departments. Sarcoidosis with rare involvement of organs as the presenting symptoms was easy to be misdiagnosed. A higher incidence of systemic constitutional symptoms （25.0%, 58.6% ; χ2 = 6. 043, P 〈 0. 05 ） and a longer duration for definite diagnosis [ 1.75 （0. 625 -3. 000）, 6（0-40） months; Z = -3. 377, P 〈0. 01 ] were found in patients with multi-organ sarcoidosis compared with thoracic sareoidosis. There was no difference in the serum ACE level between the 2 groups [ （72 ±33）, （75 ±59） U/L; t = - 0. 193 ,P 〉 0. 05 ]. Although forced expiratory volume in one second （FEV1）/ forced vital capacity （FVC）, FEV1% predicted （pred）, FVC% pred and total lung capacity （TLC） % pred showed no difference （t = O. 134 -0. 683, P 〉 0. 05 ）, the diffusing capacity of the lung of carbon monoxide （DLCO） % pred decreased more remarkably in muhi-organ sarcoidosis [ （ 84 ± 8 ）, （69 ± 21）; t = 2. 674,P 〈 0. 05~. The total cell count, alveolar lymphocyte percentage and CDJCDs ratio of BALF demonstrated no significant difference between the 2 groups（ t = -0. 628 - -0. 367, P 〉 0.05 ） , but the neutrophil percentage was significantly higher in multi-organ sarcoidosis compared with thoracic sareoidosis I（10.9_±4.9）%,（5.1 ±2.1）%; t= -4.187, P〈0.01]. Conclusions Compared with thoracic sarcoidosis, multi-organ sarcoidosis seemed to be more common in females and more serious. Increased percentage of neutrophils in BALF may be a suggestive index for multiple organ involvements.