Interstitial pulmonary fibrosis with and without associated collagen vascular disease: results of a two year follow up.
Interstitial pulmonary fibrosis with and without associated collagen vascular disease: results of a two year follow up.
复制标题
伴有或不伴有胶原血管疾病的间质性肺纤维化：两年随访的结果。
作者：
Carlos Agustí;Antoni Xaubet;J. Roca;Alvar Agusti;R. Rodríguez
背景：间质性肺纤维化是一种临床病程多变的疾病。为了确定患者选择不足是否可能解释这种差异的部分原因，我们对两组不同的间质性肺纤维化患者--孤立型肺纤维化患者(LIPF)和相关胶原血管紊乱患者(AIPF)--进行了单独研究。方法：连续20例患者(LIPF 9例，AIPF 11例)。对诊断时的临床表现、X线表现、肺功能检查、67镓肺扫描及支气管肺泡灌洗液细胞学分析结果进行比较。此外，LIPF和AIPF的演变在两年的随访后进行了对比，两组都接受了类似的皮质类固醇治疗方案。结果：在入选时，LIPF和AIPF患者年龄相似，有相似的症状和肺功能紊乱，但LIPF患者表现为手指棍棒，更明显的放射学异常，以及更高比例的支气管肺泡灌洗液中嗜酸性粒细胞。两年后，LIPF患者的FVC、FEV1、TLC、TLCO和PaO2显著降低。相比之下，AIPF患者的肺功能没有改变。同样，当比较从进入研究到研究的百分比变化时，LIPF患者的FVC、FEV1和PaO2显著降低。结论：与AIPF患者不同，LIPF患者表现为肺功能恶化，进一步发展为限制性损害和气体交换不良。这对他们的临床管理有一定的影响。
BACKGROUND: Interstitial pulmonary fibrosis is a disease with a highly variable clinical course. To ascertain if an inadequate selection of patients might explain part of this variability, two different groups of patients with interstitial pulmonary fibrosis, those with the "lone" form of the disease (LIPF) and those with associated collagen vascular disorders (AIPF), were studied separately. METHODS: Twenty consecutive patients (nine with LIPF and 11 with AIPF) were included. Their clinical and radiographic findings and results of pulmonary function tests, gallium-67 lung scanning, and cellular analysis of bronchoalveolar lavage fluid were compared at diagnosis. Moreover, the evolution of LIPF and AIPF was contrasted after a follow up of two years, both groups having received a similar treatment regimen of corticosteroids. RESULTS: At enrollment, patients with LIPF and AIPF were of similar age, and had similar symptoms and derangement of lung function, but patients with LIPF presented with finger clubbing, more obvious radiographic abnormalities, and a greater percentage of eosinophils in bronchoalveolar lavage fluid. Two years later, patients with LIPF had significantly decreased FVC, FEV1, TLC, TLCO, and PaO2. By contrast, lung function remained unaltered in patients with AIPF. Similarly, when the percentage change from entry to the study was compared, patients with LIPF showed a significant decrease in FVC, FEV1, and PaO2. CONCLUSIONS: Unlike the patients with AIPF, those with LIPF showed a deterioration in lung function and developed further restrictive impairment and poorer gas exchange. This has implications in their clinical management.
DOI：
10.1164/ajrccm/143.2.219
发表时间：
1991-02-01
期刊：
AMERICAN REVIEW OF RESPIRATORY DISEASE
影响因子：
--
作者：
AGUSTI, AGN;ROCA, J;RODRIGUEZROISIN, R
通讯作者：
RODRIGUEZROISIN, R
影响因子：
9.6
作者：
Greene,NB;Solinger,AM;Baughman,RP
通讯作者：
Baughman,RP
影响因子：
9.6
作者：
PETERSON, MW;MONICK, M;HUNNINGHAKE, GW
通讯作者：
HUNNINGHAKE, GW