Idiopathic pulmonary fibrosis and pulmonary fibrosis in diffuse systemic sclerosis: two fibroses with different prognoses.
Idiopathic pulmonary fibrosis and pulmonary fibrosis in diffuse systemic sclerosis: two fibroses with different prognoses.
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特发性肺纤维化和弥漫性系统性硬化症中的肺纤维化：具有不同预后的两种纤维化。
DOI：
10.1159/000196648
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发表时间：
1997
期刊：
影响因子：
--
通讯作者：
H. Moutsopoulos
中科院分区：
文献类型：
--
作者：
S. Papiris;P. Vlachoyiannopoulos;M. Maniati;K. Karakostas;S. Constantopoulos;H. Moutsopoulos
特发性肺纤维化和弥漫性皮肤系统性硬化症（dSSc）通过纤维化过程累及肺。近年来，人们越来越意识到这两种类型的肺纤维化的自然史可能不同。本研究的目的是以前瞻性的方式比较这两种疾病的肺部受累，以说明其临床病程的差异。在入组研究时，对43例连续患者（18例孤立性间质性肺纤维化（孤立性IPF）和25例dSSc-IPF）进行了临床、影像学和生理学评价，并通过生存分析对比了其疾病进展。与dSSc-IPF相比，孤立性IPF患者的特征为男性占优势（p < 0.001），发病年龄较大（p < 0.001），病程较短（p < 0.001），听诊及杵状音更频繁（分别为p < 0.001和p < 0.0001），更严重的呼吸困难（p < 0.0001）和更晚期的放射学受累（p < 0.0001）。功能指标显示出相当的值，除单次呼吸CO弥散量（p < 0.0001）和PaO 2（p < 0.01）值外，未达到统计学显著性差异，后者在孤立性IPF患者中更差。最后，18例孤立性IPF患者中有12例在出现呼吸道症状后2.66 +/- 1.18年内死亡，而dSSc-IPF患者中没有一例在首次出现呼吸道受累后5.6 +/- 4.25年内死亡（p < 0.001）。总之，尽管两组患者的疾病阶段不完全相当，但本研究似乎显示孤立性IPF患者的预后更差。
Idiopathic pulmonary fibrosis and diffuse cutaneous systemic sclerosis (dSSc) involve the lung by a fibrotic process. In recent years, there has been increasing awareness that the natural history of these two types of pulmonary fibrosis might be different. The purpose of this study was to compare lung involvement in these two diseases in a prospective fashion in order to address differences in their clinical course. Forty-three consecutive patients, 18 with lone interstitial pulmonary fibrosis (lone IPF) and 25 with dSSc-IPF were evaluated clinically, radiologically and physiologically at the entry into the study and the evolution of their disease was contrasted by survival analysis. Patients with lone IPF compared with dSSc-IPF were characterized by male predominance (p < 0.001), older age at disease onset (p < 0.001), shorter disease duration (p < 0.001), more frequent crackles on auscultation and clubbing (p < 0.001 and p < 0.0001, respectively), more severe dyspnea (p < 0.0001) and more advanced radiological involvement (p < 0.0001). Functional indices presented comparable values and did not reach statistically significant differences except for the values of single breath CO diffusing capacity (p < 0.0001) and the PaO2 (p < 0.01) which was worse in patients with lone IPF. Finally 12 of the 18 patients with lone IPF died in 2.66 +/- 1.18 years from the onset of respiratory symptoms, while none of the dSSc-IPF patients had died 5.6 +/- 4.25 years from the first ever appearance of respiratory involvement (p < 0.001). In conclusion, although the two groups of patients were not at an absolutely comparable stage of their disease, a worse prognosis for patients with lone IPF seems to emerge from this study.