Kuranishi Lilian Tiemi, Leslie Kevin O, Ferreira Rimarcs Gomes, Coletta Ester Aparecida Ney, Storrer Karin Mueller, Soares Maria Raquel, de Castro Pereira Carlos Alberto
Pulmonary Department, Federal University of São Paulo, Sao Paulo, Brazil.
Pathology Department, Mayo Clinic, Scottsdale, USA.
Respir Res. 2015 May 9;16(1):55. doi: 10.1186/s12931-015-0213-7.
Airway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice.
The objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival.
A retrospective study in three centers of interstitial lung disease in São Paulo, between January of 1995 and December of 2012. The surgical lung biopsy specimens were reviewed by three pathologists. The clinical, functional and tomographic findings were analyzed by a standardized protocol.
There were 68 cases of ACIF, most of them women. The mean age was 57 ± 12 yr. Dyspnea, cough, restrictive pattern at spirometry and oxygen desaturation at exercise were common. A reticular pattern with peribronchovascular infiltrates was found in 79% of the cases. The etiologies of ACIF were hypersensitivity pneumonitis in 29 (42.6%), gastroesophageal reflux disease in 17 (25.0%), collagen vascular disease in 4 (5.9%), a combination of them in 15 cases and idiopathic in 3 (4.4%). The median survival was 116 months (95% CI = 58.5 - 173.5). Lower values of oxygen saturation at rest, presence of cough and some histological findings--organizing tissue in the airways, fibroblastic foci and microscopic honeycombing--were predictors of worse survival.
ACIF is an interstitial lung disease with a better survival when compared with IPF. The main etiologies are HP and GERD. The oxygen saturation at rest, the presence of cough and some histological findings are predictors of survival.
气道中心性间质纤维化（ACIF）是我们在临床实践中观察到的一种常见病理模式。
本研究的目的是描述大量患者中与ACIF相关的病因及其对生存的影响。
对圣保罗三个间质性肺病中心在1995年1月至2012年12月期间进行的一项回顾性研究。手术肺活检标本由三名病理学家进行复查。临床、功能和断层扫描结果通过标准化方案进行分析。
共有68例ACIF患者，其中大多数为女性。平均年龄为57±12岁。呼吸困难、咳嗽、肺活量测定时的限制性模式和运动时的氧饱和度下降很常见。79%的病例发现有支气管血管周围浸润的网状模式。ACIF的病因包括29例（42.6%）过敏性肺炎、17例（25.0%）胃食管反流病、4例（5.9%）胶原血管病、15例为它们的组合以及3例（4.4%）特发性。中位生存期为116个月（95%CI = 58.5 - 173.5）。静息时较低的氧饱和度、咳嗽的存在以及一些组织学表现——气道内的机化组织、成纤维细胞灶和显微镜下的蜂窝状改变——是生存较差的预测因素。
与特发性肺纤维化相比，ACIF是一种生存期较好的间质性肺病。主要病因是过敏性肺炎和胃食管反流病。静息时的氧饱和度、咳嗽的存在以及一些组织学表现是生存的预测因素。
