Moua Teng, Zamora Martinez Ana C, Baqir Misbah, Vassallo Robert, Limper Andrew H, Ryu Jay H
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55901, USA.
Respir Res. 2014 Dec 4;15(1):154. doi: 10.1186/s12931-014-0154-6.
Although usual interstitial pneumonia (UIP) appears to portend better survival when associated with connective tissue disease (CTD-UIP), little is known about the presenting clinical, radiologic, and pathologic features that differentiate pathologically confirmed UIP with CTD from idiopathic pulmonary fibrosis (IPF). In patients with atypical radiologic and clinical features, what specific findings predict underlying IPF vs. CTD-UIP diagnosis and their respective long term survival?
A large retrospective cohort analysis was done of consecutive patients seen from 1995 through 2010 with biopsy confirmed UIP completed or reviewed at our institution. CTD-UIP was defined by independent rheumatology consultation with exclusion of all other secondary causes of lung fibrosis. Primary clinical data was collected and compared for IPF and CTD-UIP along with logistic regression performed for predictors of disease likelihood and Cox proportional hazards analysis for predictors of survival.
Six hundred and twenty five patients were included in the study of which 89 had diagnosed CTD-UIP representing 7 disease entities. Survival was better among those with CTD-UIP except in UIP associated with rheumatoid arthritis, which had similar presenting features and survival to IPF. Predictors of underlying CTD included female gender, younger age, positive autoimmune serology, and inconsistent presenting radiologic findings. Only age and forced vital capacity corrected for a priori covariates were predictive of survival in CTD-UIP.
UIP pathology occurs frequently among patients with atypically presenting clinical and radiologic features, and may represent IPF or CTD-UIP with improved prognosis if underlying CTD is diagnosed. Presenting radiologic and pathologic features alone are not predictive of underlying secondary cause or survival between the two groups.
尽管普通型间质性肺炎（UIP）与结缔组织病（CTD-UIP）相关时似乎预示着更好的生存率，但对于在病理上确诊的CTD-UIP与特发性肺纤维化（IPF）之间进行鉴别的临床表现、放射学和病理学特征知之甚少。在具有非典型放射学和临床特征的患者中，哪些特定发现可预测潜在的IPF与CTD-UIP诊断及其各自的长期生存率？
对1995年至2010年期间在我们机构就诊且活检证实为UIP并已完成或经复查的连续患者进行了一项大型回顾性队列分析。CTD-UIP通过独立的风湿病会诊定义，并排除所有其他肺纤维化的继发原因。收集了IPF和CTD-UIP的主要临床数据并进行比较，同时对疾病可能性的预测因素进行了逻辑回归分析，并对生存预测因素进行了Cox比例风险分析。
该研究纳入了625例患者，其中89例被诊断为CTD-UIP，代表7种疾病实体。CTD-UIP患者的生存率更高，但与类风湿关节炎相关的UIP除外，其临床表现和生存率与IPF相似。潜在CTD的预测因素包括女性、年龄较小、自身免疫血清学阳性以及放射学表现不一致。仅年龄和校正了先验协变量的用力肺活量可预测CTD-UIP的生存率。
UIP病理在具有非典型临床和放射学特征的患者中经常出现，如果诊断出潜在的CTD，可能代表IPF或CTD-UIP，预后较好。仅目前的放射学和病理学特征不能预测潜在的继发原因或两组之间的生存率。
