Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan.
Respirology. 2018 Nov;23(11):1032-1040. doi: 10.1111/resp.13313. Epub 2018 Apr 24.
Non-specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. This study of fibrotic NSIP (f-NSIP) aimed to elucidate prognosis and disease behaviour from the viewpoint of clinical background and determine whether long-term change of pulmonary function could provide useful prognostic information.
We analysed the medical records of 157 consecutive patients diagnosed with f-NSIP by surgical lung biopsy. Disease behaviour was categorized into two groups depending on long-term change of pulmonary function: progressive type (relative ≥5%/year decline in the slope of forced vital capacity and/or relative ≥7.5%/year decline in the slope of %diffusing capacity of lung carbon monoxide) or stable type. Predictors of disease behaviour and prognosis were determined using logistic and Cox regression models.
Our f-NSIP cohort included interstitial pneumonia with autoimmune features (IPAF) (36.9%), idiopathic (non-IPAF) (22.3%) and connective tissue disease-associated interstitial lung disease (40.8%). Multivariate analysis showed that idiopathic (non-IPAF) f-NSIP and progressive type disease were negative prognostic factors of mortality. Poor treatment response at 1 year was an independent predictor of progressive type disease, but was not related to survival. In terms of disease behaviour based on pulmonary function change, some patients with IPAF f-NSIP showed a progressive course.
Although an IPAF diagnosis was useful for identifying good prognosis in idiopathic f-NSIP, some idiopathic f-NSIP patients with or without IPAF showed progressive disease despite therapy. The definition of progressive type disease may be useful in clinical decision-making when determining therapy for f-NSIP.
非特异性间质性肺炎（NSIP）在背景、疾病行为和预后方面具有异质性特征。本研究旨在探讨纤维化 NSIP（f-NSIP）的预后和疾病行为，从临床背景的角度阐明其预后和疾病行为，并确定肺功能的长期变化是否能提供有用的预后信息。
我们分析了 157 例经外科肺活检诊断为 f-NSIP 的连续患者的病历。根据肺功能的长期变化，将疾病行为分为进展型（用力肺活量斜率相对下降≥5%/年和/或 %肺一氧化碳弥散量斜率相对下降≥7.5%/年）或稳定型。使用逻辑回归和 Cox 回归模型确定疾病行为和预后的预测因素。
我们的 f-NSIP 队列包括伴有自身免疫特征的间质性肺炎（IPAF）（36.9%）、特发性（非 IPAF）（22.3%）和结缔组织病相关的间质性肺疾病（40.8%）。多变量分析显示，特发性（非 IPAF）f-NSIP 和进展型疾病是死亡率的负预后因素。1 年时治疗反应不佳是进展型疾病的独立预测因素，但与生存无关。在基于肺功能变化的疾病行为方面，一些 IPAF f-NSIP 患者表现为进展性病程。
虽然 IPAF 诊断有助于识别特发性 f-NSIP 的良好预后，但一些特发性 f-NSIP 患者，无论是否存在 IPAF，尽管进行了治疗，但仍表现为进行性疾病。在为 f-NSIP 确定治疗方案时，进展型疾病的定义可能有助于临床决策。
