BODY MASS INDEX AND ARTERIAL BLOOD OXYGENATION AS PROGNOSTIC FACTORS IN PATIENTS WITH IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS
BODY MASS INDEX AND ARTERIAL BLOOD OXYGENATION AS PROGNOSTIC FACTORS IN PATIENTS WITH IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS
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DOI：
10.36141/svdld.v34i1.5172
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发表时间：
2017-01-01
影响因子：
1.6
通讯作者：
Azuma, Arata
中科院分区：
文献类型：
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作者：
Hayashi, Hiroki;Nei, Takahito;Azuma, Arata
背景：特发性胸膜实质纤维弹性增生症（IPPFE）最近被提议纳入罕见的特发性间质性肺炎（IIPs）。然而，其病因、临床特征及预后尚不清楚。目的：我们旨在比较IPPFE与特发性肺纤维化（IPF）的临床特征、结局和预后因素。方法：回顾性分析20例IPPFE患者和71例IPF患者。我们比较了诊断时的临床特征、血液检查数据和呼吸功能。结果：IPPFE组患者体质量指数（BMI）、用力肺活量百分比（% FVC）、总肺活量百分比（% TLC）、呼气储备量百分比（% ERV）明显低于IPF组，残气量/TLC （RV/TLC）比明显高于IPF组。IPPFE组FVC年变化（-326ml/年）明显大于IPF组（-142ml/年）。IPPFE组的生存率明显低于IPF组（P = 0.021）。BMI和动脉血氧分压（PaO2）与IPPFE的结局有显著相关。结论：我们目前的结果表明，IPPFE患者的预后比IPF患者差。我们主张在首次就诊时确定BMI和动脉血PaO2，因为这些参数与患者的预后密切相关。从亚临床阶段开始对IPPFE进行前瞻性评估是必要的，以确保在进展之前采取适当的措施。
Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recently proposed as an entity to be included among rare idiopathic interstitial pneumonias (IIPs). However, the cause, clinical features and prognosis of this rare entity have not been elucidated. Objectives: We aimed to examine the clinical features, outcomes and prognostic factors for IPPFE in comparison to those of idiopathic pulmonary fibrosis (IPF). Methods: We retrospectively analyzed 20 patients with IPPFE and 71 with IPF. We compared clinical features, blood examination data, and respiratory functions at the time of diagnosis. Results: The IPPFE group had a significantly lower body mass index (BMI), percent forced vital capacity (% FVC), total lung capacity (% TLC) and expiratory reserve volume (% ERV), as well as a higher residual volume to TLC (RV/TLC) ratio than the IPF group. The annual FVC changes in the IPPFE group (-326ml/year) were significantly larger than those in the IPF group (-142ml/year). Survival was significantly poorer in the IPPFE than in the IPF group (P = 0.021). BMI and the partial pressure of oxygen in arterial blood (PaO2) were significantly related to the outcome of IPPFE. Conclusions: Our present results indicate the prognosis of IPPFE patients to be poorer than that of IPF patients. We advocate that BMI and arterial blood PaO2 be determined at the first visit as these parameters are closely related to patients' outcomes. Prospective evaluation of IPPFE starting in the subclinical phase is necessary to assure that appropriate measures are taken before progression.