Awano Nobuyasu, Inomata Minoru, Ikushima Soichiro, Yamada Daisuke, Hotta Masatoshi, Tsukuda Shunji, Kumasaka Toshio, Takemura Tamiko, Eishi Yoshinobu
Department of Human Pathology, Tokyo Medical and Dental University Graduate School, Tokyo, Japan.
Department of Respiratory Medicine, Japanese Red Cross Medical Centre, Tokyo, Japan.
Histopathology. 2017 May;70(6):896-905. doi: 10.1111/his.13153. Epub 2017 Feb 8.
To evaluate pulmonary vasculopathy in an autopsy series of patients with combined pulmonary fibrosis and emphysema (CPFE), and compare these findings with those of patients with idiopathic pulmonary fibrosis (IPF) alone and emphysema alone.
We retrospectively analysed the clinical, radiological and pathological features of 26 patients with CPFE, 11 with IPF, and 23 with emphysema. We evaluated pulmonary vascular, venous-venular and arteriolar tissue changes in the fibrotic, emphysematous and relatively unaffected (preserved) areas by using the Heath-Edwards scoring system. We found moderate-to-severe vasculopathy in the CPFE group, but no significant differences in the fibrotic and emphysematous areas among the three groups. However, in the preserved area, the grading was significantly different among the three groups (P < 0.001), and vasculopathy in the CPFE group was the most severe. Although venous-venular and arteriolar changes in almost all fibrotic and emphysematous areas in the three groups showed no significant differences, there were significant differences in venous-venular (P = 0.004) and arteriolar (P < 0.001) changes in the preserved area among the three groups, which were most prevalent in the CPFE group. In the CPFE group, venous-venular changes and vasculopathy by Heath-Edwards grading were highest in the fibrotic area and lowest in the preserved area.
These results imply that pulmonary vasculopathy in patients with CPFE could occur in the whole lung tissue. This may explain the tendency for it to lead to the development of pulmonary hypertension in CPFE cases.
评估合并肺纤维化和肺气肿（CPFE）患者尸检系列中的肺血管病变，并将这些结果与单纯特发性肺纤维化（IPF）患者和单纯肺气肿患者的结果进行比较。
我们回顾性分析了26例CPFE患者、11例IPF患者和23例肺气肿患者的临床、放射学和病理学特征。我们使用希思-爱德华兹评分系统评估纤维化、肺气肿和相对未受影响（保留）区域的肺血管、静脉-小静脉和小动脉组织变化。我们发现CPFE组存在中度至重度血管病变，但三组之间纤维化和肺气肿区域无显著差异。然而，在保留区域，三组之间的分级有显著差异（P < 0.001），且CPFE组的血管病变最严重。尽管三组几乎所有纤维化和肺气肿区域的静脉-小静脉和小动脉变化均无显著差异，但三组保留区域的静脉-小静脉（P = 0.004）和小动脉（P < 0.001）变化存在显著差异，CPFE组最为普遍。在CPFE组中，静脉-小静脉变化和希思-爱德华兹分级的血管病变在纤维化区域最高，在保留区域最低。
这些结果表明CPFE患者的肺血管病变可能发生在整个肺组织中。这可能解释了CPFE病例中其导致肺动脉高压发展的倾向。
