Futatsuya Chizuru, Minato Hiroshi, Okayama Yurie, Katayanagi Kazuyoshi, Kurumaya Hiroshi, Yuasa Mizuki, Nishi Koichi
Department of Diagnostic Pathology, Ishikawa Prefectural Central Hospital, Japan.
Department of Respiratory Medicine, Ishikawa Prefectural Central Hospital, Japan.
Intern Med. 2020 Jun 15;59(12):1541-1547. doi: 10.2169/internalmedicine.3892-19. Epub 2020 Mar 19.
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a generally slow-progressing rare disorder of unknown etiology. The direct cause of death in cases of IPPFE is rarely investigated. We experienced an autopsy case of a Japanese man with IPPFE and found aspiration pneumonia to be the major trigger of death. The individual had left vocal cord paralysis at admission, which may have contributed to aspiration pneumonia, and which probably was affected by the fibrous adhesion of the left apex of the chest wall resulting from IPPFE. The prevention of aspiration pneumonia is important for maintaining the respiratory function, especially in IPPFE patients with repeated pneumothorax.
特发性胸膜肺实质纤维弹性组织增生症（IPPFE）是一种病因不明、通常进展缓慢的罕见疾病。IPPFE病例的直接死因很少得到研究。我们遇到一例患有IPPFE的日本男性尸检病例，发现吸入性肺炎是死亡的主要诱因。该患者入院时左侧声带麻痹，这可能导致了吸入性肺炎，而这可能受到了IPPFE所致胸壁左尖部纤维粘连的影响。预防吸入性肺炎对于维持呼吸功能很重要，尤其是对于反复发生气胸的IPPFE患者。
