妊娠合并特发性胸膜肺弹力纤维增生症致严重呼吸衰竭 1 例报告
A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy.
机构信息
Department of Obstetrics and Gynecology, Showa University Northern Yokohama Hospital, Chigasaki-chuo 35-1, Tsuzuki- ku, Yokohama-shi, Kanagawa, 224-8503, Japan.
Respiratory Disease Center, Showa University Northern Yokohama Hospital, Chigasaki-chuo 35-1, Tsuzuki-ku, Yokohama-shi, Kanagawa, 224-8503, Japan.
出版信息
BMC Pulm Med. 2020 Oct 14;20(1):264. doi: 10.1186/s12890-020-01308-2.
BACKGROUND
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare lung disease that manifests as parenchymal fibrosis of the upper lung lobe and pleura. There have been no reports of IPPFE complicating pregnancy. Here, we report a case of IPPFE that deteriorated rapidly during pregnancy.
CASE PRESENTATION
A 29-year-old woman presented with dyspnea and dry cough at 19 weeks of gestation. IPPFE with acute exacerbation was suspected on chest computed tomography (CT). Despite steroid treatment, her condition progressed. A cesarean section was performed at 28 weeks of gestation. On postoperative day 26, she underwent living-donor lung transplantation. She was discharged a year after transplantation.
CONCLUSION
Our experience suggested that when pregnancy is complicated by PPFE, the disease may deteriorate rapidly. In this case, even though IPPFE with acute exacerbation was diagnosed during pregnancy, live birth was achieved, and the mother survived after lung transplantation. Lung transplantation should be considered in these patients because, once advanced, pulmonary lesions may be irreversible.
背景
特发性胸膜肺弹力纤维增生症（IPPFE）是一种罕见的肺部疾病，表现为肺上叶实质纤维化和胸膜纤维化。尚未有 IPPFE 合并妊娠的报道。本文报告了一例在妊娠期间迅速恶化的 IPPFE 病例。
病例介绍
一名 29 岁女性在妊娠 19 周时出现呼吸困难和干咳。胸部 CT 提示 IPPFE 伴急性加重。尽管给予了类固醇治疗，但病情仍在进展。患者在妊娠 28 周时行剖宫产术。术后第 26 天，她接受了活体供肺移植。移植 1 年后患者出院。
结论
我们的经验表明，当妊娠合并特发性胸膜肺弹力纤维增生症时，疾病可能迅速恶化。在本例中，尽管在妊娠期间诊断为 IPPFE 伴急性加重，但仍成功分娩，且患者在肺移植后存活。对于这些患者，应考虑进行肺移植，因为一旦出现晚期病变，肺部损伤可能是不可逆的。
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