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Case Reports
. 2020 Oct 14;20(1):264.
doi: 10.1186/s12890-020-01308-2.

A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy

Aiko Sekine  1 , Kohei Seo  2 , Satoshi Matsukura  3 , Masaaki Sato  4 , Aya Shinozaki-Ushiku  5 , Takashi Ogura  6 , Akihiko Kitami  3 , Mitsutaka Kadokura  3 , Satoshi Dohi  1 , Kiyotake Ichizuka  1 , Masaaki Nagatsuka  1
Affiliations
Case Reports

A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy

Aiko Sekine et al. BMC Pulm Med. .

Abstract

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.

Keywords: Acute exacerbation; Case report; Idiopathic pleuroparenchymal fibroelastosis; Lung transplant; Pregnancy.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Chest radiography findings. (a) Chest radiography 3 years before admission showed apical pleural thickening and small reticular opacity (b) Chest radiography at 24 weeks of gestation showed subpleural thickening, reduced pneumatization in the upper lung, and reticular opacity throughout the lung
Fig. 2
Fig. 2
Chest computed tomography findings (CT). (a) Chest CT 3 years before admission showed apical pleural thickening and reticular opacity in the peripheral lung field (b) Chest CT at 24 weeks of gestation showed upper lobe-predominant pleural thickening, bronchiectasis, reticular opacity, and ground-glass opacity throughout the lung
Fig. 3
Fig. 3
Descriptions of the lobes. (a) Gross description: upper lobes were reduced and pleura was irregularly thickened (b) Microscopic description of the upper lobe: aggregations of alveolar elastic fibers and collagen fiber growth; Van Gieson elastic staining (c) Microscopic description of the lower lobe: extensive organization; Van Gieson elastic staining
See this image and copyright information in PMC

References

    1. Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–748. doi: 10.1164/rccm.201308-1483ST. - DOI - PMC - PubMed
    1. Yoshida Y, Nagata N, Tsuruta N, Kitasato Y, Wakamatsu K, Yoshimi M, et al. Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Respir Investig. 2016;54:162–169. doi: 10.1016/j.resinv.2015.11.002. - DOI - PubMed
    1. Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev. 2013;9:229–237. doi: 10.2174/1573398X0904140129125307. - DOI - PMC - PubMed
    1. Oda T, Ogura T, Kitamura H, Hagiwara T, Baba T, Enomoto T, et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146:1248–1255. doi: 10.1378/chest.13-2866. - DOI - PubMed
    1. Bonifazi M, Montero MA, Renzoni EA. Renzoni Idiopathic pleuroparenchymalfibroelastosis. CurrPulmonol Rep. 2017;6(9):15. - PMC - PubMed

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