A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy
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 pregna...
| Main Authors: | , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2020-10-01
|
| Series: | BMC Pulmonary Medicine |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12890-020-01308-2 |
| _version_ | 1818771847611678720 |
|---|---|
| author | Aiko Sekine Kohei Seo Satoshi Matsukura Masaaki Sato Aya Shinozaki-Ushiku Takashi Ogura Akihiko Kitami Mitsutaka Kadokura Satoshi Dohi Kiyotake Ichizuka Masaaki Nagatsuka |
| author_facet | Aiko Sekine Kohei Seo Satoshi Matsukura Masaaki Sato Aya Shinozaki-Ushiku Takashi Ogura Akihiko Kitami Mitsutaka Kadokura Satoshi Dohi Kiyotake Ichizuka Masaaki Nagatsuka |
| author_sort | Aiko Sekine |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-18T09:59:58Z |
| format | Article |
| id | doaj.art-30830e97354b4fc6ade1e81ba14fe7d9 |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-12-18T09:59:58Z |
| publishDate | 2020-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-30830e97354b4fc6ade1e81ba14fe7d92022-12-21T21:11:41ZengBMCBMC Pulmonary Medicine1471-24662020-10-012011510.1186/s12890-020-01308-2A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancyAiko Sekine0Kohei Seo1Satoshi Matsukura2Masaaki Sato3Aya Shinozaki-Ushiku4Takashi Ogura5Akihiko Kitami6Mitsutaka Kadokura7Satoshi Dohi8Kiyotake Ichizuka9Masaaki Nagatsuka10Department of Obstetrics and Gynecology, Showa University Northern Yokohama HospitalDepartment of Obstetrics and Gynecology, Showa University Northern Yokohama HospitalRespiratory Disease Center, Showa University Northern Yokohama HospitalDepartment of Thoracic Surgery, University of Tokyo Graduate School of MedicineDepartment of Pathology, University of Tokyo Graduate School of MedicineDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterRespiratory Disease Center, Showa University Northern Yokohama HospitalRespiratory Disease Center, Showa University Northern Yokohama HospitalDepartment of Obstetrics and Gynecology, Showa University Northern Yokohama HospitalDepartment of Obstetrics and Gynecology, Showa University Northern Yokohama HospitalDepartment of Obstetrics and Gynecology, Showa University Northern Yokohama HospitalAbstract 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.http://link.springer.com/article/10.1186/s12890-020-01308-2Acute exacerbationIdiopathic pleuroparenchymal fibroelastosisLung transplantPregnancyCase report |
| spellingShingle | Aiko Sekine Kohei Seo Satoshi Matsukura Masaaki Sato Aya Shinozaki-Ushiku Takashi Ogura Akihiko Kitami Mitsutaka Kadokura Satoshi Dohi Kiyotake Ichizuka Masaaki Nagatsuka A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy BMC Pulmonary Medicine Acute exacerbation Idiopathic pleuroparenchymal fibroelastosis Lung transplant Pregnancy Case report |
| title | A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| title_full | A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| title_fullStr | A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| title_full_unstemmed | A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| title_short | A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| title_sort | case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy |
| topic | Acute exacerbation Idiopathic pleuroparenchymal fibroelastosis Lung transplant Pregnancy Case report |
| url | http://link.springer.com/article/10.1186/s12890-020-01308-2 |
| work_keys_str_mv | AT aikosekine acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT koheiseo acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT satoshimatsukura acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT masaakisato acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT ayashinozakiushiku acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT takashiogura acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT akihikokitami acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT mitsutakakadokura acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT satoshidohi acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT kiyotakeichizuka acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT masaakinagatsuka acasereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT aikosekine casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT koheiseo casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT satoshimatsukura casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT masaakisato casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT ayashinozakiushiku casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT takashiogura casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT akihikokitami casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT mitsutakakadokura casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT satoshidohi casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT kiyotakeichizuka casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy AT masaakinagatsuka casereportofidiopathicpleuroparenchymalfibroelastosiswithsevererespiratoryfailureinpregnancy |