A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy
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CASE REPORT
Open Access
A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy Aiko Sekine1, Kohei Seo1* , Satoshi Matsukura2, Masaaki Sato3, Aya Shinozaki-Ushiku4, Takashi Ogura5, Akihiko Kitami2, Mitsutaka Kadokura2, Satoshi Dohi1, Kiyotake Ichizuka1 and Masaaki Nagatsuka1
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, Idiopathic pleuroparenchymal fibroelastosis, Lung transplant, Pregnancy, Case report
Background Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare entity characterized by upper lobe-dominant subpleural fibroelastosis and dense fibrous thickening of the visceral pleura [1]. According to some reports, this disease progresses slowly; however, there have been reports of rapid deterioration [2–4]. It has been reported that the onset age is highly heterogeneous, ranging from 13 to 87 years, and bimodal peaks occur in the 30 s and 60 s [5]. There are no effective medications, and the prognosis is considered poor; therefore, diagnosis and careful observation during the asymptomatic stage are * Correspondence: [email protected] 1 Department of Obstetrics and Gynecology, Showa University Northern Yokohama Hospital, Chigasaki-chuo 35-1, Tsuzuki- ku, Yokohama-shi, Kanagawa 224-8503, Japan Full list of author information is available at the end of the article
important, and early lung transplantation should be considered when symptoms appear. No cases of IPPFE complicating pregnancy have been reported. Here, we report our experience of a young woman with IPPFE that deteriorated rapidly during pregnancy. In this case, live birth was achieved, and the mother was subsequently able to undergo lung transplantation.
Case presentation The patient was a primigravida, para 0, 29-year-old woman with unremarkable family history and a medical history of idiopathic thrombocytopenia. Her chief complaints were cough and respiratory distress. The patient had been exa
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