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Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax and bilateral pleural effusion: A case report
Y. Zhang, L. Yang, Y. Li
Corresponding author
liyuechuandoctor1@163.com

Corresponding author at: Department of Respiratory and Critical Care Medicine, Tianjin Chest Hospital, No. 261, Taierzhuang South Road, Tianjin, 300222, China.
, H. Ma, Y. Zhang
Department of Respiratory and Critical Care Medicine, Tianjin Chest Hospital, Tianjin, 300222, China
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Chest radiograph shows pneumothorax on the right side&#59; High resolution computed tomography &#40;HRCT&#41; coronal &#40;B&#41; and axial &#40;C&#41; show pneumothorax on the right side&#44; bilateral pleurae thickening and subpleural parenchymal consolidation &#40;red arrow&#41;&#59; &#40;D&#41; HRCT shows that the fibrosis and consolidation are scanty of the lower lobes&#46; &#40;E&#41; Section of pleurae biopsy &#40;H&#38;E stain &#215;100&#41; shows the thickened pleura&#59; &#40;F&#41; Masson&#8217;s trichrome stain &#40;&#215;100&#41; demonstrates dense fibrosis in subpleural lung parenchyma&#59; &#40;G&#41; Elastic Van Gieson &#40;EVG&#41; stain &#40;&#215;100&#41; highlights excessive elastin fibers deposition in pleura&#59; &#40;H&#41; EVG stain &#40;&#215;100&#41; reveals subpleural lung parenchyma&#46; &#40;I&#41; Chest radiograph shows peumothorax on the left side&#59; &#40;J&#41; HRCT coronal plane shows progressive bilateral pleurae thickening&#59; &#40;K&#41; HRCT axial plane exhibits increasing linear and patchy opacities in the lower lobe&#59; &#40;L&#41; HRCT exhibits bilateral pleural effusion &#40;red arrow&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Idiopathic pleuroparenchymal fibroelastosis &#40;IPPFE&#41; is widely recognized as a specific entity belonging to idiopathic interstitial pneumonia<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and is usually characterized with progressive exertional dyspnea and pneumothorax&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> There are no previous reports of an IPPFE case with recurrent pneumothorax and bilateral pleural effusion&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Here&#44; we reported an unusual IPPFE case with recurrent pneumothorax with bilateral pleural effusion&#46; A case of a 28-year-old female&#44; non-smoker was initially admitted to our hospital due to dry cough and progressively worsening dyspnea&#46; High resolution computed tomography &#40;HRCT&#41; revealed pneumothorax in the right upper zone and consolidation in the bilateral upper zone &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig</a> A&#8211;D&#41;&#46; Biopsy of right upper lobe and right pleura revealed obvious fibrosis and elastosis in thickening pleura and subpleural parenchyma&#44; which were in keeping with IPPFE &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig</a> E&#8211;H&#41;&#46; 5 months later&#44; the patient was re-admitted for progressive hypoxaemia and respiratory distress &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig</a> I&#8211;L&#41;&#46; Imaging showed bilateral apical pleural thickening with concurrent bilateral pleural effusion&#46; Following antibiotics therapy and oxygen therapy for a week&#44; she was discharged after the return of stable vital signs&#46; However&#44; she suffered from gradually worsened wheezing and died on the 120th day after discharge from the hospital&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">In conclusion&#44; IPPFE should be considered if a case presents with bilateral pleurae thickening and subpleural parenchymal lesions with an upper lobe predominance&#46; Elastin fiber stain should be performed routinely in patients with the clinical and radiological features of IPPFE&#44; if biopsy specimen can be obtained&#46; Pleural effusion may implicate disease progression and poor prognosis&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethics approval and consent to participate</span><p id="par0020" class="elsevierStylePara elsevierViewall">The study was conducted in accordance with the principles of the Declaration of Helsinki and Good Clinical Practice guidelines&#46; The study was approved by the Ethics Committee of Tianjin Chest Hospital&#46; Written informed consent was obtained from individual&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Consent to publication</span><p id="par0025" class="elsevierStylePara elsevierViewall">Written informed consent has been obtained from the relative of the participant&#44; who is approved the publication of the manuscript with anonymity&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Availability of data and materials</span><p id="par0030" class="elsevierStylePara elsevierViewall">All data generated or analyzed during this study are included in this published article&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conflicts of interest</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Funding</span><p id="par0040" class="elsevierStylePara elsevierViewall">This work was supported by the fund of <span class="elsevierStyleGrantSponsor" id="gs0005">Tianjin Chest Hospital</span> &#40;<span class="elsevierStyleGrantNumber" refid="gs0005">2018XKZ29</span>&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Authors&#8217; contributions</span><p id="par0045" class="elsevierStylePara elsevierViewall">YXZ and YCL input into the concept and design of the study&#46; LY&#44; HM and YZ analyzed imaging data&#46; YXZ&#44; LY&#44; YCL analyzed the medical file and wrote the manuscript&#46; All authors have read&#44; revised the manuscript and approved the final version&#46;</p></span></span>"
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