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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Idiopathic pleuroparenchymal fibroelastosis &#40;IPPFE&#41; is a rare condition characterized by fibroelastotic thickening of the pleural and subpleural lung parenchyma&#44; mainly in the upper lobes&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This entity was first mentioned in English-language medical literature by Frankel et al&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> in 2004&#44; although the same concept designated as idiopathic pulmonary upper lobe fibrosis was proposed in 1992 by Aminati et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> There is little known about its etiology and most cases are considered to be idiopathic&#44; although a few cases are believed to occur in a familial&#47;genetic lung disease context<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and others have been reported in association with previous bone marrow transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> New data have been published suggesting that recurrent infections may have a role in its pathogenesis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Recently IPPFE was recognized as a specific rare idiopathic interstitial pneumonia &#40;IIP&#41; in the update of the international multidisciplinary classification of the IIPs<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a>&#59; IPPFE was characterized by an elastotic fibrosis present with an intra-alveolar fibrosis&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> High-resolution chest tomography &#40;HRCT&#41; shows dense subpleural consolidation with traction bronchiectasis&#44; architectural distortion&#44; and upper lobe volume loss&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> The authors present two cases of IPPFE in which the final diagnosis was obtained after CT-guided transthoracic core lung biopsy&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Case report 1</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 66-year-old female&#44; caucasian&#44; nonsmoker&#44; had worked as art gallery assistant&#44; had a history of esophageal hiatus hernia and was chronically medicated with omeprazole&#46; She had no known family history of pulmonary disease&#46; In September 2010&#44; she complained of dyspnea on exertion and nonproductive cough&#44; with progressive worsening&#46; The patient was examined by a general practitioner and a chest X-ray was performed that revealed a reticular densification with peripheral distribution and predominance in the upper lobes&#46; Lung function tests were as follows&#58; FVC 1&#46;74<span class="elsevierStyleHsp" style=""></span>L &#40;79&#46;4&#37; predicted&#41;&#44; FEV1 1&#46;51<span class="elsevierStyleHsp" style=""></span>L &#40;83&#46;5&#37; predicted&#41;&#44; FEV1&#47;FVC 86&#46;8&#37;&#44; TLC 4&#46;16<span class="elsevierStyleHsp" style=""></span>L &#40;95&#46;2&#37; predicted&#41;&#44; DLco 2&#46;85<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;44&#46;6&#37; predicted&#41;&#46; Blood gas parameters were in the reference range values&#46; She walked 450<span class="elsevierStyleHsp" style=""></span>m at 6-min walk test &#40;6-WT&#41; with an oxygen desaturation of 12&#37; &#40;initial SatO2-97&#37; and final SatO2-85&#37;&#41;&#46; HRCT images showed pleural and subpleural thickening with fibrotic changes in the marginal parenchyma&#44; mainly in the upper lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; A short course of corticosteroids was prescribed with no clinical improvement&#46; The patient was then referred to the Interstitial Lung Disease outpatient clinic&#46; On chest examination&#44; fine bibasilar inspiratory rales were identified&#46; Autoimmune serological testing was negative&#46; A bronchoscopy was performed with no noticeable airways abnormalities&#46; Bronchoalveolar lavage revealed a lymphocytosis &#40;28&#46;4&#37;&#41; with a very high CD4&#47;CD8 ratio &#40;14&#46;5&#41;&#44; and no microorganism or malignant cells were identified&#46; Transbronchial and bronchial biopsies had no evidence of malignancy or granulomas&#46; The patient underwent a CT-guided transthoracic core lung biopsy in two distinct locations&#44; complicated by pneumothorax with bronchopleural fistula and subcutaneous emphysema&#46; Histological evaluation showed marked thickened visceral pleura and prominent predominantly elastic sub-pleural fibrosis with mild&#44; patchy lymphoplasmacytic inflammatory infiltrate&#59; orcein stain showed that the elastosis was within the alveolar walls&#44; marking the intra-alveolar fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Despite the prescription of azathioprine &#40;2<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41;&#44; the patient is clinically and functionally worse after 20 months of follow-up&#58; FVC 1&#46;63<span class="elsevierStyleHsp" style=""></span>L &#40;82&#37; predicted&#41;&#44; FEV1 1&#46;44<span class="elsevierStyleHsp" style=""></span>L &#40;89&#37; predicted&#41;&#44; FEV1&#47;FVC 89&#46;5&#37;&#44; TLC 3&#46;28<span class="elsevierStyleHsp" style=""></span>L &#40;80&#37; predicted&#41;&#44; DLco 1&#46;66<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;26&#37; predicted&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Case report 2</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 59-year-old female&#44; caucasian&#44; nonsmoker&#44; accountant&#44; with history of pulmonary tuberculosis &#40;5-years-old&#41;&#44; post-infectious bronchiectasis&#44; recurrent respiratory infections&#44; and osteoporosis&#46; The patient had no known family history of pulmonary disease&#46; Since the age of 50 she had attended a respiratory outpatient clinic because of dyspnea on exertion and pleural thickening in the upper lobes&#46; These findings were interpreted as within the clinical picture of sequelae of tuberculosis and the patient was medicated with an inhaled association of budesonide and formoterol&#46; However&#44; after 7 years of follow-up&#44; the patient presented a worsening of dyspnea on exertion and dry cough&#46; On chest examination&#44; fine bibasilar inspiratory rales were identified&#46; Chest X-ray revealed a linear densification with peripheral distribution and predominance in the upper lobes&#44; with significant worsening compared to previous ones&#46; HRCT showed a pleural thickening with associated subpleural fibrosis&#44; mainly in the upper lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Autoimmune serological testing was negative&#46; Lung function tests showed moderate restrictive ventilatory impairment and a significant decrease in diffusion capacity&#58; FVC 1&#46;37<span class="elsevierStyleHsp" style=""></span>L &#40;56&#46;1&#37; predicted&#41;&#44; FEV1 1&#46;17<span class="elsevierStyleHsp" style=""></span>L &#40;57&#46;3&#37; predicted&#41;&#44; FEV1&#47;FVC 85&#46;5&#37;&#44; TLC 2&#46;87<span class="elsevierStyleHsp" style=""></span>L &#40;64&#46;7&#37; predicted&#41;&#44; DLco 2&#46;07<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;29&#46;4&#37; predicted&#41;&#46; Blood gas parameters were in the reference range values&#46; She walked 435<span class="elsevierStyleHsp" style=""></span>m at 6-WT with an oxygen desaturation of 10&#37; &#40;initial SatO2-96&#37; and final SatO2-86&#37;&#41;&#46; A bronchoscopy was performed with no noticeable airways abnormalities&#46; Bronchoalveolar lavage revealed neutrophilic &#40;53&#46;8&#37;&#41; and eosinophilic &#40;14&#37;&#41; alveolitis and no microorganism or malignant cells were identified&#46; CT-guided transthoracic core lung biopsy was then performed with iatrogenic pneumothorax&#46; Histological evaluation showed marked thickened visceral pleura and sharply demarcated elastic fibrosis&#44; without significant inflammation&#59; orcein stain showed elastosis of the alveolar walls with a predominant intra-alveolar fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; Treatment was started with 400<span class="elsevierStyleHsp" style=""></span>mg&#47;day of hydroxychloroquine and 7&#46;5<span class="elsevierStyleHsp" style=""></span>mg&#47;day of prednisolone&#46; At the time of writing&#44; after a 10-month follow-up period&#44; the patient is clinically and functionally stable&#58; FVC 1&#46;27<span class="elsevierStyleHsp" style=""></span>L &#40;54&#37; predicted&#41;&#44; FEV1 1&#46;07<span class="elsevierStyleHsp" style=""></span>L &#40;54&#37; predicted&#41;&#44; FEV1&#47;FVC 84&#46;4&#37;&#44; TLC 2&#46;94<span class="elsevierStyleHsp" style=""></span>L &#40;68&#37; predicted&#41;&#44; DLco 1&#46;52<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;22&#37; predicted&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">This report describes two cases in which clinical presentation&#44; imaging&#44; and histopathological features are compatible with IPPFE&#46; The recent update of the international multidisciplinary classification of the IIPs recognizes IPPFE as a specific rare IIP&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">A review of the available data shows that these two patients were older than the reported median age of 57 years at diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> However&#44; the absence of smoking habits is in line with the previous reports&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">There is little information and insufficient knowledge regarding the etiology of IPPFE and most cases are considered idiopathic&#44; although a few cases have underlying diseases or conditions such as collagen vascular diseases&#44; bone-marrow transplantation&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> or lung transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Genetic predisposition is probably another factor&#46; Frankel et al<span class="elsevierStyleItalic">&#46;</span> reported two cases believed to have familial&#47;genetic lung disease&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Reddy et al<span class="elsevierStyleItalic">&#46;</span> reported that just over half of the patients studied had recurrent lower respiratory tract infections and speculated that repeated inflammatory damage in a predisposed individual may lead to a pattern of intra-alveolar fibrosis with septal elastosis &#40;IAFE&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In fact&#44; the second case described in this manuscript has a history of recurrent respiratory infections&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">There is a certain variability of clinical presentation among the reported IPPFE patients&#44; such as spontaneous pneumothorax&#44; dyspnea&#44; or chronic cough&#46; However&#44; in a recently published series of clinical cases&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> the most frequent presentation symptoms were shortness of breath in 91&#46;7&#37; and dry cough in 50&#37; of the patients&#44; which fit in with the complaints mentioned by the patients reported in this paper&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Computed tomography findings in both cases revealed bilateral pleuroparenchymal thickening&#44; which was mostly marked in the upper zones&#44; with an associated subpleural reticular pattern consistent with fibrosis&#46; These findings are also consistent with previous series described in literature&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In both cases&#44; CT-guided transthoracic lung biopsies were made in upper lobes and demonstrated IAFE&#46; The transition between the fibroelastosis and the underlying normal lung parenchyma was abrupt&#46; However&#44; there is no standard histological criterion for the diagnosis of IPPFE&#46; Reddy et al&#46;&#44; using published histological criteria&#44; characterized them as &#8220;definite&#8221; when there was upper zone pleural fibrosis with subjacent intra-alveolar fibrosis accompanied by alveolar septal elastosis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Kusagaya et al<span class="elsevierStyleItalic">&#46;</span> used the pathological criteria for the diagnosis of IPPFE as follows&#58; intense fibrosis of the visceral pleura&#59; prominent&#44; homogeneous&#44; subpleural fibroelastosis&#59; sparing of the parenchyma distant from the pleura&#59; mild&#44; patchy lymphoplasmocytic infiltrates&#44; and presence of small numbers of fibroblastic foci&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> IAFE is not specific to IPPFE&#44; being a pathway of lung injury common to a variety of disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> A multidisciplinary approach&#44; integrating all clinical&#44; imaging and histopathological findings led to an IPPFE diagnosis in both cases&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Published case series<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5&#44;8</span></a> have included patients who were submitted to surgical lung biopsy&#46; However&#44; considering that the two described cases were already complicated by iatrogenic pneumothorax&#44; the risk of performing this procedure was very high&#46; Becker et al&#46; postulate that these patients may be prone to the development of secondary spontaneous pneumothorax and reported a death following a surgical lung biopsy complicated by a large bronchopleural fistulae&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Since the histological features obtained by CT-guided transthoracic core lung biopsy were considered representative and compatible with the diagnosis of IPPFE &#40;after exclusion&#44; in a multidisciplinary discussion&#44; of apical cap&#44; the most frequent differential diagnosis&#41;&#44; it was decided not to carry out a surgical biopsy&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">There is no defined treatment and the therapeutic approach varies greatly and is largely empirical in the published clinical cases&#44; with some of them reporting aggressive treatment with corticosteroids and immunosupressants&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Based on this report&#44; we also considered this therapeutic approach with the first patient diagnosed with IPPFE&#46; However&#44; since the hypothesis of recurrent respiratory infections as a possible etiology of this disease had been described and increasingly considered likely&#44; we decided on a different option for the second patient with the prescription of hydroxychloroquine and a low dose of corticosteroids&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">There is more recognition of the etiology and physiopathology of IPPFE&#44; however it would be important for us to know more about the efficacy of different therapeutics administered so far and the evolution of the patients&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflict of interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Idiopathic pleuroparenchymal fibroelastosis &#40;IPPFE&#41; is a recently described rare entity&#44; characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes&#46; The etiology and pathophysiology are unknown&#46; The prognosis is poor&#44; with no effective therapies other than lung transplantation&#46; IPPFE should be properly identified so that it can be approached correctly&#46; This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE&#46;</p></span>"
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Case report
Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity
M.T. Redondoa,
Corresponding author
margarida.tredondo@gmail.com

Corresponding author.
, N. Meloa, P.C. Motaa,b, J.M. Jesusc, C.S. Mourab,d, S. Guimarãesd, A. Moraisa,b
a Department of Pneumology, Centro Hospitalar de São João, Portugal
b Faculty of Medicine of University of Porto, Portugal
c Department of Radiology, Centro Hospitalar de São João, Portugal
d Department of Pathology, Centro Hospitalar de São João, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Idiopathic pleuroparenchymal fibroelastosis &#40;IPPFE&#41; is a rare condition characterized by fibroelastotic thickening of the pleural and subpleural lung parenchyma&#44; mainly in the upper lobes&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This entity was first mentioned in English-language medical literature by Frankel et al&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> in 2004&#44; although the same concept designated as idiopathic pulmonary upper lobe fibrosis was proposed in 1992 by Aminati et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> There is little known about its etiology and most cases are considered to be idiopathic&#44; although a few cases are believed to occur in a familial&#47;genetic lung disease context<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and others have been reported in association with previous bone marrow transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> New data have been published suggesting that recurrent infections may have a role in its pathogenesis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Recently IPPFE was recognized as a specific rare idiopathic interstitial pneumonia &#40;IIP&#41; in the update of the international multidisciplinary classification of the IIPs<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a>&#59; IPPFE was characterized by an elastotic fibrosis present with an intra-alveolar fibrosis&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> High-resolution chest tomography &#40;HRCT&#41; shows dense subpleural consolidation with traction bronchiectasis&#44; architectural distortion&#44; and upper lobe volume loss&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> The authors present two cases of IPPFE in which the final diagnosis was obtained after CT-guided transthoracic core lung biopsy&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Case report 1</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 66-year-old female&#44; caucasian&#44; nonsmoker&#44; had worked as art gallery assistant&#44; had a history of esophageal hiatus hernia and was chronically medicated with omeprazole&#46; She had no known family history of pulmonary disease&#46; In September 2010&#44; she complained of dyspnea on exertion and nonproductive cough&#44; with progressive worsening&#46; The patient was examined by a general practitioner and a chest X-ray was performed that revealed a reticular densification with peripheral distribution and predominance in the upper lobes&#46; Lung function tests were as follows&#58; FVC 1&#46;74<span class="elsevierStyleHsp" style=""></span>L &#40;79&#46;4&#37; predicted&#41;&#44; FEV1 1&#46;51<span class="elsevierStyleHsp" style=""></span>L &#40;83&#46;5&#37; predicted&#41;&#44; FEV1&#47;FVC 86&#46;8&#37;&#44; TLC 4&#46;16<span class="elsevierStyleHsp" style=""></span>L &#40;95&#46;2&#37; predicted&#41;&#44; DLco 2&#46;85<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;44&#46;6&#37; predicted&#41;&#46; Blood gas parameters were in the reference range values&#46; She walked 450<span class="elsevierStyleHsp" style=""></span>m at 6-min walk test &#40;6-WT&#41; with an oxygen desaturation of 12&#37; &#40;initial SatO2-97&#37; and final SatO2-85&#37;&#41;&#46; HRCT images showed pleural and subpleural thickening with fibrotic changes in the marginal parenchyma&#44; mainly in the upper lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; A short course of corticosteroids was prescribed with no clinical improvement&#46; The patient was then referred to the Interstitial Lung Disease outpatient clinic&#46; On chest examination&#44; fine bibasilar inspiratory rales were identified&#46; Autoimmune serological testing was negative&#46; A bronchoscopy was performed with no noticeable airways abnormalities&#46; Bronchoalveolar lavage revealed a lymphocytosis &#40;28&#46;4&#37;&#41; with a very high CD4&#47;CD8 ratio &#40;14&#46;5&#41;&#44; and no microorganism or malignant cells were identified&#46; Transbronchial and bronchial biopsies had no evidence of malignancy or granulomas&#46; The patient underwent a CT-guided transthoracic core lung biopsy in two distinct locations&#44; complicated by pneumothorax with bronchopleural fistula and subcutaneous emphysema&#46; Histological evaluation showed marked thickened visceral pleura and prominent predominantly elastic sub-pleural fibrosis with mild&#44; patchy lymphoplasmacytic inflammatory infiltrate&#59; orcein stain showed that the elastosis was within the alveolar walls&#44; marking the intra-alveolar fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Despite the prescription of azathioprine &#40;2<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41;&#44; the patient is clinically and functionally worse after 20 months of follow-up&#58; FVC 1&#46;63<span class="elsevierStyleHsp" style=""></span>L &#40;82&#37; predicted&#41;&#44; FEV1 1&#46;44<span class="elsevierStyleHsp" style=""></span>L &#40;89&#37; predicted&#41;&#44; FEV1&#47;FVC 89&#46;5&#37;&#44; TLC 3&#46;28<span class="elsevierStyleHsp" style=""></span>L &#40;80&#37; predicted&#41;&#44; DLco 1&#46;66<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;26&#37; predicted&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Case report 2</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 59-year-old female&#44; caucasian&#44; nonsmoker&#44; accountant&#44; with history of pulmonary tuberculosis &#40;5-years-old&#41;&#44; post-infectious bronchiectasis&#44; recurrent respiratory infections&#44; and osteoporosis&#46; The patient had no known family history of pulmonary disease&#46; Since the age of 50 she had attended a respiratory outpatient clinic because of dyspnea on exertion and pleural thickening in the upper lobes&#46; These findings were interpreted as within the clinical picture of sequelae of tuberculosis and the patient was medicated with an inhaled association of budesonide and formoterol&#46; However&#44; after 7 years of follow-up&#44; the patient presented a worsening of dyspnea on exertion and dry cough&#46; On chest examination&#44; fine bibasilar inspiratory rales were identified&#46; Chest X-ray revealed a linear densification with peripheral distribution and predominance in the upper lobes&#44; with significant worsening compared to previous ones&#46; HRCT showed a pleural thickening with associated subpleural fibrosis&#44; mainly in the upper lobes &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Autoimmune serological testing was negative&#46; Lung function tests showed moderate restrictive ventilatory impairment and a significant decrease in diffusion capacity&#58; FVC 1&#46;37<span class="elsevierStyleHsp" style=""></span>L &#40;56&#46;1&#37; predicted&#41;&#44; FEV1 1&#46;17<span class="elsevierStyleHsp" style=""></span>L &#40;57&#46;3&#37; predicted&#41;&#44; FEV1&#47;FVC 85&#46;5&#37;&#44; TLC 2&#46;87<span class="elsevierStyleHsp" style=""></span>L &#40;64&#46;7&#37; predicted&#41;&#44; DLco 2&#46;07<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;29&#46;4&#37; predicted&#41;&#46; Blood gas parameters were in the reference range values&#46; She walked 435<span class="elsevierStyleHsp" style=""></span>m at 6-WT with an oxygen desaturation of 10&#37; &#40;initial SatO2-96&#37; and final SatO2-86&#37;&#41;&#46; A bronchoscopy was performed with no noticeable airways abnormalities&#46; Bronchoalveolar lavage revealed neutrophilic &#40;53&#46;8&#37;&#41; and eosinophilic &#40;14&#37;&#41; alveolitis and no microorganism or malignant cells were identified&#46; CT-guided transthoracic core lung biopsy was then performed with iatrogenic pneumothorax&#46; Histological evaluation showed marked thickened visceral pleura and sharply demarcated elastic fibrosis&#44; without significant inflammation&#59; orcein stain showed elastosis of the alveolar walls with a predominant intra-alveolar fibrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; Treatment was started with 400<span class="elsevierStyleHsp" style=""></span>mg&#47;day of hydroxychloroquine and 7&#46;5<span class="elsevierStyleHsp" style=""></span>mg&#47;day of prednisolone&#46; At the time of writing&#44; after a 10-month follow-up period&#44; the patient is clinically and functionally stable&#58; FVC 1&#46;27<span class="elsevierStyleHsp" style=""></span>L &#40;54&#37; predicted&#41;&#44; FEV1 1&#46;07<span class="elsevierStyleHsp" style=""></span>L &#40;54&#37; predicted&#41;&#44; FEV1&#47;FVC 84&#46;4&#37;&#44; TLC 2&#46;94<span class="elsevierStyleHsp" style=""></span>L &#40;68&#37; predicted&#41;&#44; DLco 1&#46;52<span class="elsevierStyleHsp" style=""></span>mL&#47;min&#47;mmHg &#40;22&#37; predicted&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0020" class="elsevierStylePara elsevierViewall">This report describes two cases in which clinical presentation&#44; imaging&#44; and histopathological features are compatible with IPPFE&#46; The recent update of the international multidisciplinary classification of the IIPs recognizes IPPFE as a specific rare IIP&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">A review of the available data shows that these two patients were older than the reported median age of 57 years at diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> However&#44; the absence of smoking habits is in line with the previous reports&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">There is little information and insufficient knowledge regarding the etiology of IPPFE and most cases are considered idiopathic&#44; although a few cases have underlying diseases or conditions such as collagen vascular diseases&#44; bone-marrow transplantation&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> or lung transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Genetic predisposition is probably another factor&#46; Frankel et al<span class="elsevierStyleItalic">&#46;</span> reported two cases believed to have familial&#47;genetic lung disease&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Reddy et al<span class="elsevierStyleItalic">&#46;</span> reported that just over half of the patients studied had recurrent lower respiratory tract infections and speculated that repeated inflammatory damage in a predisposed individual may lead to a pattern of intra-alveolar fibrosis with septal elastosis &#40;IAFE&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In fact&#44; the second case described in this manuscript has a history of recurrent respiratory infections&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">There is a certain variability of clinical presentation among the reported IPPFE patients&#44; such as spontaneous pneumothorax&#44; dyspnea&#44; or chronic cough&#46; However&#44; in a recently published series of clinical cases&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> the most frequent presentation symptoms were shortness of breath in 91&#46;7&#37; and dry cough in 50&#37; of the patients&#44; which fit in with the complaints mentioned by the patients reported in this paper&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Computed tomography findings in both cases revealed bilateral pleuroparenchymal thickening&#44; which was mostly marked in the upper zones&#44; with an associated subpleural reticular pattern consistent with fibrosis&#46; These findings are also consistent with previous series described in literature&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In both cases&#44; CT-guided transthoracic lung biopsies were made in upper lobes and demonstrated IAFE&#46; The transition between the fibroelastosis and the underlying normal lung parenchyma was abrupt&#46; However&#44; there is no standard histological criterion for the diagnosis of IPPFE&#46; Reddy et al&#46;&#44; using published histological criteria&#44; characterized them as &#8220;definite&#8221; when there was upper zone pleural fibrosis with subjacent intra-alveolar fibrosis accompanied by alveolar septal elastosis&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Kusagaya et al<span class="elsevierStyleItalic">&#46;</span> used the pathological criteria for the diagnosis of IPPFE as follows&#58; intense fibrosis of the visceral pleura&#59; prominent&#44; homogeneous&#44; subpleural fibroelastosis&#59; sparing of the parenchyma distant from the pleura&#59; mild&#44; patchy lymphoplasmocytic infiltrates&#44; and presence of small numbers of fibroblastic foci&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> IAFE is not specific to IPPFE&#44; being a pathway of lung injury common to a variety of disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> A multidisciplinary approach&#44; integrating all clinical&#44; imaging and histopathological findings led to an IPPFE diagnosis in both cases&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Published case series<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5&#44;8</span></a> have included patients who were submitted to surgical lung biopsy&#46; However&#44; considering that the two described cases were already complicated by iatrogenic pneumothorax&#44; the risk of performing this procedure was very high&#46; Becker et al&#46; postulate that these patients may be prone to the development of secondary spontaneous pneumothorax and reported a death following a surgical lung biopsy complicated by a large bronchopleural fistulae&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Since the histological features obtained by CT-guided transthoracic core lung biopsy were considered representative and compatible with the diagnosis of IPPFE &#40;after exclusion&#44; in a multidisciplinary discussion&#44; of apical cap&#44; the most frequent differential diagnosis&#41;&#44; it was decided not to carry out a surgical biopsy&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">There is no defined treatment and the therapeutic approach varies greatly and is largely empirical in the published clinical cases&#44; with some of them reporting aggressive treatment with corticosteroids and immunosupressants&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Based on this report&#44; we also considered this therapeutic approach with the first patient diagnosed with IPPFE&#46; However&#44; since the hypothesis of recurrent respiratory infections as a possible etiology of this disease had been described and increasingly considered likely&#44; we decided on a different option for the second patient with the prescription of hydroxychloroquine and a low dose of corticosteroids&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">There is more recognition of the etiology and physiopathology of IPPFE&#44; however it would be important for us to know more about the efficacy of different therapeutics administered so far and the evolution of the patients&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflict of interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Idiopathic pleuroparenchymal fibroelastosis &#40;IPPFE&#41; is a recently described rare entity&#44; characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes&#46; The etiology and pathophysiology are unknown&#46; The prognosis is poor&#44; with no effective therapies other than lung transplantation&#46; IPPFE should be properly identified so that it can be approached correctly&#46; This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE&#46;</p></span>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Histological features of patient no&#46; 1 shows a sub-pleural elastotic fibrosis highlighted by the orcein elastic stain&#44; that marks the alveolar walls elastosis &#40;&#916;&#41; and the intra-alveolar fibrosis &#40;<span class="elsevierStyleInlineFigure"><elsevierMultimedia class="elsevierStyleLink" ident="fx1"></elsevierMultimedia></span>&#41; &#40;H&#38;E&#44; 100&#215;&#59; Orcein&#44; 40&#215;&#41;&#46;</p>"
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Article information
ISSN: 21735115
Original language: English
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Pulmonology

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