was read the article
array:20 [ "pii" => "X0873215915856150" "issn" => "08732159" "doi" => "10.1016/j.rppnen.2014.04.008" "estado" => "S300" "fechaPublicacion" => "2015-01-01" "documento" => "article" "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/" "subdocumento" => "fla" "cita" => "Rev Port Pneumol. 2015;21:41-4" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 6972 "formatos" => array:3 [ "EPUB" => 290 "HTML" => 5538 "PDF" => 1144 ] ] "itemSiguiente" => array:16 [ "pii" => "X0873215915856169" "issn" => "08732159" "doi" => "10.1016/j.rppnen.2014.10.002" "estado" => "S300" "fechaPublicacion" => "2015-01-01" "documento" => "article" "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/" "subdocumento" => "fla" "cita" => "Rev Port Pneumol. 2015;21:45" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 3728 "formatos" => array:3 [ "EPUB" => 271 "HTML" => 2539 "PDF" => 918 ] ] "en" => array:8 [ "idiomaDefecto" => true "titulo" => "Drug related toxicity in lung transplant recipients" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:1 [ "paginaInicial" => "45" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. van Zeller, C. Damas" "autores" => array:2 [ 0 => array:2 [ "Iniciales" => "M." "apellidos" => "van Zeller" ] 1 => array:2 [ "Iniciales" => "C." "apellidos" => "Damas" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X0873215915856169?idApp=UINPBA00004E" "url" => "/08732159/0000002100000001/v0_201604141151/X0873215915856169/v0_201604141151/en/main.assets" ] "itemAnterior" => array:16 [ "pii" => "X0873215915856142" "issn" => "08732159" "doi" => "10.1016/j.rppnen.2014.09.007" "estado" => "S300" "fechaPublicacion" => "2015-01-01" "documento" => "article" "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/" "subdocumento" => "fla" "cita" => "Rev Port Pneumol. 2015;21:36-40" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 9511 "formatos" => array:3 [ "EPUB" => 271 "HTML" => 7880 "PDF" => 1360 ] ] "en" => array:11 [ "idiomaDefecto" => true "titulo" => "Postoperative surgical complications after lung transplantation" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "36" "paginaFinal" => "40" ] ] "contieneResumen" => array:1 [ "en" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385614fig1.jpg" "Alto" => 844 "Ancho" => 995 "Tamanyo" => 181581 ] ] "descripcion" => array:1 [ "en" => "CT image showing a bronchial dehiscence in posterior aspect of a left bronchial suture after double-lung transplantation." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. de la Torre, R. Fernández, E. Fieira, D. González, M. Delgado, L. Méndez, J.M. Borro" "autores" => array:7 [ 0 => array:2 [ "Iniciales" => "M." "apellidos" => "de la Torre" ] 1 => array:2 [ "Iniciales" => "R." "apellidos" => "Fernández" ] 2 => array:2 [ "Iniciales" => "E." "apellidos" => "Fieira" ] 3 => array:2 [ "Iniciales" => "D." "apellidos" => "González" ] 4 => array:2 [ "Iniciales" => "M." "apellidos" => "Delgado" ] 5 => array:2 [ "Iniciales" => "L." "apellidos" => "Méndez" ] 6 => array:2 [ "Iniciales" => "J.M." "apellidos" => "Borro" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X0873215915856142?idApp=UINPBA00004E" "url" => "/08732159/0000002100000001/v0_201604141151/X0873215915856142/v0_201604141151/en/main.assets" ] "en" => array:14 [ "idiomaDefecto" => true "titulo" => "Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "41" "paginaFinal" => "44" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "M.T. Redondo, N. Melo, P.C. Mota, J.M. Jesus, C.S. Moura, S. Guimarães, A. Morais" "autores" => array:7 [ 0 => array:4 [ "Iniciales" => "M.T." "apellidos" => "Redondo" "email" => array:1 [ 0 => "margarida.tredondo@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor1" ] ] ] 1 => array:3 [ "Iniciales" => "N." "apellidos" => "Melo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] ] ] 2 => array:3 [ "Iniciales" => "P.C." "apellidos" => "Mota" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] ] ] 3 => array:3 [ "Iniciales" => "J.M." "apellidos" => "Jesus" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] ] 4 => array:3 [ "Iniciales" => "C.S." "apellidos" => "Moura" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "affd" ] ] ] 5 => array:3 [ "Iniciales" => "S." "apellidos" => "Guimarães" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "affd" ] ] ] 6 => array:3 [ "Iniciales" => "A." "apellidos" => "Morais" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Department of Pneumology, Centro Hospitalar de São João, Portugal" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:3 [ "entidad" => "Faculty of Medicine of University of Porto, Portugal" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] 2 => array:3 [ "entidad" => "Department of Radiology, Centro Hospitalar de São João, Portugal" "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] 3 => array:3 [ "entidad" => "Department of Pathology, Centro Hospitalar de São João, Portugal" "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "affd" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "<span class="elsevierStyleSup">*</span>" "correspondencia" => "Corresponding author. margarida.tredondo@gmail.com" ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "High-resolution chest tomography in patient no. 1 shows pleural and subpleural thickening with fibrotic changes in the marginal parenchyma, mainly in the upper lobes." ] ] ] "textoCompleto" => "<p class="elsevierStylePara"><a name="sec0005" class="elsevierStyleCrossRefs"></a></p><span class="elsevierStyleSectionTitle"> Introduction</span><p class="elsevierStylePara"> Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare condition characterized by fibroelastotic thickening of the pleural and subpleural lung parenchyma, mainly in the upper lobes.<a href="#bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a> This entity was first mentioned in English-language medical literature by Frankel et al.<a href="#bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a> in 2004, although the same concept designated as idiopathic pulmonary upper lobe fibrosis was proposed in 1992 by Aminati et al.<a href="#bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a> There is little known about its etiology and most cases are considered to be idiopathic, although a few cases are believed to occur in a familial/genetic lung disease context<a href="#bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a> and others have been reported in association with previous bone marrow transplantation.<a href="#bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a> New data have been published suggesting that recurrent infections may have a role in its pathogenesis.<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> Recently IPPFE was recognized as a specific rare idiopathic interstitial pneumonia (IIP) in the update of the international multidisciplinary classification of the IIPs<a href="#bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a>; IPPFE was characterized by an elastotic fibrosis present with an intra-alveolar fibrosis.<a href="#bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a> High-resolution chest tomography (HRCT) shows dense subpleural consolidation with traction bronchiectasis, architectural distortion, and upper lobe volume loss.<a href="#bib6" class="elsevierStyleCrossRefs"><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.</p><p class="elsevierStylePara"><a name="sec0010" class="elsevierStyleCrossRefs"></a></p><span class="elsevierStyleSectionTitle"> Case report 1</span><p class="elsevierStylePara"> The patient was a 66-year-old female, caucasian, nonsmoker, had worked as art gallery assistant, had a history of esophageal hiatus hernia and was chronically medicated with omeprazole. She had no known family history of pulmonary disease. In September 2010, she complained of dyspnea on exertion and nonproductive cough, with progressive worsening. 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. Lung function tests were as follows: FVC 1.74 L (79.4% predicted), FEV1 1.51 L (83.5% predicted), FEV1/FVC 86.8%, TLC 4.16 L (95.2% predicted), DLco 2.85 mL/min/mmHg (44.6% predicted). Blood gas parameters were in the reference range values. She walked 450 m at 6-min walk test (6-WT) with an oxygen desaturation of 12% (initial SatO2-97% and final SatO2-85%). HRCT images showed pleural and subpleural thickening with fibrotic changes in the marginal parenchyma, mainly in the upper lobes (<a href="#f0005" class="elsevierStyleCrossRefs">Figure 1</a>). A short course of corticosteroids was prescribed with no clinical improvement. The patient was then referred to the Interstitial Lung Disease outpatient clinic. On chest examination, fine bibasilar inspiratory rales were identified. Autoimmune serological testing was negative. A bronchoscopy was performed with no noticeable airways abnormalities. Bronchoalveolar lavage revealed a lymphocytosis (28.4%) with a very high CD4/CD8 ratio (14.5), and no microorganism or malignant cells were identified. Transbronchial and bronchial biopsies had no evidence of malignancy or granulomas. The patient underwent a CT-guided transthoracic core lung biopsy in two distinct locations, complicated by pneumothorax with bronchopleural fistula and subcutaneous emphysema. Histological evaluation showed marked thickened visceral pleura and prominent predominantly elastic sub-pleural fibrosis with mild, patchy lymphoplasmacytic inflammatory infiltrate; orcein stain showed that the elastosis was within the alveolar walls, marking the intra-alveolar fibrosis (<a href="#f0010" class="elsevierStyleCrossRefs">Figure 2</a>). Despite the prescription of azathioprine (2 mg/kg/day), the patient is clinically and functionally worse after 20 months of follow-up: FVC 1.63 L (82% predicted), FEV1 1.44 L (89% predicted), FEV1/FVC 89.5%, TLC 3.28 L (80% predicted), DLco 1.66 mL/min/mmHg (26% predicted).</p><p class="elsevierStylePara"><a name="f0005" class="elsevierStyleCrossRefs"></a></p><p class="elsevierStylePara"><img alt="High-resolution chest tomography in patient no. 1 shows pleural and subpleural thickening with fibrotic changes in the marginal parenchyma, mainly in the upper lobes." src="420v21n01-90385615fig1.jpg"></img></p><p class="elsevierStylePara"> Figure 1. High-resolution chest tomography in patient no. 1 shows pleural and subpleural thickening with fibrotic changes in the marginal parenchyma, mainly in the upper lobes.</p><p class="elsevierStylePara"><a name="f0010" class="elsevierStyleCrossRefs"></a></p><p class="elsevierStylePara"><img src="420v21n01-90385615fig2.jpg"></img></p><p class="elsevierStylePara"> Figure 2. Histological features of patient no. 1 shows a sub-pleural elastotic fibrosis highlighted by the orcein elastic stain, that marks the alveolar walls elastosis (Δ) and the intra-alveolar fibrosis (<a name="0" class="elsevierStyleCrossRefs"></a><img src="420v21n01-90385615figfx1.jpg"></img> ) (H&E, 100×; Orcein, 40×).</p><p class="elsevierStylePara"><a name="sec0015" class="elsevierStyleCrossRefs"></a></p><span class="elsevierStyleSectionTitle"> Case report 2</span><p class="elsevierStylePara"> The patient was a 59-year-old female, caucasian, nonsmoker, accountant, with history of pulmonary tuberculosis (5-years-old), post-infectious bronchiectasis, recurrent respiratory infections, and osteoporosis. The patient had no known family history of pulmonary disease. Since the age of 50 she had attended a respiratory outpatient clinic because of dyspnea on exertion and pleural thickening in the upper lobes. 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. However, after 7 years of follow-up, the patient presented a worsening of dyspnea on exertion and dry cough. On chest examination, fine bibasilar inspiratory rales were identified. Chest X-ray revealed a linear densification with peripheral distribution and predominance in the upper lobes, with significant worsening compared to previous ones. HRCT showed a pleural thickening with associated subpleural fibrosis, mainly in the upper lobes (<a href="#f0015" class="elsevierStyleCrossRefs">Figure 3</a>). Autoimmune serological testing was negative. Lung function tests showed moderate restrictive ventilatory impairment and a significant decrease in diffusion capacity: FVC 1.37 L (56.1% predicted), FEV1 1.17 L (57.3% predicted), FEV1/FVC 85.5%, TLC 2.87 L (64.7% predicted), DLco 2.07 mL/min/mmHg (29.4% predicted). Blood gas parameters were in the reference range values. She walked 435 m at 6-WT with an oxygen desaturation of 10% (initial SatO2-96% and final SatO2-86%). A bronchoscopy was performed with no noticeable airways abnormalities. Bronchoalveolar lavage revealed neutrophilic (53.8%) and eosinophilic (14%) alveolitis and no microorganism or malignant cells were identified. CT-guided transthoracic core lung biopsy was then performed with iatrogenic pneumothorax. Histological evaluation showed marked thickened visceral pleura and sharply demarcated elastic fibrosis, without significant inflammation; orcein stain showed elastosis of the alveolar walls with a predominant intra-alveolar fibrosis (<a href="#f0020" class="elsevierStyleCrossRefs">Figure 4</a>). Treatment was started with 400 mg/day of hydroxychloroquine and 7.5 mg/day of prednisolone. At the time of writing, after a 10-month follow-up period, the patient is clinically and functionally stable: FVC 1.27 L (54% predicted), FEV1 1.07 L (54% predicted), FEV1/FVC 84.4%, TLC 2.94 L (68% predicted), DLco 1.52 mL/min/mmHg (22% predicted).</p><p class="elsevierStylePara"><a name="f0015" class="elsevierStyleCrossRefs"></a></p><p class="elsevierStylePara"><img alt="High-resolution chest tomography in patient no. 2 shows a pleural thickening with associated subpleural fibrosis, mainly in the upper lobes." src="420v21n01-90385615fig3.jpg"></img></p><p class="elsevierStylePara"> Figure 3. High-resolution chest tomography in patient no. 2 shows a pleural thickening with associated subpleural fibrosis, mainly in the upper lobes.</p><p class="elsevierStylePara"><a name="f0020" class="elsevierStyleCrossRefs"></a></p><p class="elsevierStylePara"><img src="420v21n01-90385615fig4.jpg"></img></p><p class="elsevierStylePara"> Figure 4. Histological features of patient no. 2 shows the sharped transition between normal lung parenchyma and the fibroelastotic lesion; orcein elastic stain marks the alveolar walls elastosis (Δ) and the intra-alveolar fibrosis (<a name="1" class="elsevierStyleCrossRefs"></a><img src="420v21n01-90385615figfx1.jpg"></img> ) (H&E, 40×, 100×).</p><p class="elsevierStylePara"><a name="sec0020" class="elsevierStyleCrossRefs"></a></p><span class="elsevierStyleSectionTitle"> Discussion</span><p class="elsevierStylePara"> This report describes two cases in which clinical presentation, imaging, and histopathological features are compatible with IPPFE. The recent update of the international multidisciplinary classification of the IIPs recognizes IPPFE as a specific rare IIP.<a href="#bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a></p><p class="elsevierStylePara"> A review of the available data shows that these two patients were older than the reported median age of 57 years at diagnosis.<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> However, the absence of smoking habits is in line with the previous reports.<a href="#bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a></p><p class="elsevierStylePara"> There is little information and insufficient knowledge regarding the etiology of IPPFE and most cases are considered idiopathic, although a few cases have underlying diseases or conditions such as collagen vascular diseases, bone-marrow transplantation,<a href="#bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a> or lung transplantation.<a href="#bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> Genetic predisposition is probably another factor. Frankel et al<span class="elsevierStyleItalic">.</span> reported two cases believed to have familial/genetic lung disease.<a href="#bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a> Reddy et al<span class="elsevierStyleItalic">.</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 (IAFE).<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> In fact, the second case described in this manuscript has a history of recurrent respiratory infections.</p><p class="elsevierStylePara"> There is a certain variability of clinical presentation among the reported IPPFE patients, such as spontaneous pneumothorax, dyspnea, or chronic cough. However, in a recently published series of clinical cases,<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> the most frequent presentation symptoms were shortness of breath in 91.7% and dry cough in 50% of the patients, which fit in with the complaints mentioned by the patients reported in this paper.</p><p class="elsevierStylePara"> Computed tomography findings in both cases revealed bilateral pleuroparenchymal thickening, which was mostly marked in the upper zones, with an associated subpleural reticular pattern consistent with fibrosis. These findings are also consistent with previous series described in literature.<a href="#bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a></p><p class="elsevierStylePara"> In both cases, CT-guided transthoracic lung biopsies were made in upper lobes and demonstrated IAFE. The transition between the fibroelastosis and the underlying normal lung parenchyma was abrupt. However, there is no standard histological criterion for the diagnosis of IPPFE. Reddy et al., using published histological criteria, characterized them as “definite” when there was upper zone pleural fibrosis with subjacent intra-alveolar fibrosis accompanied by alveolar septal elastosis.<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> Kusagaya et al<span class="elsevierStyleItalic">.</span> used the pathological criteria for the diagnosis of IPPFE as follows: intense fibrosis of the visceral pleura; prominent, homogeneous, subpleural fibroelastosis; sparing of the parenchyma distant from the pleura; mild, patchy lymphoplasmocytic infiltrates, and presence of small numbers of fibroblastic foci.<a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">8</span></a> IAFE is not specific to IPPFE, being a pathway of lung injury common to a variety of disorders.<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> A multidisciplinary approach, integrating all clinical, imaging and histopathological findings led to an IPPFE diagnosis in both cases.</p><p class="elsevierStylePara"> Published case series<a href="#bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">, </span><a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">8</span></a> have included patients who were submitted to surgical lung biopsy. However, considering that the two described cases were already complicated by iatrogenic pneumothorax, the risk of performing this procedure was very high. Becker et al. 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.<a href="#bib9" class="elsevierStyleCrossRefs"><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 (after exclusion, in a multidisciplinary discussion, of apical cap, the most frequent differential diagnosis), it was decided not to carry out a surgical biopsy.</p><p class="elsevierStylePara"> There is no defined treatment and the therapeutic approach varies greatly and is largely empirical in the published clinical cases, with some of them reporting aggressive treatment with corticosteroids and immunosupressants.<a href="#bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> Based on this report, we also considered this therapeutic approach with the first patient diagnosed with IPPFE. However, since the hypothesis of recurrent respiratory infections as a possible etiology of this disease had been described and increasingly considered likely, we decided on a different option for the second patient with the prescription of hydroxychloroquine and a low dose of corticosteroids.</p><p class="elsevierStylePara"> There is more recognition of the etiology and physiopathology of IPPFE, 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.</p><p class="elsevierStylePara"><a name="sec0025" class="elsevierStyleCrossRefs"></a></p><span class="elsevierStyleSectionTitle"> Conflict of interest</span><p class="elsevierStylePara"> The authors have no conflicts of interest to declare.</p><p class="elsevierStylePara"> Received 3 December 2013 <br></br> Accepted 29 April 2014</p><p class="elsevierStylePara"> Corresponding author. margarida.tredondo@gmail.com</p>" "pdfFichero" => "320v21n01a90385616pdf001.pdf" "tienePdf" => true "PalabrasClave" => array:1 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec641003" "palabras" => array:3 [ 0 => "Interstitial lung disease" 1 => "Pleuroparenchymal fibrosis" 2 => "Fibroelastosis" ] ] ] ] "tieneResumen" => true "resumen" => array:1 [ "en" => array:1 [ "resumen" => "<span class="elsevierStyleSectionTitle"> Abstract</span><br/><p class="elsevierStylePara"> Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare entity, characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. The etiology and pathophysiology are unknown. The prognosis is poor, with no effective therapies other than lung transplantation. IPPFE should be properly identified so that it can be approached correctly. This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE.</p>" ] ] "multimedia" => array:6 [ 0 => array:6 [ "identificador" => "fig1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "High-resolution chest tomography in patient no. 1 shows pleural and subpleural thickening with fibrotic changes in the marginal parenchyma, mainly in the upper lobes." ] ] 1 => array:5 [ "identificador" => "fig2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" ] 2 => array:5 [ "identificador" => "fig3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" ] 3 => array:6 [ "identificador" => "fig4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "High-resolution chest tomography in patient no. 2 shows a pleural thickening with associated subpleural fibrosis, mainly in the upper lobes." ] ] 4 => array:5 [ "identificador" => "fig5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" ] 5 => array:5 [ "identificador" => "fig6" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "High resolution CT and histological findings in idiopathic Pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 2011; 12:1-5." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "High resolution CT and histological findings in idiopathic Pleuroparenchymal fibroelastosis: features and differential diagnosis." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "Piciucchi S" 1 => "Tomassetti S" 2 => "Casoni G" 3 => "Sverzellati N" 4 => "Carloni A" 5 => "Dubini A" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/1465-9921-12-1" "Revista" => array:6 [ "tituloSerie" => "Respir Res. " "fecha" => "2011" "volumen" => "12" "paginaInicial" => "1" "paginaFinal" => "5" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21194498" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib2" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathological entity. Chest. 2004; 126:2007-13." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathological entity." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "Frankel SK" 1 => "Cool CD" 2 => "Lynch DA" 3 => "Brown KK." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1378/chest.126.6.2007" "Revista" => array:6 [ "tituloSerie" => "Chest. " "fecha" => "2004" "volumen" => "126" "paginaInicial" => "2007" "paginaFinal" => "2013" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15596706" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib3" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Idiopathic pulmonary upper lobe fibrosis. Kokyu. 1992; 11:693-9." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Idiopathic pulmonary upper lobe fibrosis." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "Aminati R" 1 => "Niimi A" 2 => "Kuse F." ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Kokyu. " "fecha" => "1992" "volumen" => "11" "paginaInicial" => "693" "paginaFinal" => "699" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib4" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011; 24:1633-9." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "von der Thusen JH" 1 => "Hansell DM" 2 => "Tominaga M" 3 => "Veys PA" 4 => "Ashworth MT" 5 => "Owens CM" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1038/modpathol.2011.114" "Revista" => array:6 [ "tituloSerie" => "Mod Pathol. " "fecha" => "2011" "volumen" => "24" "paginaInicial" => "1633" "paginaFinal" => "1639" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21822205" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib5" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012; 40:377-85." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "Reddy TL" 1 => "Tominaga M" 2 => "Hansell DM" 3 => "von der Thusen J" 4 => "Rassl D" 5 => "Parfrey H" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1183/09031936.00165111" "Revista" => array:6 [ "tituloSerie" => "Eur Respir J. " "fecha" => "2012" "volumen" => "40" "paginaInicial" => "377" "paginaFinal" => "385" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22441748" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib6" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "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; 188:733-48." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "An official American Thoracic Society/European Respiratory Society statement: update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "Travis WD" 1 => "Costabel U" 2 => "Hansell DM" 3 => "King TE" 4 => "Lynch DA" 5 => "Nicholson AG" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1164/rccm.201308-1483ST" "Revista" => array:6 [ "tituloSerie" => "Am J Respir Crit Care Med. " "fecha" => "2013" "volumen" => "188" "paginaInicial" => "733" "paginaFinal" => "748" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24032382" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib7" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?. Eur Respir J. 2013; 41:243-5." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "Hirota T" 1 => "Fujita M" 2 => "Matsumoto T" 3 => "Higuchi T" 4 => "Shiraishi T" 5 => "Minami M" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1183/09031936.00103912" "Revista" => array:6 [ "tituloSerie" => "Eur Respir J. " "fecha" => "2013" "volumen" => "41" "paginaInicial" => "243" "paginaFinal" => "245" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23277524" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib8" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm. Med. 2012; 12:72." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:7 [ 0 => "Kusagaya H" 1 => "Nakamura Y" 2 => "Kono M" 3 => "Kaida Y" 4 => "Kuroishi S" 5 => "Enomoto N" 6 => "et-al." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/1471-2466-12-72" "Revista" => array:5 [ "tituloSerie" => "BMC Pulm. Med. " "fecha" => "2012" "volumen" => "12" "paginaInicial" => "72" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23216996" "web" => "Medline" ] ] ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib9" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?. Mod Pathol. 2008; 21:784-7." "contribucion" => array:1 [ 0 => array:3 [ "titulo" => "Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?." "idioma" => "en" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "Becker CD" 1 => "Gil J" 2 => "Padilla ML." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1038/modpathol.2008.56" "Revista" => array:6 [ "tituloSerie" => "Mod Pathol. " "fecha" => "2008" "volumen" => "21" "paginaInicial" => "784" "paginaFinal" => "787" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18408658" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/08732159/0000002100000001/v0_201604141151/X0873215915856150/v0_201604141151/en/main.assets" "Apartado" => array:4 [ "identificador" => "50825" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Case reports" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/08732159/0000002100000001/v0_201604141151/X0873215915856150/v0_201604141151/en/320v21n01a90385616pdf001.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X0873215915856150?idApp=UINPBA00004E" ]
Year/Month | Html | Total | |
---|---|---|---|
2024 November | 27 | 4 | 31 |
2024 October | 162 | 21 | 183 |
2024 September | 282 | 32 | 314 |
2024 August | 231 | 37 | 268 |
2024 July | 200 | 48 | 248 |
2024 June | 159 | 21 | 180 |
2024 May | 122 | 36 | 158 |
2024 April | 137 | 42 | 179 |
2024 March | 130 | 42 | 172 |
2024 February | 106 | 38 | 144 |
2024 January | 128 | 50 | 178 |
2023 December | 107 | 53 | 160 |
2023 November | 119 | 52 | 171 |
2023 October | 135 | 78 | 213 |
2023 September | 117 | 73 | 190 |
2023 August | 118 | 56 | 174 |
2023 July | 84 | 50 | 134 |
2023 June | 69 | 35 | 104 |
2023 May | 94 | 29 | 123 |
2023 April | 124 | 27 | 151 |
2023 March | 138 | 24 | 162 |
2023 February | 116 | 19 | 135 |
2023 January | 98 | 23 | 121 |
2022 December | 121 | 32 | 153 |
2022 November | 128 | 41 | 169 |
2022 October | 149 | 48 | 197 |
2022 September | 113 | 36 | 149 |
2022 August | 123 | 58 | 181 |
2022 July | 88 | 41 | 129 |
2022 June | 120 | 37 | 157 |
2022 May | 98 | 42 | 140 |
2022 April | 103 | 45 | 148 |
2022 March | 129 | 47 | 176 |
2022 February | 125 | 43 | 168 |
2022 January | 130 | 26 | 156 |
2021 December | 76 | 37 | 113 |
2021 November | 78 | 32 | 110 |
2021 October | 90 | 57 | 147 |
2021 September | 97 | 27 | 124 |
2021 August | 96 | 36 | 132 |
2021 July | 88 | 19 | 107 |
2021 June | 116 | 32 | 148 |
2021 May | 89 | 24 | 113 |
2021 April | 221 | 62 | 283 |
2021 March | 184 | 31 | 215 |
2021 February | 115 | 21 | 136 |
2021 January | 73 | 19 | 92 |
2020 December | 67 | 14 | 81 |
2020 November | 141 | 21 | 162 |
2020 October | 98 | 23 | 121 |
2020 September | 141 | 34 | 175 |
2020 August | 119 | 21 | 140 |
2020 July | 153 | 22 | 175 |
2020 June | 161 | 21 | 182 |
2020 May | 139 | 22 | 161 |
2020 April | 133 | 17 | 150 |
2020 March | 130 | 9 | 139 |
2020 February | 187 | 25 | 212 |
2020 January | 139 | 24 | 163 |
2019 December | 115 | 17 | 132 |
2019 November | 154 | 20 | 174 |
2019 October | 142 | 33 | 175 |
2019 September | 149 | 33 | 182 |
2019 August | 245 | 21 | 266 |
2019 July | 244 | 26 | 270 |
2019 June | 238 | 8 | 246 |
2019 May | 254 | 28 | 282 |
2019 April | 193 | 22 | 215 |
2019 March | 302 | 9 | 311 |
2019 February | 233 | 12 | 245 |
2019 January | 261 | 24 | 285 |
2018 December | 113 | 4 | 117 |
2018 November | 39 | 0 | 39 |
2018 October | 47 | 12 | 59 |
2018 September | 24 | 6 | 30 |
2018 August | 38 | 27 | 65 |
2018 July | 40 | 16 | 56 |
2018 June | 32 | 17 | 49 |
2018 May | 53 | 18 | 71 |
2018 April | 78 | 19 | 97 |
2018 March | 70 | 16 | 86 |
2018 February | 53 | 15 | 68 |
2018 January | 55 | 11 | 66 |
2017 December | 78 | 18 | 96 |
2017 November | 33 | 10 | 43 |
2017 October | 15 | 15 | 30 |
2017 September | 12 | 12 | 24 |
2017 August | 18 | 10 | 28 |
2017 July | 12 | 17 | 29 |
2017 June | 23 | 17 | 40 |
2017 May | 22 | 15 | 37 |
2017 April | 9 | 5 | 14 |
2017 March | 15 | 4 | 19 |
2017 February | 8 | 4 | 12 |
2017 January | 8 | 11 | 19 |
2016 December | 10 | 6 | 16 |
2016 November | 10 | 2 | 12 |
2016 October | 17 | 9 | 26 |
2016 September | 3 | 3 | 6 |
2016 August | 9 | 6 | 15 |
2016 July | 4 | 7 | 11 |
2016 June | 5 | 5 | 10 |
2016 May | 20 | 4 | 24 |
2016 April | 98 | 2 | 100 |
2016 March | 173 | 53 | 226 |
2016 February | 167 | 39 | 206 |
2016 January | 148 | 40 | 188 |
2015 December | 147 | 33 | 180 |
2015 November | 128 | 27 | 155 |
2015 October | 135 | 64 | 199 |
2015 September | 114 | 31 | 145 |
2015 August | 114 | 25 | 139 |
2015 July | 144 | 11 | 155 |
2015 June | 66 | 12 | 78 |
2015 May | 76 | 24 | 100 |
2015 April | 102 | 36 | 138 |
2015 March | 111 | 41 | 152 |
2015 February | 82 | 67 | 149 |