Evaluation of clinical presentation and outcome of patients with respiratory bronchiolitis-associated interstitial lung disease
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(C) Computed tomography scan showing diffuse thickened bronchial walls and small bronchiectasis in the upper left lobe. (D) Positron-emission tomography/Computed tomography with 18-fludeoxyglucose (FDG) displaying increased metabolic activity in the right hilum region. On bronchoscopic examination (E), a cauliflower lesion was observed on the posterior segment of the right upper bronchus. (F) High-power microscopic appearance of fibrovascular cores surrounded by squamous epithelium (original magnification 100×, haematoxylin and eosin stain)." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "A.T. Castro, L. Carvalho, S. Freitas" "autores" => array:3 [ 0 => array:2 [ "Iniciales" => "A.T." "apellidos" => "Castro" ] 1 => array:2 [ "Iniciales" => "L." "apellidos" => "Carvalho" ] 2 => array:2 [ "Iniciales" => "S." 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Sargento, S. Longo, N. Lousada, R.P. dos Reis" "autores" => array:4 [ 0 => array:2 [ "Iniciales" => "L." "apellidos" => "Sargento" ] 1 => array:2 [ "Iniciales" => "S." "apellidos" => "Longo" ] 2 => array:2 [ "Iniciales" => "N." "apellidos" => "Lousada" ] 3 => array:2 [ "Iniciales" => "R.P." "apellidos" => "dos Reis" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X0873215915856177?idApp=UINPBA00004E" "url" => "/08732159/0000002100000001/v0_201604141151/X0873215915856177/v0_201604141151/en/main.assets" ] "en" => array:11 [ "idiomaDefecto" => true "titulo" => "Evaluation of clinical presentation and outcome of patients with respiratory bronchiolitis-associated interstitial lung disease" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "47" "paginaFinal" => "48" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "M. Vaz, N. Melo, P.C. Mota, R. Cunha, J.M. Pereira" "autores" => array:5 [ 0 => array:4 [ "Iniciales" => "M." "apellidos" => "Vaz" "email" => array:1 [ 0 => "manuelsvaz@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" => "R." "apellidos" => "Cunha" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] ] 4 => array:3 [ "Iniciales" => "J.M." "apellidos" => "Pereira" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Department of Pneumology, Centro Hospitalar de São João, Porto, Portugal" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "affa" ] 1 => array:3 [ "entidad" => "Faculty of Medicine, University of Porto, Porto, Portugal" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "affb" ] 2 => array:3 [ "entidad" => "Department of Imagiology, Centro Hospitalar de São João, Porto, Portugal" "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "affc" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "<span class="elsevierStyleSup">*</span>" "correspondencia" => "Corresponding author. manuelsvaz@gmail.com" ] ] ] ] "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" => "420v21n01-90385618fig1.jpg" "Alto" => 576 "Ancho" => 1399 "Tamanyo" => 265588 ] ] "descripcion" => array:1 [ "en" => "Axial CT images show ill-defined centrilobular ground glass nodules with predominant upper lobe distribution. Areas of paraseptal emphysema are present." ] ] ] "textoCompleto" => "<p class="elsevierStylePara">To the Editor,</p><p class="elsevierStylePara">Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a rare interstitial pneumonia that usually affects current smokers in the fourth and fifth decades of life with a history of heavy smoking (usually more than 30 pack-years).<a href="#bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a> In most cases the symptoms of RB-ILD are subclinical and detected coincidentally or it is associated with a mild clinical presentation, usually a gradual onset of dyspnea and/or a presence of persistent cough.<a href="#bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a> Lung function tests reveal only minor restrictive or obstructive defects in most cases, often combined with hyperinflation. Additionally, carbon monoxide diffusing capacity (DLCO) is often slightly to moderately impaired.<a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib9" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a> High Resolution Chest CT-scan (HRCT) classic features are essentially ground glass centrilobular nodules located in the upper lobes which in the appropriate clinical context are normally not difficult to differentiate from other interstitial lung diseases (ILD).<a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a> Bronchoalveolar Lavage (BAL), may help in the differential diagnosis with other ILD and it is characterized by a raised total cell count induced by an increased number of intense brown pigment-laden macrophages, although a mild neutrophilia may also be present.<a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> The evolution is unpredictable and heterogeneous regardless of smoking cessation and more data are required in order to be precise about the outcome of this disease due the conflicting data published so far.<a href="#bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">, </span><a href="#bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">, </span><a href="#bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a></p><p class="elsevierStylePara">The medical records of patients diagnosed with RB-ILD during the period of January 2008 to July 2013, followed in ILD outpatient clinic at Centro Hospitalar S. João, were retrospectively reviewed. RB-ILD diagnosis was based on 2002 American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias.<a href="#bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a> Fifteen patients were included with a mean age of 48 (29–65) years and 13 (86.6%) were male. All of them were heavy cigarette smokers (median: 46.3 pack-year). Most patients (73.4%) have mild symptoms, dyspnea on exertion (<span class="elsevierStyleItalic">n</span> = 8/53.3%) and cough (<span class="elsevierStyleItalic">n</span> = 7/46.6%) were the only symptoms identified. Typical centrilobular ground-glass nodules predominantly in the upper lobes were the overall pattern on the HRCT (<a href="#f0005" class="elsevierStyleCrossRefs">Figure 1</a>). Concomitant upper lobe centrilobular emphysema was identified in 5 (33.3%) patients. Pulmonary function tests showed a slight obstructive impairment (median FEV1 – 76%) associated with a normal median value in the diffusion capacity (median DL<span class="elsevierStyleInf">CO</span> – 76%), although there was a minor decrease (DL<span class="elsevierStyleInf">CO</span> – 61–75%) in six patients. BAL was performed in 12 patients and besides the increased cellularity associated with the intense brown pigment-laden macrophages (<a href="#f0010" class="elsevierStyleCrossRefs">Figure 2</a>), two (16.6%) had mild neutrophilia and another two mild eosinophilia. Three patients were submitted to surgical lung biopsy, two of them performed during the context of cardiothoracic surgeries not related with RB-ILD and the other one during a video-assisted thoracoscopic surgery for pneumothorax with a persistent air-leak. All patients were referred to smoking cessation programs but only three gave up, the remainder just reduced the amount of tobacco consumption. None of them had any additional therapeutic interventions besides inhaled bronchodilators prescribed to those patients that were more symptomatic. Regardless of whether they maintained smoking habits, after a mean follow-up of 49 (12–72) months, all the patients included remained clinically stable, with similar functional (mean FEV1 – 74% and DLCO – 75%) and radiological features.</p><a name="f0005" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="420v21n01-90385618fig1.jpg" alt="Axial CT images show ill-defined centrilobular ground glass nodules with predominant upper lobe distribution. Areas of paraseptal emphysema are present."></img></p><p class="elsevierStylePara">Figure 1. Axial CT images show ill-defined centrilobular ground glass nodules with predominant upper lobe distribution. Areas of paraseptal emphysema are present.</p><a name="f0010" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="420v21n01-90385618fig2.jpg" alt="BAL from a patient with RB-ILD with an increased number of intense brown pigment-laden macrophages."></img></p><p class="elsevierStylePara">Figure 2. BAL from a patient with RB-ILD with an increased number of intense brown pigment-laden macrophages.</p><p class="elsevierStylePara">This group of RB-ILD patients had the usual characteristic clinical presentation described previously in other series. Additionally, they showed a favorable clinical course, even those who continued to smoke, which confirms a good prognosis associated with this disease.</p><a name="sec0005" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Conflict of interest</span><p class="elsevierStylePara">The authors declare no conflict of interest.</p><p class="elsevierStylePara">Corresponding author. manuelsvaz@gmail.com</p>" "pdfFichero" => "320v21n01a90385619pdf001.pdf" "tienePdf" => true "multimedia" => array:4 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385618fig1.jpg" "Alto" => 576 "Ancho" => 1399 "Tamanyo" => 265588 ] ] "descripcion" => array:1 [ "en" => "Axial CT images show ill-defined centrilobular ground glass nodules with predominant upper lobe distribution. Areas of paraseptal emphysema are present." ] ] 1 => array:8 [ "identificador" => "fig2" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385618fig2.jpg" "Alto" => 738 "Ancho" => 990 "Tamanyo" => 492020 ] ] "descripcion" => array:1 [ "en" => "BAL from a patient with RB-ILD with an increased number of intense brown pigment-laden macrophages." ] ] 2 => array:6 [ "identificador" => "fig3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "Axial CT images show ill-defined centrilobular ground glass nodules with predominant upper lobe distribution. Areas of paraseptal emphysema are present." ] ] 3 => array:6 [ "identificador" => "fig4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "BAL from a patient with RB-ILD with an increased number of intense brown pigment-laden macrophages." ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib7" "etiqueta" => "1" "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." 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| 2022 March | 58 | 41 | 99 |
| 2022 February | 73 | 34 | 107 |
| 2022 January | 76 | 22 | 98 |
| 2021 December | 48 | 36 | 84 |
| 2021 November | 63 | 26 | 89 |
| 2021 October | 88 | 43 | 131 |
| 2021 September | 53 | 21 | 74 |
| 2021 August | 51 | 27 | 78 |
| 2021 July | 45 | 25 | 70 |
| 2021 June | 72 | 22 | 94 |
| 2021 May | 67 | 25 | 92 |
| 2021 April | 163 | 89 | 252 |
| 2021 March | 111 | 20 | 131 |
| 2021 February | 84 | 19 | 103 |
| 2021 January | 65 | 16 | 81 |
| 2020 December | 53 | 10 | 63 |
| 2020 November | 67 | 12 | 79 |
| 2020 October | 56 | 15 | 71 |
| 2020 September | 93 | 19 | 112 |
| 2020 August | 90 | 25 | 115 |
| 2020 July | 104 | 25 | 129 |
| 2020 June | 92 | 18 | 110 |
| 2020 May | 107 | 14 | 121 |
| 2020 April | 83 | 6 | 89 |
| 2020 March | 64 | 7 | 71 |
| 2020 February | 73 | 16 | 89 |
| 2020 January | 85 | 21 | 106 |
| 2019 December | 70 | 22 | 92 |
| 2019 November | 77 | 14 | 91 |
| 2019 October | 73 | 19 | 92 |
| 2019 September | 84 | 16 | 100 |
| 2019 August | 198 | 12 | 210 |
| 2019 July | 192 | 16 | 208 |
| 2019 June | 200 | 7 | 207 |
| 2019 May | 271 | 12 | 283 |
| 2019 April | 214 | 31 | 245 |
| 2019 March | 220 | 11 | 231 |
| 2019 February | 191 | 8 | 199 |
| 2019 January | 233 | 30 | 263 |
| 2018 December | 140 | 9 | 149 |
| 2018 November | 41 | 5 | 46 |
| 2018 October | 61 | 11 | 72 |
| 2018 September | 51 | 3 | 54 |
| 2018 August | 39 | 19 | 58 |
| 2018 July | 46 | 20 | 66 |
| 2018 June | 59 | 15 | 74 |
| 2018 May | 60 | 16 | 76 |
| 2018 April | 75 | 22 | 97 |
| 2018 March | 67 | 24 | 91 |
| 2018 February | 30 | 11 | 41 |
| 2018 January | 53 | 14 | 67 |
| 2017 December | 71 | 16 | 87 |
| 2017 November | 23 | 7 | 30 |
| 2017 October | 11 | 12 | 23 |
| 2017 September | 23 | 11 | 34 |
| 2017 August | 48 | 19 | 67 |
| 2017 July | 28 | 15 | 43 |
| 2017 June | 30 | 17 | 47 |
| 2017 May | 35 | 14 | 49 |
| 2017 April | 20 | 7 | 27 |
| 2017 March | 21 | 1 | 22 |
| 2017 February | 15 | 4 | 19 |
| 2017 January | 8 | 6 | 14 |
| 2016 December | 15 | 10 | 25 |
| 2016 November | 10 | 4 | 14 |
| 2016 October | 10 | 4 | 14 |
| 2016 September | 4 | 1 | 5 |
| 2016 August | 2 | 2 | 4 |
| 2016 July | 3 | 6 | 9 |
| 2016 June | 9 | 8 | 17 |
| 2016 May | 15 | 2 | 17 |
| 2016 April | 24 | 1 | 25 |
| 2016 March | 32 | 23 | 55 |
| 2016 February | 53 | 27 | 80 |
| 2016 January | 35 | 23 | 58 |
| 2015 December | 32 | 17 | 49 |
| 2015 November | 34 | 9 | 43 |
| 2015 October | 30 | 19 | 49 |
| 2015 September | 34 | 21 | 55 |
| 2015 August | 22 | 13 | 35 |
| 2015 July | 15 | 1 | 16 |
| 2015 June | 21 | 7 | 28 |
| 2015 May | 31 | 12 | 43 |
| 2015 April | 39 | 42 | 81 |
| 2015 March | 75 | 41 | 116 |
| 2015 February | 65 | 0 | 65 |
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