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Evaluation and correlations of the extension of pulmonary cysts in lymphangioleiomyomatosis

Mariana Sponholz Araujo, Carolina Salim Gonçalves Freitas, Bruno Guedes Baldi, Gustavo Borges da Silva Teles, Suzana Pinheiro Pimenta, Daniel Antunes Silva Pereira, Olívia Meira Dias, André Nathan Costa, Leticia Kawano-Dourado, Alexandre Kawassaki, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho
European Respiratory Journal 2013 42: P2342; DOI:
Mariana Sponholz Araujo
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Carolina Salim Gonçalves Freitas
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Bruno Guedes Baldi
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Gustavo Borges da Silva Teles
2Department of Radiology, University of Sao Paulo Medical School, São Paulo, SP, Brazil
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Suzana Pinheiro Pimenta
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Daniel Antunes Silva Pereira
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Olívia Meira Dias
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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André Nathan Costa
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Leticia Kawano-Dourado
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Alexandre Kawassaki
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Ronaldo Adib Kairalla
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Carlos Roberto Ribeiro Carvalho
1Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Sp, Brazil
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Abstract

Introduction: The best method to assess the extension of pulmonary cysts in HRCT and whether it correlates with six-minute walk test (6MWT) variables, metalloproteinases (MMPs) or vascular endothelial growth factor-D (VEGF-D) dosage have not been defined in lymphangioleiomyomatosis (LAM). Objectives: To study the extension of pulmonary cysts in HRCT and to determine its correlations with pulmonary function tests, MMPs and VEGF-D dosage, the walking distance and dessaturation–distance ratio (DDR) in 6MWT in LAM (Pimenta, S.P. et al. Clinics 2010;65:841-6). Methods: Data from 23 women with LAM followed at the University of Sao Paulo were evaluated. The extension of pulmonary cysts in HRCT was assessed by computing the cyst volume over the entire lung volume. Spearman correlation coefficient was used to establish the association between the extension of cysts and the other variables. Results: The mean age was 46 ± 8 years. The extension of cysts in HRCT was 6.8% (median). Mean FEV1/FVC, FEV1, DLCO and RV/TLC were, respectively, 0.68 ± 0.19, 76 ± 25%pred, 65 ± 25%pred and 0.37 ± 0.08. The distance walked was 508 ± 112m, while the DDR was 4 (median). VEGF-D serum level was 464 (median). The extension of cysts correlated best with DLCO (r=-0.82, p<0.0001), FEV1/FVC (r=-0.84, p<0.0001), RV/TLC (r=0.64, p<0.0001), and DDR (r=0.76, p<0.0001). A weak correlation was found with VEGF-D (r=0.45, p=0.03). There was no correlation with the walking distance or the level of MMPs. Conclusions: The severity of pulmonary cystic involvement was mild and correlated with airway obstruction, air trapping, DLCO, and DDR. The use of VEGF-D and MMPs to assess the severity of LAM remains controversial.

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Evaluation and correlations of the extension of pulmonary cysts in lymphangioleiomyomatosis
Mariana Sponholz Araujo, Carolina Salim Gonçalves Freitas, Bruno Guedes Baldi, Gustavo Borges da Silva Teles, Suzana Pinheiro Pimenta, Daniel Antunes Silva Pereira, Olívia Meira Dias, André Nathan Costa, Leticia Kawano-Dourado, Alexandre Kawassaki, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho
European Respiratory Journal Sep 2013, 42 (Suppl 57) P2342;

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Evaluation and correlations of the extension of pulmonary cysts in lymphangioleiomyomatosis
Mariana Sponholz Araujo, Carolina Salim Gonçalves Freitas, Bruno Guedes Baldi, Gustavo Borges da Silva Teles, Suzana Pinheiro Pimenta, Daniel Antunes Silva Pereira, Olívia Meira Dias, André Nathan Costa, Leticia Kawano-Dourado, Alexandre Kawassaki, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho
European Respiratory Journal Sep 2013, 42 (Suppl 57) P2342;
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