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Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoanti...

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Autores principales: Pereira, Daniel Antunes Silva, Dias, Olívia Meira, de Almeida, Guilherme Eler, Araujo, Mariana Sponholz, Kawano-Dourado, Letícia Barbosa, Baldi, Bruno Guedes, Kairalla, Ronaldo Adib, Carvalho, Carlos Roberto Ribeiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Pneumologia e Tisiologia 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4428852/
https://www.ncbi.nlm.nih.gov/pubmed/25972968
http://dx.doi.org/10.1590/S1806-37132015000004443
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author Pereira, Daniel Antunes Silva
Dias, Olívia Meira
de Almeida, Guilherme Eler
Araujo, Mariana Sponholz
Kawano-Dourado, Letícia Barbosa
Baldi, Bruno Guedes
Kairalla, Ronaldo Adib
Carvalho, Carlos Roberto Ribeiro
author_facet Pereira, Daniel Antunes Silva
Dias, Olívia Meira
de Almeida, Guilherme Eler
Araujo, Mariana Sponholz
Kawano-Dourado, Letícia Barbosa
Baldi, Bruno Guedes
Kairalla, Ronaldo Adib
Carvalho, Carlos Roberto Ribeiro
author_sort Pereira, Daniel Antunes Silva
collection PubMed
description OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.
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spelling pubmed-44288522015-05-13 Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features Pereira, Daniel Antunes Silva Dias, Olívia Meira de Almeida, Guilherme Eler Araujo, Mariana Sponholz Kawano-Dourado, Letícia Barbosa Baldi, Bruno Guedes Kairalla, Ronaldo Adib Carvalho, Carlos Roberto Ribeiro J Bras Pneumol Original Article OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria. Sociedade Brasileira de Pneumologia e Tisiologia 2015 /pmc/articles/PMC4428852/ /pubmed/25972968 http://dx.doi.org/10.1590/S1806-37132015000004443 Text en http://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Pereira, Daniel Antunes Silva
Dias, Olívia Meira
de Almeida, Guilherme Eler
Araujo, Mariana Sponholz
Kawano-Dourado, Letícia Barbosa
Baldi, Bruno Guedes
Kairalla, Ronaldo Adib
Carvalho, Carlos Roberto Ribeiro
Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title_full Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title_fullStr Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title_full_unstemmed Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title_short Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
title_sort lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4428852/
https://www.ncbi.nlm.nih.gov/pubmed/25972968
http://dx.doi.org/10.1590/S1806-37132015000004443
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