Colagenose pulmão dominante em pacientes com doença pulmonar intersticial: prevalência, estabilidade funcional e manifestações extratorácicas comuns

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Citações na Scopus
14
Tipo de produção
article
Data de publicação
2015
Editora
SOC BRASILEIRA PNEUMOLOGIA TISIOLOGIA
DOI
Indexadores
SciELO
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Autores
Autor de Grupo de pesquisa
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Coordenadores
Organizadores
Citação
JORNAL BRASILEIRO DE PNEUMOLOGIA, v.41, n.2, p.151-160, 2015
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
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 (CO). 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.
Palavras-chave
Idiopathic interstitial pneumonias, Autoantibodies, Connective tissue diseases, Autoimmunity
URI
https://observatorio.fm.usp.br/handle/OPI/9723
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MCP
Artigos e Materiais de Revistas Científicas - HC/InCor
Artigos e Materiais de Revistas Científicas - LIM/09
Artigos e Materiais de Revistas Científicas - ODS/03