Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease.
Turk J Med Sci
; 52(1): 83-88, 2022 Feb.
Article
em En
| MEDLINE
| ID: mdl-36161593
ABSTRACT
BACKGROUND:
To explore the frequency and clinical associations of radiologic pleuroparenchymal fibroelastosis (PPFE) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).METHODS:
In this single-center retrospective study, high resolution computed tomography (HRCT) images of 105 patients with SSc-ILD were examined for the presence of PPFE. Demographic, clinical, laboratory, and pulmonary function test (PFT) data of patients with and without PPFE were compared.RESULTS:
PPFE was detected in 19 (18.1%) patients ('definite PPFE' in 13 and 'consistent with PPFE' in 6 patients). Patients with PPFE had higher age and longer disease duration than PPFE (-) patients (p < 0.05 for both). Radiologic usual interstitial pneumoniae (UIP) pattern was more frequent (26.3% vs. 4.7%, p = 0.01) and median force vital capacity (FVC) was lower in patients with PPFE (64% vs. 82%, p = 0.005). Spontaneous pneumothorax developed in one patient with PPFE. More deaths occured in PPFE (+) group during follow-up (31% vs. 11%, p = 0.04).Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Escleroderma Sistêmico
/
Doenças Pulmonares Intersticiais
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Fibrose Pulmonar Idiopática
Tipo de estudo:
Observational_studies
/
Risk_factors_studies
Limite:
Humans
Idioma:
En
Revista:
Turk J Med Sci
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Turquia