• Alper Sarı, Ömer Önder, Berkan Armağan, Ertuğrul Çağrı Bölek, Bayram Farisoğulları, Emre Bilgin, YardımcıGözde KübraGKDepartment of Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Macit Arıyürek, and Ali Akdoğan.
• Department of Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
• Turk J Med Sci. 2022 Feb 1; 52 (1): 838883-88.

BackgroundTo explore the frequency and clinical associations of radiologic pleuroparenchymal fibroelastosis (PPFE) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).MethodsIn 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.ResultsPPFE 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).

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