Open AccessComparison of clinical courses and mortality of connective tissue disease‐associated interstitial pneumonias and chronic fibrosing idiopathic interstitial pneumonias
Author(s) -
Yıldırım Fatma,
Türk Murat,
Bitik Berivan,
Erbaş Gonca,
Köktürk Nurdan,
Haznedaroğlu Şeminur,
Türktaş Haluk
Publication year - 2019
Publication title -
the kaohsiung journal of medical sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.439
H-Index - 36
eISSN - 2410-8650
pISSN - 1607-551X
DOI - 10.1002/kjm2.12066
Subject(s) - medicine , usual interstitial pneumonia , interstitial lung disease , idiopathic pulmonary fibrosis , dlco , idiopathic interstitial pneumonia , hazard ratio , high resolution computed tomography , connective tissue disease , pulmonary function testing , hypersensitivity pneumonitis , vital capacity , diffusing capacity , confidence interval , lung , disease , lung function , autoimmune disease
Interstitial lung disease (ILD) is a common pulmonary manifestation of connective tissue diseases (CTD). Prognostic effect of radiological usual interstitial pneumonia (UIP) pattern in CTD‐associated interstitial lung disease (CTD‐ILD) is unknown. This study aimed to investigate the disease progression and mortality of patients with CTD‐ILD and idiopathic interstitial pneumonias (IIP) including idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia and the prognostic impact of the radiological UIP pattern on both disease groups. The medical records of 91 patients (55 with CTD‐ILD and 36 with IIP) diagnosed with ILD at pulmonary medicine department, Faculty of Medicine, Gazi University from 2004 to 2014 were retrospectively reviewed. Patients included whose baseline high‐resolution computed tomography (HRCT) scans showed either a UIP or non‐UIP pattern. While 67.3% (n = 37) of CTD‐ILD patients possessed UIP pattern, 38.9% (n = 14) of IIP patients had UIP pattern in HRCT. Respiratory functions including the forced expiratory volume in the first second (FEV 1 ), functional vital capacity (FVC), and transfer coefficient for carbon monoxide (diffusing capacity of the lung for carbon monoxide [DLCO]) of IIP group at the time of diagnosis were significantly lower than CTD‐ILD group ( P = .007, P = .002, and P = .019, respectively). There was no significant survival difference between CTD‐ILD and IIP by using the log‐rank test ( P = .76). Multivariate analysis revealed that UIP pattern in HRCT (Hazard ratio: 1.85; 95% Confidence interval = 1.14‐3; P = .013), annual FVC (Hazard ratio: 0.521; 95% Confidence interval = 0.32‐0.84; P = .007), and annual DLCO declines (Hazard ratio: 0.943; 95% Confidence interval = 0.897‐0.991; P = .02) were independent risk factors for mortality in both CTD‐ILD and IIP groups. We found that UIP pattern in HRCT and annual losses in respiratory functions were the main determinants of prognosis of ILDs either idiopathic or CTD‐associated.