Comparison of clinical courses and mortality of connective tissue disease‐associated interstitial pneumonias and chronic fibrosing idiopathic interstitial pneumonias

• Published in: The Kaohsiung journal of medical sciences Vol. 35; no. 6; pp. 365 - 372
• Main Authors: Yıldırım, Fatma, Türk, Murat, Bitik, Berivan, Erbaş, Gonca, Köktürk, Nurdan, Haznedaroğlu, Şeminur, Türktaş, Haluk
• Format: Journal Article
• Language: English
• Published: BP, Asia Wiley Publishing Asia Pty Ltd 01-06-2019; John Wiley & Sons, Inc; Wiley
• Subjects: Aged; Bacterial pneumonia; Carbon monoxide; Carbon Monoxide - metabolism; Cardiovascular disease; Chronic obstructive pulmonary disease; connective tissue disease‐associated interstitial lung disease; Development and progression; Disease Progression; Dyspnea; Female; Forced Expiratory Volume; high‐resolution computed tomography; Humans; idiopathic interstitial pneumonia; Idiopathic Interstitial Pneumonias - diagnostic imaging; Idiopathic Interstitial Pneumonias - mortality; Idiopathic Interstitial Pneumonias - physiopathology; Idiopathic Pulmonary Fibrosis - diagnostic imaging; Idiopathic Pulmonary Fibrosis - mortality; Idiopathic Pulmonary Fibrosis - physiopathology; Lung - diagnostic imaging; Lung - physiopathology; Lung diseases; Lung Diseases, Interstitial - diagnostic imaging; Lung Diseases, Interstitial - mortality; Lung Diseases, Interstitial - physiopathology; Male; Medical prognosis; Middle Aged; Mortality; Multivariate Analysis; Pneumonia; Prognosis; Pulmonary fibrosis; Regression analysis; Retrospective Studies; Rheumatoid arthritis; Risk factors; Smooth muscle; Statistical analysis; Survival Analysis; Tomography; Tomography, X-Ray Computed; usual interstitial pneumonia; Vital Capacity; usual interstitial pneumonia; connective tissue disease-associated interstitial lung disease; high-resolution computed tomography; prognosis; idiopathic interstitial pneumonia
• ISSN: 1607-551X; 2410-8650
• DOI: 10.1002/kjm2.12066

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 (FEV1), 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.