Clinical characteristics and treatment outcome of 147 cases of common connective tissue disease-related interstitial lung disease / 西安交通大学学报(医学版)

ABSTRACT

Objective: To investigate the clinical and imaging features of common connective tissue disease-related interstitial lung disease (CTD-ILD). Methods: We made a retrospective analysis of 147 cases of common CTD-ILD patients hospitalized in the Department of Rheumatology of The First Affiliated Hospital of Xi'an Jiaotong University from June 2014 to December 2016. Their clinical data including general information, manifestations, blood gas analysis, pulmonary function test, lung high resolution computed tomography (HRCT) were analyzed. Results: The average age of the 147 patients with common CTD-ILD was (56.2±12.3) years and the majority of the patients were women (72.1%). Skin lesions (51.7%) and fatigue (50.3%) were the most common clinical manifestations, which were higher than respiratory symptoms such as dyspnea (47.6%), expectotation (36.0%), and cough (32.0%). Nearly half (49.6%) of the patients had hypoxemia, and pulmonary function changes were mostly manifested as mixed ventilation dysfunction with diffuse dysfunction. Pulmonary imaging was characterized by ground-glass opacity (78.3%). The most common radiographic type was non-specific interstitial pneumonia (NSIP), accounting for 67.2%. During the follow-up period of 1-3.5 years, the mortality rate was 26.2%. PM/DM (66.7%) was higher than SSc (15.1%), RA (15.1%) and SS (3.0%). The main causes of death were infections (42.4%) and acute exacerbation of CTD-ILD (33.3%). Conclusion: The common CTD-ILD is predominant in women. The incidence of systemic symptoms such as skin changes and fatigue are higher than that of respiratory symptoms such as shortness of breath, cough and sputum. The mortality rate is high and the main causes are infections and acute exacerbation of CTD-ILD. Therefore, blood gas analysis, pulmonary function test and lung HRCT examination are helpful for diagnosis and treatment of CTD-ILD.