Abstract:

Objective To improve the awareness and diagnosis of connective tissue disease (CTD) with pulmonary diseases. Methods The clinical manifestations of the respiratory system, pulmonary function, and characteristics of lung imaging of 678 patients with CTD were retrospectively analyzed. The pathological types of pulmonary lesions of patients with interstitial lung disease associated with connective tissue disease (CTDILD) were determined. Results Among 678 patients with CTD, 117 patients (17.3%) were with pulmonary diseases. Pulmonary diseases mainly occurred in the course of (32.1±19.2) months. The first symptom of 17 cases (2.5%) was pulmonary manifestations. Pulmonary diseases associated with CTD included interstitial lung disease (94 cases, 13.9%), pulmonary hypertension (11 cases, 1.6%), pleural effusion (46 cases, 6.9%), pleural thickening (6 cases, 0.9%), and infection (6 cases, 0.9%). The primarily respiratory symptoms of CTD-ILD were dry cough (63.8%), dyspnea (43.6%), chest pain (13.8%), and hemoptysis (2.1%). The clinical manifestations of 62.8% of patients were chronic; 36.2% were asymptomatic; and only one (with dermatomyositis/polymyositis) was acute. According to the high-resolution computed tomography (HRCT), pathological types of CTD-ILD were non-specific interstitial pneumonia (70.2%), desquamative interstitial pneumonia (17.0%), and usual interstitial pneumonia (9.6%). Conclusion CTD is often associated with pulmonary diseases and the most common one is CTD-ILD. The clinical manifestations of the respiratory system of most CTD patients with pulmonary diseases are not typical. HRCT is helpful for the diagnosis of CTD-ILD and determination of its pathological types.

Key words: connective tissue disease, pulmonary disease, interstitial lung disease, clinical manifestations, high-resolution computed tomography