Abstract: The objectives of the study were to characterize the clinical manifestations and data of laboratory tests of 35 patients with collagen vascular disease with pulmonary fibrosis (CVD-PF) , and then to improve the diagnosis and treatment of CVD-PF. Aretrospective review was carried out in 35 patients with the diagnosis of CVD-PF. Most CVD-PF patients were older than sixty years old and had a cough, expectoration and insidious onset of progressive dyspnea. Inspiratory crackles were noted on auscultation in most patients. The most impressive appearance of their radiography was peripheral reticular opacities, most profuse at the lung bases. Pulmonary function test was consistent with restrictive impairment and impaired gas exchange. The resting arterial blood gases revealed hypoxemia and respiratory alkalosis. All patients were excluded of other interstitial lung disease by history, physical examination and laboratory tests. Pulmonary fibrosis is more common in three diseases: rheumatoid arthritis, progressive systemic sclerosis and Sjogren's syndrome. The clinical manifestations, pulmonary function and chest radiographs or high-resolution CT scans are similar with idopathic pulmonary fibrosis. The definite diagnosis of CVD-PF includes the following: 1) Exclusion of other known cases of interstitial lung disease; 2) Abnormal pulmonary function including evidence of restriction and /or impaired gas exchange; 3) Abnomalities on conventional chest radiographs or high-resolution CT scans.

Key words: collagen vascular disease, pulmonary fibrosis, clinical analysis