Undifferentiated Connective Tissue Disease-Associated Interstitial Lung Disease: Changes in Lung Function

作者：Kinder Brent W^*; Shariat Cyrus; Collard Harold R; Koth Laura L; Wolters Paul J; Golden Jeffrey A; Panos Ralph J; King Talmadge E Jr

来源：Lung, 2010, 188(2): 143-149.

DOI：10.1007/s00408-009-9226-7

• Idiopathic interstitial pneumonia
• Systemic rheumatic disease
• Undifferentiated connective tissue
• Collagen vascular disease
• Autoimmune disease

Undifferentiated connective tissue disease (UCTD) is a distinct clinical entity that may be accompanied by interstitial lung disease (ILD). The natural history of UCTD-ILD is unknown. We hypothesized that patients with UCTD-ILD would be more likely to have improvement in lung function than those with idiopathic pulmonary fibrosis (IPF) during longitudinal follow-up. We identified subjects enrolled in the UCSF ILD cohort study with a diagnosis of IPF or UCTD. The primary outcome compared the presence or absence of a a parts per thousand yen5% increase in percent predicted forced vital capacity (FVC) in IPF and UCTD. Regression models were used to account for potential confounding variables. Ninety subjects were identified; 59 subjects (30 IPF, 29 UCTD) had longitudinal pulmonary function data for inclusion in the analysis. After accounting for baseline pulmonary function tests, treatment, and duration between studies, UCTD was associated with substantial improvement in FVC (odds ratio = 8.23, 95% confidence interval, 1.27-53.2; p = 0.03) during follow-up (median, 8 months) compared with IPF. Patients with UCTD-ILD are more likely to have improved pulmonary function during follow-up than those with IPF. These findings demonstrate the clinical importance of identifying UCTD in patients presenting with an "idiopathic" interstitial pneumonia.

• 出版日期2010-4