Comparison of Characteristics of Connective Tissue Disease-Associated Interstitial Lung Diseases, Undifferentiated Connective Tissue Disease-Associated Interstitial Lung Diseases, and Idiopathic Pulmonary Fibrosis in Chinese Han Population: A Retrospective Study

Lin Pan,Yuan Liu,Guixiu Shi,Mingyu Fan,Rongfei Sun

doi:10.1155/2013/121578

Abstract

Our study compared the prevalence and characteristics of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD), or idiopathic pulmonary fibrosis (IPF) between January 2009 and December 2012 in West China Hospital, western China. Patients who met the criteria for ILD were included and were assigned to CTD-ILD, UCTD-ILD, or IPF group when they met the criteria for CTD, UCTD, or IPF, respectively. Clinical characteristics, laboratory tests, and high-resolution CT images were analyzed and compared among three groups. 203 patients were included, and all were Han nationality. CTD-ILD was identified in 31%, UCTD-ILD in 32%, and IPF in 37%. Gender and age differed among groups. Pulmonary symptoms were more common in IPF, while extrapulmonary symptoms were more common in CTD-ILD and UCTD-ILD group. Patients with CTD-ILD had more abnormal antibody tests than those of UCTD-ILD and IPF. Little significance was seen in HRCT images among three groups. A systematic evaluation of symptoms and serologic tests in patients with ILD can identify CTD-ILD, UCTD-ILD, and IPF.

Highlights

Interstitial lung disease (ILD) is a heterogeneous group of parenchymal lung disorders that result from variable etiologies but share common radiologic, pathologic, and clinical manifestations [1]
Study Population. 207 patients were diagnosed as ILD during the study period, and all were Han patients. 4 patients with environmental exposures and other known causes of ILD were excluded. 203 patients were included in our study. 63 patients met the criteria for Connective tissue disease-associated ILD (CTD-ILD), 65 patients met the criteria for undifferentiated connective tissue disease (UCTD)-ILD, and 75 patients met the criteria for idiopathic pulmonary fibrosis (IPF)
The percentage of male patients was 31.7% in the CTD-ILD group, significantly lower than the UCTD-ILD (63.1%) and IPF group (69.3%), and the percentage of ever smoker was significantly lower in CTD-ILD group than in the UCTD-ILD and IPF group, which indicate that young female ILD patients were more prone to be CTD-ILD patients, while older male ILD patients with smoking history were more prone to be UCTD-ILD and IPF patients

Summary

Introduction

Interstitial lung disease (ILD) is a heterogeneous group of parenchymal lung disorders that result from variable etiologies but share common radiologic, pathologic, and clinical manifestations [1]. Several rheumatologic conditions are associated with the development of ILD [6] These diseases include systemic sclerosis (SSc), rheumatoid arthritis (RA), polymyositis/dermatomyositis (PM/DM), Sjogren’s syndrome, systemic lupus erythematosus (SLE), and mixed connective tissue disease(CTD) [1]. A large number of ILD patients who have one or several features of systemic autoimmune disease but do not fulfill American College of Rheumatology (ACR) classification criteria for defined CTD have been classified [8]. These patients are considered to have undifferentiated connective tissue disease (UCTD) and take up as many as 25% of ILD patients as reported [8]

Methods

Results

Conclusion