Chest
Original ResearchDiffuse Lung DiseaseAutoimmune-Featured Interstitial Lung Disease: A Distinct Entity
Section snippets
Study Subjects
Patients referred to the ILD clinic at the University of Chicago were invited to participate in a prospective study designed to describe the clinical characteristics and disease progression of ILD patients. The study was approved by the institutional review board at the University of Chicago, and all enrolled patients provided informed consent (No. 14163A). Data from patients enrolled from September 2005 to September 2008 were available for analysis. The institutional review board approved the
Study Population
Two hundred patients completed the symptoms questionnaire and had serologic testing and composed our study group of interest. Forty-two patients had known causes of ILD and were excluded. Sixty-three subjects met the criteria for AIF-ILD. Fifty-eight patients had IPF, and 37 subjects had CTD-ILD. In this study, the prevalence of AIF-ILD was 63 in 200 patients (32%), IPF 58 in 200 (29%), and CTD-ILD 37 in 200 (19%).
Demographics of AIF-ILD, IPF, and CTD-ILD Groups
Demographic and clinical characteristics of study subjects are shown in Table 2.
Discussion
Our study shows that a systematic evaluation of symptoms and serologic tests in patients with ILD can identify AIF-ILD, which appears to represent a distinct subgroup of ILD, with different characteristics and outcomes from those of IPF and CTD-ILD. In this study of 200 patients with ILD, AIF-ILD was the most common subgroup, having a higher prevalence than IPF or CTD-ILD. Our analysis reveals that subjects with AIF-ILD have several distinct attributes.
The demographic profile revealed that the
Conclusions
In summary, we have shown that comprehensive evaluation of symptoms and serologic tests in patients referred to a tertiary care ILD clinic can identify a distinct, common, and novel group of ILD patients with autoimmune features, AIF-ILD. The predominant radiographic and histopathologic finding in this AIF-ILD population was UIP. Although clinical characteristics differed among patients with AIF-ILD, IPF, and CTD-ILD, survival for patients with AIF-ILD remained poor, similar to that of patients
Acknowledgments
Author contributions: Drs Noth and Strek take responsibility for the integrity of the work as a whole.
Dr Vij: contributed to the study design, data analysis, and preparation of the manuscript.
Dr Noth: contributed to the study design, senior authorship, and editing of the manuscript.
Dr Strek: contributed to the study design, senior authorship, and editing of the manuscript
Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Noth has
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Funding/Support: This work was supported by the National Institutes of Health, National Heart, Lung, and Blood Institute [Grant HL080513]; the Pulmonary Fibrosis Foundation (Chicago, IL); and the Coalition for Pulmonary Fibrosis (San Jose, CA).
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