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Frequency and Implication of Autoimmune Serologies in Idiopathic Pulmonary Fibrosis



      To assess the frequency and clinical implications of positive autoimmune serologies in patients with biopsy-confirmed idiopathic pulmonary fibrosis (IPF).

      Patients and Methods

      We reviewed the records of patients at our institution with biopsy-confirmed usual interstitial pneumonia (UIP) from January 1, 1995, through December 31, 2010, for frequency and distribution of autoimmune serologies. Patients with IPF with and without positive serologies were compared.


      Three hundred eighty-nine consecutive patients with biopsy-confirmed IPF underwent serologic testing, with positive serologic test results being found in 112 (29%). Of 2051 individual screening serologic tests performed, results of 163 tests were positive (8%), with antinuclear antibody being the most frequent (47%). There was no difference in age at biopsy (P=.21), gender (P=.21), or presenting radiologic features between those with or without positive serology. More frequent use of immunosuppressive treatment (P=.02) was noted in those with positive serology. No survival difference was observed (log-rank; P=.43). Median follow-up for the whole cohort was 43.5 months.


      Positive autoimmune serology may occur in as much as one-third of the patients with biopsy-confirmed IPF with no associated clinical implication or survival advantage. Systematic use of autoimmune laboratory panels in patients without clinical features of connective tissue disease should be reconsidered in patients with suspected UIP on chest computed tomography scan or confirmed UIP on biopsy.

      Abbreviations and Acronyms:

      ANA (antinuclear antibody), CT (computed tomography), CTD (connective tissue disease), CTD-ILD (connective tissue disease-associated interstitial lung disease), DLCO (lung diffusing capacity for carbon monoxide), FVC (forced vital capacity), HRCT (high-resolution computed tomography), ILD (interstitial lung disease), IPF (idiopathic pulmonary fibrosis), NSIP (nonspecific interstitial pneumonia), RF (rheumatoid factor), UIP (usual interstitial pneumonia)
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