Abstract
Objective
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.
Results
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.
Conclusion
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)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Mayo Clinic ProceedingsAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management.Am J Respir Crit Care Med. 2011; 183: 788-824
- The clinical course of patients with idiopathic pulmonary fibrosis.Ann Intern Med. 2005; 142: 963-967
- Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement.Eur Respir J. 2009; 33: 882-896
- Usual interstitial pneumonia: idiopathic pulmonary fibrosis versus collagen vascular diseases.Respiration. 2001; 68: 151-159
- Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes.Am J Respir Crit Care Med. 2007; 175: 705-711
- Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia.Chest. 2009; 136: 23-30
- Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS).Am J Respir Crit Care Med. 2000; 161: 646-664
- Positive antinuclear factor in patients with unexplained pulmonary fibrosis.Ann Int Med. 1969; 70: 1135-1145
- Serologic factors in idiopathic diffuse interstitial pulmonary fibrosis.Am J Med. 1965; 39: 405-410
- Circulating immune complexes in the idiopathic interstitial pneumonias.N Engl J Med. 1978; 298: 353-357
- Definition and clinical relevance of antibodies to nuclear ribonucleoprotein and other nuclear antigens in patients with cryptogenic fibrosing alveolitis.Am Rev Respir Dis. 1984; 130: 439-443
- Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis.Am J Respir Crit Care Med. 1998; 157: 199-203
- Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia.Eur Respir J. 2002; 19: 1114-1118
- Clinical significance of histological classification of idiopathic interstitial pneumonia.Eur Respir J. 2002; 19: 275-283
- Radiological versus histological diagnosis in UIP and NSIP: survival implications.Thorax. 2003; 58: 143-148
- Idiopathic pulmonary fibrosis: spectrum of high-resolution CT findings.AJR Am J Roentgenol. 2005; 185: 1531-1539
- Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung.Radiology. 1997; 205: 229-234
- Autoimmune-featured interstitial lung disease: a distinct entity.Chest. 2011; 140: 1292-1299
- Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?.Am J Respir Crit Care Med. 2007; 176: 691-697
- Standardisation of spirometry.Eur Respir J. 2005; 26: 319-338
- Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis.Am J Respir Crit Care Med. 2010; 181: 832-837
- Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia.Chest. 2003; 124: 1215-1223
- Significance of connective tissue disease features in idiopathic interstitial pneumonia.Eur Respir J. 2012; 39: 661-668
- Interstitial lung disease in connective tissue disorders.Lancet. 2012; 380: 689-698
- Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders?.Eur Respir J. 2011; 38: 384-391
- Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease.Respir Med. 2009; 103: 1152-1158
- Progressive preclinical interstitial lung disease in rheumatoid arthritis.Arch Intern Med. 2008; 168: 159-166
- Prevalence and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis.Respir Med. 2013; 107: 249-255
- Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function.Lung. 2010; 188: 143-149
- A multidimensional index and staging system for idiopathic pulmonary fibrosis.Ann Intern Med. 2012; 156: 684-691
Article Info
Identification
Copyright
© 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
