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Imre Noth, MD, FCCP; Rekha Vij, MD; Mary E. Strek, MD, FCCP
Author and Funding Information

From the Section of Pulmonary and Critical Care, Department of Medicine, University of Chicago.

Correspondence to: Imre Noth, MD, FCCP, Section of Pulmonary and Critical Care, Department of Medicine, University of Chicago, 5841 S Maryland Ave, MC 6076, Chicago, IL 60637; e-mail: inoth@medicine.bsd.uchicago.edu


Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Strek has received institutional grants from ImmuneWorks, Gilead, and InterMune to conduct industry-sponsored clinical trials. Dr Noth has received institutional grants from Centacor, Immuneworks, Actelion, and the National Institutes of Health for conduct of clinical trials in IPF. Dr Vij has reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Funding/Support: Dr Noth is supported by the National Institutes of Health, National Heart, Lung, and Blood Institute [Grant HL080513].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2012 American College of Chest Physicians


Chest. 2012;141(5):1361. doi:10.1378/chest.12-0026
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We thank Dr Medford for his insightful comments regarding our study1 evaluating the characteristics and survival of patients with autoimmune-featured interstitial lung disease (AIF-ILD). These patients had signs or symptoms and serologic test results suggestive of autoimmunity but did not meet the criteria for a defined connective tissue disease. Although survival for all patients with AIF-ILD was comparable to patients with idiopathic pulmonary fibrosis, the subgroup of patients with AIF-ILD and an antinuclear antibody (ANA) titer≥1:1,280 had improved survival. As Dr Medford noted, nonspecific interstitial pneumonia patterns, which are generally associated with improved outcomes,2 were seen more commonly in the subgroup of patients with AIF-ILD and high ANA titers. Nonetheless, the majority of patients with AIF-ILD and high ANA titers had a usual interstitial pneumonia pattern on CT scan and lung biopsy (59% and 73%, respectively), suggesting that radiographic and histopathologic patterns may not fully account for the differences in survival. The small number of patients with AIF-ILD and high ANA titers precluded subgroup analysis to determine the impact of radiographic and histopathologic patterns on survival. Our results underscore the significance of performing a systematic evaluation for autoimmune features in patients with interstitial lung disease regardless of radiographic and pathologic patterns, as patients with usual interstitial pneumonia may meet the criteria for AIF-ILD. More importantly, the presence of elevated ANA titers may have prognostic value.

Evaluation for underlying autoimmune disease may evolve as newer autoantibodies are discovered, as in the case of the antisynthetase syndrome.3 As Dr Medford suggests, this may also be true for patients with AIF-ILD and high ANA titers. Until then, we believe that it is crucial to evaluate for and recognize the subgroup of patients with AIF-ILD so that additional studies of their disease course, predictors of survival, and response to therapy can be performed.

We hypothesize that there is a spectrum of autoimmunity in interstitial lung disease and that the presence of autoimmunity may impact both response to treatment and survival. Current diagnostic criteria for AIF-ILD rely on clinical characteristics, some of which are nonspecific. As a result, AIF-ILD encompasses a heterogeneous group of patients: Some may have a clinical course similar to patients with idiopathic pulmonary fibrosis, while others may have a course similar to patients with connective tissue diseases. Additional studies of the molecular differences related to autoimmunity for patients with interstitial lung diseases4 may lead to the development of additional criteria that can be used to objectively distinguish between these subgroups of patients.

Role of sponsors: The sponsor had no role in the design of the study, the collection and analysis of the data, or in the preparation of the manuscript.

Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;1405:1292-1299. [CrossRef] [PubMed]
 
Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;582:143-148. [CrossRef] [PubMed]
 
Fischer A, Swigris JJ, du Bois RM, et al. Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med. 2009;10311:1719-1724. [CrossRef] [PubMed]
 
Vij R, Huang Y, Ma SF, et al. Molecular pathways associated with autoimmunity in patients with idiopathic pulmonary fibrosis. Poster presented at: Pulmonary Fibrosis Foundation IPF Summit; December 1-3, 2011; Chicago, IL.
 

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References

Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;1405:1292-1299. [CrossRef] [PubMed]
 
Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;582:143-148. [CrossRef] [PubMed]
 
Fischer A, Swigris JJ, du Bois RM, et al. Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med. 2009;10311:1719-1724. [CrossRef] [PubMed]
 
Vij R, Huang Y, Ma SF, et al. Molecular pathways associated with autoimmunity in patients with idiopathic pulmonary fibrosis. Poster presented at: Pulmonary Fibrosis Foundation IPF Summit; December 1-3, 2011; Chicago, IL.
 
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