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Original Research: INTERSTITIAL LUNG DISEASE |

Minor Salivary Gland Biopsy To Detect Primary Sjögren Syndrome in Patients With Interstitial Lung Disease

Aryeh Fischer, MD; Jeffrey J. Swigris, DO, MS; Roland M. du Bois, MD; Steve D. Groshong, MD, PhD; Carlyne D. Cool, MD; Hakan Sahin, MD; David A. Lynch, MD; JoAnn Z. Gillis, MD; Marc D. Cohen, MD; Richard T. Meehan, MD; Kevin K. Brown, MD, FCCP
Author and Funding Information

Affiliations: From the Autoimmune Lung Disease Center (Drs. Fischer, Swigris, du Bois, and Brown), and the Departments of Radiology (Drs. Sahin and Lynch), Pathology (Drs. Groshong and Cool), and Rheumatology (Drs. Fischer, Gillis, Cohen, and Meehan), National Jewish Medical and Research Center, Denver, CO.

Correspondence to: Aryeh Fischer, MD, Autoimmune and Interstitial Lung Disease Program, Department of Medicine, National Jewish Health, 1400 Jackson St, Denver, CO 80206; e-mail: fischera@njhealth.org


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


© 2009 American College of Chest Physicians


Chest. 2009;136(4):1072-1078. doi:10.1378/chest.08-2839
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Purpose:  To describe a cohort of patients who presented with interstitial lung disease (ILD) of unknown cause, features of primary Sjögren syndrome (pSS), and a positive minor salivary gland biopsy (MSGB).

Methods:  Thirty-eight patients with ILD evaluated at our center underwent an MSGB to confirm a diagnosis of pSS. All of the samples were reviewed by pathologists experienced in the evaluation of salivary gland histology. We defined a positive MSGB finding as a lymphocyte focus score of >1.

Results:  At presentation, all patients had ILD, and symptoms of cough and dyspnea. None had a definable connective tissue disease (CTD) or known cause for their ILD. Thirteen patients (34%) had positive MSGB findings. Of these, the median age was 61 years (age range, 33 to 75 years); 7 patients (54%) were women; 8 patients (62%) had a smoking history; and 10 patients (77%) had sicca symptoms. In all patients, a thoracic high-resolution CT scan evaluation demonstrated bibasilar, peripheral-predominant, ground-glass, and reticular opacities. Four patients (31%) were negative for both antinuclear autoantibody (ANA) and rheumatoid factor (RF) autoantibody, and three patients (23%) were negative for ANA, RF, Sjögren syndrome (SS)-A, and SS-B autoantibodies. No patients experienced any complications from the MSGB. The identification of underlying pSS did not affect the management of ILD in these patients.

Conclusions:  Confirming a diagnosis of pSS-related ILD by performing MSGB allows for a more precise CTD classification. This study provides evidence that CTD may exist subclinically, and longitudinal studies are needed to determine whether identifying occult CTD impacts on management, longitudinal changes in lung function, or survival.

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