Minor salivary gland biopsy to detect primary Sjogren syndrome in patients with interstitial lung disease.
OBJECTIVE
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.
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO; Department of Rheumatology, National Jewish Medical and Research Center, Denver, CO. Electronic address: fischera@njhealth.org.
Aryeh
A
Fischer
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO.
Jeffrey J
JJ
Swigris
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO.
Roland M
RM
du Bois
Department of Pathology, National Jewish Medical and Research Center, Denver, CO.
Steve D
SD
Groshong
Department of Pathology, National Jewish Medical and Research Center, Denver, CO.
Carlyne D
CD
Cool
Department of Radiology, National Jewish Medical and Research Center, Denver, CO.
Hakan
H
Sahin
Department of Radiology, National Jewish Medical and Research Center, Denver, CO.
David A
DA
Lynch
Department of Rheumatology, National Jewish Medical and Research Center, Denver, CO.
JoAnn Z
JZ
Gillis
Department of Rheumatology, National Jewish Medical and Research Center, Denver, CO.
Marc D
MD
Cohen
Department of Rheumatology, National Jewish Medical and Research Center, Denver, CO.
Richard T
RT
Meehan
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO.
Kevin K
KK
Brown
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D001706:Biopsy; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000000981:diagnostic imaging / Q000209:etiology; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012470:Salivary Glands, Minor / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000473:pathology*; D014057:Tomography, X-Ray Computed
D001323:Autoantibodies