Background/Purpose: Although lung disease can occur with primary Sjogren’s syndrome (pSS), far too little is known about the clinical phenotype or natural history of its lung involvement. The objective of this retrospective observational study was to characterize a cohort of subjects with pSS-associated lung disease. It also seems that there is an association between pSS and nontuberculousis mycobacterial (NTM) infection.

Methods:  We identified all subjects evaluated at our center between Jan. 2008 – May 2012 that fulfilled the American-European classification criteria for the diagnosis of pSS and that had respiratory symptoms along with thoracic high resolution CT (HRCT) available to review. All clinical data were extracted from the medical record by comprehensive review. We excluded patients with secondary Sjogren’s syndrome.

Results: A total of 38 subjects were identified. Mean age was 63.7 years (range 50-78) and all but one of the subjects were women. A history of past smoking was reported in 14 (37%) and only one subject was an active smoker.  All reported xerostomia and keratoconjunctivitis sicca  Other pSS associated manifestations included the presence of Reynaud’s in 5 (13%), arthritis in 8 (21.0%), arthralgias in 31 (82%), and esophageal reflux in 27 (71%).  All of the subjects had positive SS-A and/or SS-B antibodies. 37 were SS-A positive, 26 were SS-B positive and 25 had both positive. (Mean SS-A & SS-B were 106.5 and 78.1 units respectively) Also all of the patients had positive ANA (titer range 1:160-1:2560) by direct immunofluorescence.  ANA patterns identified; 26 (68.4%) speckled, 1 (2.6%) homogeneous, 1 (2.6%) centromere, 6 (15.7%) speckled and nucleolar, and 4 (10.5%) speckled and homogeneous. 22 were rheumatoid factor positive.  All of the subjects had respiratory symptoms as manifested either by isolated cough (n=33, 87%), dyspnea (n=36, 95%) or both.

12(31.6%) patients had proven infection with Nontuberculousis Mycobacteriosis.(NTM)

High resolution CT (HRCT) of the chest showed 24 (63.1%) patients with airway disease including 1 (2.6%) with large airway disease, 3 (7.9%) with bronchiolitis, 3 (7.9%) with bronchiolitis plus bronchiectasis and 17 (44.7) with bronchiectasis out of which 11 (28.9%) had proven NTM infection as well. 

4 (10.5%) patients were found to have interstitial lung disease (ILD); 3 (7.9%) patients with lymphoid interstitial pneumonia (LIP) and 1 (2.6%) patient with nonspecific interstitial pneumonia (NSIP).

10 (26.3) patients had combined ILD and airway disease including 1 (2.6%) with LIP, bronchiectasis and bronchioloitis; 4 (10.5%) with LIP and bronchiectasis; 3 (7.9%) with NSIP and bronchiolitis and 2 (5.3%) with NSIP and bronchiectasis. One (2.6%) of the patients with LIP and bronchiectasis also had concomitant NTM infection.

Conclusion: There is a myriad of pulmonary manifestations associated with pSS. In this cohort, the most common lung manifestation was airway disease. It also seems that pSS may predispose patients to NTM infection and NTM having a causative effect on worsening their lung condition. Further studies are needed to better define the natural history of pSS-associated lung disease and possible relationship with NTM infection especially among middle age and elderly women.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/high-resolution-ct-findings-and-concomitant-nontuberculosis-mycobacterial-infection-ntm-in-patients-with-the-diagnosis-of-primary-sjogrens-syndrome-evaluated-at-a-respiratory-referral-center/