Background/Purpose:  In previous studies in primary Sjögren’s syndrome (pSS), the prevalence of pulmonary involvement varied greatly depending on differences in inclusion criteria, imaging modalities and definitions of pulmonary involvement. The EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) has been developed for standardizing definitions of the main organ involvements. Our aim was to evaluate the prevalence and types of pulmonary involvement in pSS patients and to classify the manifestations using the respiratory domain of the ESSDAI.

Methods: This retrospective cohort study included all consecutive pSS patients, fulfilling the AECG and/or ACR classification criteria, who visited the department of Rheumatology and Clinical Immunology of the UMCG in 2015. Data were obtained from electronic patient records of the first visit in 2015. Pulmonary complaints were defined as persistent cough, dyspnoea during exercise, dyspnoea in rest and recurrent lower respiratory infections. Pulmonary additional tests included conventional chest radiography, (high-resolution; HR) CT and pulmonary function test (PFT). Pulmonary involvement was defined as the presence of conditions 1) for which therapy is needed and/or follow-up is recommended and 2) with (possible) relation with pSS instead of coincidental factors. The respiratory domain of the ESSDAI was determined for all these patients at time of visit. In some cases, the difference between pulmonary manifestations caused by pSS or coincidental factors remained unclear, resulting in a range of assumed to possible pulmonary involvement in pSS.

Results: Of the 262 included pSS patients, 93% were female and mean age was 56 ± 15 years. Pulmonary complaints were present in 88 (34%) patients; 70 (27%) patients with cough, 42 (16%) with dyspnoea during exercise, 17 (7%) with recurrent lower respiratory infections and no patients with dyspnoea in rest. Additional pulmonary diagnostics was performed in 225 (86%) patients; chest radiography in 203 (78%) patients, PFT in 147 (56%) patients and (HR)CT in 87 (33%) patients. Pulmonary involvement was present in 25-39 (10-15%) pSS patients. Overall, most common was interstitial lung disease (ILD), which was present in 15 patients; especially non-specific interstitial pneumonia (NSIP), followed by lymphocytic interstitial pneumonia (LIP) and organising pneumonia (OP). Isolated bronchiectasis and/or bronchopathy was present in 11 patients, pleuritis in 4 patients, pulmonary hypertension in 2 patients and 2 patients had a mucosa-associated lymphoid tissue (MALT) lymphoma in the lung. In total, 16 (6%) patients had a positive ESSDAI for the respiratory domain; 4 patients with low, 11 with moderate and one patient with high activity. Pulmonary involvement in pSS was not scored in the ESSDAI for patients with long lasting features (>1 year) which are assumed to be not active (n=14), MALT lymphoma which was rated in another domain (lymphadenopathy and lymphoma; n=2), or when (HR)CT and/or PFT was not recently performed (n=7).

Conclusion: In this cross-sectional study in daily clinical practice, pulmonary involvement was present in 10-15% of pSS patients. Of all pSS patients, 6% were scored as active on the respiratory domain of the ESSDAI.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-involvement-as-part-of-the-essdai-in-primary-sjogrens-syndrome/