Scripties UMCG - Rijksuniversiteit Groningen
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Pulmonary involvement in primary Sjögren’s syndrome

(2016) Heus, A.

Background: In previous studies in primary Sjögren’s syndrome (pSS), the prevalence of
pulmonary involvement varied greatly depending on differences in inclusion criteria and
definitions of pulmonary involvement. The ESSDAI has been developed for standardized
assessment 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
pulmonary 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. 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. Additional pulmonary diagnostics
was performed in 225 (86%) 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). In total, 16 (6%) patients had
a positive ESSDAI for the pulmonary domain; low activity (n=4), moderate activity (n=11) and
high activity (n=1).
Conclusion: In this cross-sectional study in daily clinical practice, pulmonary involvement was
present in 10-15% of pSS patients, most common ILD. Of all pSS patients, 6% were scored as
active on the pulmonary domain of the ESSDAI.

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