Background In previous studies, 5 to 35% of patients with primary Sjögren's syndrome (pSS) are reported to have respiratory symptoms (RS). Pulmonary involvement varies from a dry cough due to airway dryness to life-threatening interstitial lung disease.
Objectives To evaluate RS prevalence in patients with pSS and compare characteristics of pSS patients with and without RS to those in patients without pSS suffering from ocular or oral dryness.
Methods Cross-sectional study of patients at the time of their inclusion in the OASIS cohort between 2014 and September 2016. This UK prospective research cohort includes patients with suspected pSS or known pSS and aims to collect long-term high quality data with regular clinical, dental and ophthalmological assessments. We asked systematically all the patients if they had any RS. In case of clinically significant RS, pulmonary function tests (PFTs) were requested, and if needed, a high-resolution chest tomography (HRCT) was performed. We included in the analysis only patients fulfilling the AECG (2002) criteria for pSS and excluded patients with secondary Sjögren's syndrome. Characteristics of pSS patients with and without RS and non-pSS patients with sicca symptoms were compared. For statistical analysis, we used unpaired t test, Mann-Whitney test, Fisher's exact test and Chi-square test when appropriate. P≤0.05 was considered statistically significant.
Results Among the 157 patients included in the cohort, 70 fulfil the AECG criteria for pSS and 63 have sicca symptoms without pSS. In the pSS/sicca non-pSS groups, 25.7%/15.9% had RS (cough 10.0%/7.9% and breathlessness 15.7%/6.3%) and 5.7%/1.6% an abnormal chest clinical examination respectively. PSS patients with pre-existing lung disease (n=11) had significantly more RS than pSS patients without it (n=59): 54.5% versus 20.3% (p=0.03).
PSS patients with RS or abnormal chest clinical examination (n=21) had a higher ESSDAI index value (mean ±SD) than patients without them (n=49) (7.8±5.7 versus 5.0±4.9, p=0.04), essentially due to a higher constitutional domain score (1.7±2.1 versus 0.6±1.5, p=0.01) and a higher respiratory domain score (1.3±2.8 versus 0, p<0.01). They also had a higher ESSPRI index value (mean ±SD), which is a patient reported outcome: 7.4±1.7 versus 6.0±2.1 (p=0.05). There were no differences between pSS patients with and without RS in terms of demographic characteristics, objective measurements of tear and saliva production, histological focus scores and auto-immunity profiles.
In this same group of pSS patients, 10 PFTs and 9 HRCTs were requested and showed abnormal results in 60.0% and 55.6% respectively. A reduced gas transfer was the most common finding in PFTs (DLCO mM/min/kPa, % predicted value, mean ±SD: 66.7±11.7). Among these patients, 2 patients were diagnosed with interstitial lung disease. Both had abnormal PFTs and HRCT.
Conclusions One third of pSS patients presented with respiratory symptoms or abnormal chest clinical examination at inclusion in our cohort. These patients had higher ESSDAI and ESSPRI index values but did not differ in terms of objective saliva and tear production measurements and auto-antibody profile. Reduced gas transfer was the most common abnormal finding in PFTs.
Disclosure of Interest None declared