Abstract
Objective: To evaluate in a longitudinal study the influence of airway hyperresponsiveness (AHR) on lung function in patients with primary Sjögren’s syndrome (pSS).Methods: Lung function was studied over an eight-year period in 15 patients who fulfilled the Copenhagen criteria for primary Sjögren’s syndrome and who were covered in our earlier published study on AHR in patients with Sjögren’s syndrome. Standard spirometry and measurements of lung volumes, diffusing capacity (DLCO), and AHR to methacholine were performed.Results: A significant decline over time was found in total lung capacity (TLC), vital capacity (VC), forced vital capacity (FVC), functional residual capacity (FRC), and expiratory midflows (FEF50). A sign of small airway obstruction (decrease in FEF50) at entry correlated with VC at follow-up (r = .8, P < .003), and the individual change in FEF50 during the observation period correlated with the individual change in VC (r = .6, P < .05). Six patients had increased AHR, and three of them had decreased DLCO. Six of the patients progressively reduced DLCO over time, and five of them had spirometric signs of increased small airway obstruction.Conclusions: During this eight-year follow-up we observed that one-third of the patients with pSS developed a significant reduction in lung function. Our findings suggest that small airways obstruction and AHR are associated with reduction of VC and development of impaired DLCO as a sign of interstitial lung disease in this group of patients.
Highlights
Primary Sjo€gren’s syndrome is a chronic autoimmune inflammatory disease that mainly affects exocrine glands of the mucous membranes [1]
When evaluating the airway obstruction over time, we found that FEF50 at baseline was correlated with vital capacity (VC) at follow-up (r 1⁄4 .8, P < .003), and there was an association between the FEF50 at baseline and the change (D) in VC over time (Figure 1)
The individual change in FEF50 during the observation period correlated with the individual change in VC (r 1⁄4 .6, P < .05) (Figure 2)
Summary
Primary Sjo€gren’s syndrome (pSS) is a chronic autoimmune inflammatory disease that mainly affects exocrine glands of the mucous membranes [1]. A significant number of patients with pSS have symptoms of xerotracheitis, characterized by a chronic, dry, non-productive cough and dyspnoea. These symptoms have been attributed to dryness in the large airways caused by dysfunction in the tracheal glands due to lymphocyte infiltration [7]. These xerotracheitis symptoms are similar to the symptoms of airway hyperresponsiveness (AHR). It is important to distinguish between bronchial asthma and respiratory symptoms due to pSS, as the Sjo€gren’s patients may run the risk of developing interstitial pneumonitis and small airway disease, with irreversible lung dysfunction as a possible end result [4]
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