Lung involvement in patients with primary Sjögren’s syndrome: what are the predictors?

Abstract

The aim of this study was to investigate the prevalence, predictors and radiological findings of primary Sjögren's syndrome (pSS)-associated lung involvement. This retrospective cohort study included 123 patients with demographic, clinical, laboratory and radiological data who were diagnosed with pSS. Lung involvement was defined based on the presence of pulmonary signs/symptoms and/or impaired pulmonary function tests along with alterations in high-resolution computerized tomography (HRCT). Thirty patients (24.4%) had pulmonary signs/symptoms at the initial presentation and/or during the follow-up period. Based on the criteria, 14 patients (11.4%) were defined as having pSS with lung involvement. The smoking rate, male/female ratio and the mean ages were found to be higher in patients with lung involvement (P < 0.05). Positive IgM-rheumatoid factor (RF), anti-La and anti-Ro results, the presence of hypergammaglobulinemia and lymphopenia had high specificity despite the low sensitivity rates to detect pSS-associated lung disease. A significant difference was found in forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV(1)) results between the patients with and without lung involvement. Impaired FEV(1) had high specificity and positive predictive value compared to impaired FVC, particularly in non-smoker patients. The most frequent HRCT finding was ground-glass attenuation (64.3%). Other common findings were bronchiectasis, reticular pattern and honeycombing. The lesions involved predominantly the lower lobes. In conclusion, the presence of hypergammaglobulinemia and lymphopenia, positivity for RF, anti-La and anti-Ro, and impaired (FVC) and/or FEV(1) values could be the predictive parameters with a high specificity despite the low sensitivity rates. Smoking history, male gender and age are also risk factors. These parameters may be helpful to distinguish pSS-associated lung involvement from lung disorders unrelated to pSS.

Chest HRCT findings in patients with primary Sjögren’s syndrome

“…At the beginning of the study, changes were observed only in 6% of patients and after 75 months the percentage increased to 15%. The mean total ESSDAI score for this domain was low (2)(3)(4)(5). Older patients at the time of diagnosis (> 70 years) were less active during follow-up, but had a higher pulmonary activity score.…”

“…The prevalence of respiratory symptoms in pSS varies a lot (9-75%), depending on the publications and assessment methods (clinical symptoms, chest X-ray, computed tomography). 2 High Resolution Computed Tomography (HRCT) is a relatively noninvasive method and is currently the most important method for detecting early lung parenchymal abnormalities and reduced lung function. Common HRCT findings of pSS in the lungs include ground-glass attenuation, bronchiectasis, a reticular pattern and honeycomb appearance.…”

Clinical and laboratory profiles of primary Sjogren's syndrome in a Chinese population: A retrospective analysis of 315 patients

“…ILD is the most common complication of pSS and the incidence of ILD varied in many studies. 18,26,27 The clinical diagnosis of pSS-ILD is somewhat difficult, because the majority of patients lack specific respiratory symptoms. Diagnostic approaches for pSS-ILD include: (i) chest X-rays; (ii) pulmonary function tests; (iii) HRCT; (iv) bronchoalveolar lavage; (v) nuclide examination; and (vi) lung biopsy, which are the gold standard for diagnosis.…”

“…The number of new cases from 1999 to 2012 was 3,7,7,7,12,15,15,11,26,33,43,46, 58 and 32, respectively, which showed a gradual upward trend (Fig. 1).…”

Interstitial Lung Diseases in Rheumatology Practice: A Single Center Experience

“…Females are also prone to other autoimmune diseases as well (6,15,27,28). We think that both CTD and CTD-OLD are more frequent in females.…”