THU0353 Lung ultrasound of pleural irregularities (PI-US) in primary sjÖgren’s syndrome (PSS)-associated interstitial lung disease(ILD): clinical, functional, radiographic and ultrasonographic short-term follow-up
Authors: F. Ferro, A. Bulleri, A. Delle Sedie, S. Barsotti, N. Luciano, E. Elefante, M. Mosca, C. Baldini
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Ultrasound of Pleural Irregularities (PI-US) has recently been suggested as a useful tool for the diagnosis and the assessment of interstitial lung disease (ILD) in primary Sjögren's Syndrome (pSS). However, no data are available regarding its role in the post-therapy evaluation of pSS-ILD. <h3>Objectives</h3> Aim of this study was to describe the post-therapeutic changes of the pleural profile in a monocentric cohort of pSS-ILD patients analysing clinical, functional, US and radiographic features. <h3>Methods</h3> Eighteen patients with pSS-ILD were included in the study. PI-US, HRCT and pulmonary function test (PFT) were performed in each patient at baseline. In 15/18 patients who had been treated according to international guidelines for ILD, clinical assessment, PFT and PI-US were repeated after 6 months. PI-US was performed by a single operator using a MyLab-25 (Esaote), 10 MHz, 5 cm linear probe. PI was defined as the loss of normal hyperechoic linear pleural contour (score 0–2: normal, minimal and major changes at each intercostal space). PI-US total score represented the sum of partial scores assigned to 6 lung fields (2 for the anterior, 2 for postero-superior and 2 for postero-inferior chest surface). HRCT- abnormal findings, distinguishing C-NSIP, F-NSIP and UIP pattern, were quantified using the Warrick score system. <h3>Results</h3> Eighteen pSS-ILD patients (14 F:4 M, mean age=68.8±9.9 years) were included in the study. The median PI-US score was 45 (range 25.5–73.5). Both PI-US total score and partial postero-inferior PI-US score strongly correlated with the Warrick HRCT score (r=0.813, p=0.000 and r=0.914, p=0.000) and inversely correlated with FVC (r=-0.849, p=0.000 and r=-0.836, p=0.000), TLC (r=-0.895, p=0.000 and r=-0.829, p=0.000), and DLCO (r=-0.953, p=0.000 and r=-0.883, p=0.001). Both PI-US score and PI-US of the poster-inferior field directly correlated with FEV1/SVC (r=0.701, p=0.004 and r=0.619, p=0.01) and with FEV1/FVC (r=0.600, p=0.02 and r=0.501, p=0.05). After 6 months of therapy, 15 patients (12F:3M, mean age 65.3 years) presenting at HRCT different ILD pattern distributions (4 C-NSIP, 7 F-NSIP, 4 UIP), were re-evaluated after appropriate medical treatment. Out of these 15 patients, 5 had been treated with glucocorticoids (GC) alone, 2 with azathioprine, 4 with hydroxychlorochine, 2 with Mycophenolate Mofetil, 1 with Rituximab and 1 with abatacept. Clinical, radiographic and functional evaluation showed stability of all parameters in the majority of the patients (13/15), maintaining unchanged the correlations with PI-US scores. Moreover, 2/15 patients with active C-NSIP pattern, showed a significant improvement in clinical, radiographic and functional parameters. In these patients a significant reduction of the PI-US scores was also observed (PI-US score from 22 to 10 and from 57 to 30). <h3>Conclusions</h3> This study confirms the usefulness of PI-US evaluation for the diagnosis and assessment of pSS-ILD, demonstrating a strong correlation between PI-US and both HRCT findings and PFT. Furthermore, the significant reduction of PI-US score in patients with clinical, radiographic and functional improvement suggests that this tool may play a role in the follow-up of treated patients with active pSS-ILD. <h3>Disclosure of Interest</h3> None declared