THU0404 Esophageal involvement predicts pulmonary function deterioration in patients with systemic sclerosis
Authors: E. De Lorenzis, G. Natalello, L. Berardini, G. Canestrari, L. Verardi, L. Gigante, S. Bosello, L. Richeldi, G. Ferraccioli, E. Gremese
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Interstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc) but its pathogenesis and the risk factors of pulmonary function deterioration are not fully understood. Esophageal disease is high frequent in SSc and motor activity abnormalities with occult micro-aspiration of both acid and non-acid gastro oesophageal reflux has been implicated in the pathogenesis of ILD. DL<sub>CO</sub> reduction is considered the earliest sign of microaspiration-induced lung damage<sup>1</sup>. Cross-sectional studies have demonstrated an association of SSc-ILD and esophageal abnormalities on 24 hours intraesophageal pH-monitoring and esophageal manometry but prospective evaluation of lung deterioration is lacking<sup>2,3</sup>. Esophagogram was proposed as a useful tool to evaluate disease severity of upper gastrointestinal tract involvement in SSc<sup>4</sup>. <h3>Objectives</h3> To assess the role of esophagogram in predicting pulmonary function test deterioration in SSc-patients. <h3>Methods</h3> We retrospectively evaluated 160 consecutive SSc patients who underwent esophagogram because of suspected upper gastro-intestinal involvement. All patients underwent baseline pulmonary function tests and global clinical evaluation. Eighty-five patients underwent a High Resolution CT within 3 months from esophagogram because of suspected lung involvement. One hundred twenty three patients underwent pulmonary function test every 6 months up to 24 months. <h3>Results</h3> Seventy five patients (46.9%) presented abnormalities of peristaltic waves, 50 patients (31.2%) showed structural changes (hypotonic oesophagus or dilatation) while indirect signs of cardial incontinence (patent cardia or gastro-esophageal reflux) were present in 36 patients (22.5%). A reduced peristaltic activity with a prolongation of transit time was associated to reduced DLCO (50.16%±19.80% vs 60.36±22.69%, p=0.002) and TLC (87.05%±20.43% vs 95.09±20.59%, p=0.017). An hypotonic oesophagus was reported in 25.2% of patients and it was associated to ILD on CT (72.0% vs 28.0%, p=0.033). Patients with hypotonic oesophagus presented a reduced FVC (84.63%±22.86% vs 102.93±21.40%, p&lt;0.0001), TLC (79.85%±19.62% vs 95.29±19.80, p&lt;0.0001) and DLCO (42.88%±20.00% vs 59.89±20.78%, p&lt;0.0001) at baseline and to a faster deterioration of DLCO median values (5.10%±20.61% vs −4.77±14.23%,p=0.012) at follow-up. Patients with hypotonic oesophagus have an higher prevalence of diffuse skin disease and ongoing immunosuppressive treatment, but were comparable in term of age, sex, BMI, smoking habits, disease duration and prevalence of autoantibodies to the patients without this alteration. <h3>Conclusions</h3> The esophagogram is wide available, well tolerated and inexpensive tool to assess upper gastro-intestinal tract involvement and its abnormalities are associated to SSc-ILD severity. Because of a faster deterioration of lung function is associated to esophagogram abnormalities, a complete gastro-intestinal evaluation in ILD-SSc patients is mandatory. <h3>References</h3> [1] Schachter LM, et al. Chest2003. [2] Christmann RB, et al. Semin Arthritis Rheum2010. [3] Marie I, et al. Arthritis Care Res2001. [4] Medsger TA, et al. Clin Exp Rheumatol2003. <h3>Disclosure of Interest</h3> None declared