Esophageal involvement and pulmonary manifestations in systemic sclerosis

Objective. To assess whether esophageal manometric motor disturbances are a ssociated with abnormalities consistent with interstitial lung disease (ILD ) on both pulmonary function tests (PFT) and high resolution computerized t omography (HRCT) scans in patients with systemic sclerosis (SSc), during in itial evaluation and at 2 years followup. Methods. Esophageal manometry, PFT, and HRCT scans were performed in 43 con secutive SSc patients. PFT and HRCT scan parameters were compared between p atients with severe esophageal motor dysfunction (i.e., aperistalsis and de creased low esophageal sphincter pressure), patients with moderate esophage al motor dysfunction (hypoperistalsis), and patients without esophageal mot or dysfunction on manometry. Results. During initial evaluation of SSc, patients with severe esophageal motor impairment, compared with those with moderate and without esophageal dysmotility, exhibited significantly decreased median values of diffusing c apacity for carbon monoxide (DLco) (68% vs. 94% vs. 104%) and exhibited hig her prevalence of evidence for ILD on HRCT scan (57% vs. 27% vs. 18%). At 2 years followup of SSc, patients with severe esophageal motor disturbances, compared with those without, had faster deterioration of DLco median value s (-16% vs. +1%) and higher frequency of ILD on HRCT scan (70% vs. 25%). Conclusion. Our series underscores a correlation between the degree of esop hageal manometric motor disturbances and evidence for ILD on PFT and HRCT s can in SSc patients, suggesting that gastroesophageal reflux (GER) may be o ne of the contributing factors of ILD in SSc. Our findings further indicate that patients with severe esophageal impairment may require closer followu p of lung parameters. In turn, it suggests that aggressive therapy of GER s hould be initiated in these SSc patients, as it may result in decreased det erioration of pulmonary function.