Esophageal involvement and pulmonary manifestations in systemic sclerosis

To assess whether esophageal manometric motor disturbances are associated with abnormalities consistent with interstitial lung disease (ILD) on both pulmonary function tests (PFT) and high resolution computerized tomography (HRCT) scans in patients with systemic sclerosis (SSc), during initial evaluation and at 2 years followup.

Esophageal manometry, PFT, and HRCT scans were performed in 43 consecutive SSc patients. PFT and HRCT scan parameters were compared between patients with severe esophageal motor dysfunction (i.e., aperistalsis and decreased low esophageal sphincter pressure), patients with moderate esophageal motor dysfunction (hypoperistalsis), and patients without esophageal motor dysfunction on manometry.

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 capacity for carbon monoxide (DLco) (68% vs. 94% vs. 104%) and exhibited higher 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 values (-16% vs. +1%) and higher frequency of ILD on HRCT scan (70% vs. 25%).

Our series underscores a correlation between the degree of esophageal manometric motor disturbances and evidence for ILD on PFT and HRCT scan in SSc patients, suggesting that gastroesophageal reflux (GER) may be one of the contributing factors of ILD in SSc. Our findings further indicate that patients with severe esophageal impairment may require closer followup of lung parameters. In turn, it suggests that aggressive therapy of GER should be initiated in these SSc patients, as it may result in decreased deterioration of pulmonary function.