Abstract

Rationale: Idiopathic pulmonary fibrosis (IPF) identifies patients with pulmonary fibrosis in a usual interstitial pattern, with no identifiable associations. Esophageal motility disorders and gastroesophageal reflux (GER) are common in patients with end-stage lung disease including IPF. GER-induced aspiration has been proposed as a risk factor for development of IPF and may contribute to further lung injury. Recognition of subtle manifestations of GER in this population is therefore imperative. We intend to characterize esophageal motility disturbances and GER in IPF and explore their relationship to clinical symptoms and lung function. Methods: We performed a retrospective data review of patients with idiopathic pulmonary fibrosis (IPF) referred for combined 24-hour pH-impedance off PPI and high resolution esophageal manometry studies between January 2009 and October 2013 from an interstitial lung disease clinic and a pre-lung transplant clinic. Results: Twenty-eight patients qualified for our study, 71% male, mean age 64±1.4 years, with a mean total lung capacity (TLC) of 62.8±2.9% predicted. Sixty-eight percent of patients had abnormal high resolution manometry: 22% of all patients had ineffective esophageal motility (IEM), 18% had weak upper esophageal sphincters, 14% had weak lower esophageal sphincters (LES), 11% had elevated LES resting pressures, and 28% had a hiatus hernia. Fifteen patients had symptomatic heartburn at the time of investigations, of whom three had a DeMeester score >14.7. Four of the 28 patients (14.3%) reported esophageal symptoms (dysphagia or regurgitation) at the time of the investigations, which was associated with a sensitivity of 16%, and a specificity of 89% for a manometric abnormality. There was no association between abnormal esophageal motility, increased esophageal acid exposure and forced vital capacity (FVC), diffusion capacity of carbon monoxide adjusted for alveolar volume, or 6MWT. Conclusions: Esophageal motility is frequently abnormal in patients with IPF. Despite symptoms of heartburn, GERD is infrequent in patients with IPF. The presence or absence of GERD and/or esophageal dysmotility is not associated with the severity of lung function in IPF patients.

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