Rationale: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease despite the available treatment regimes. Increased acid gastroesophageal reflux (GER) occurs in IPF patients.
Objectives: To follow the course of IPF in patients while being treated for acid GER alone.
Methods: A retrospective review of the clinical outcomes of four patients with newly diagnosed IPF and increased acid GER who chose to be treated solely with anti-acid GER therapy were followed up regularly with pulmonary function tests (PFTs) [measuring FVC and the diffusing capacity of the lung for carbon monoxide] over a period of 2 to 6 years. Anti-acid GER therapy was administered using proton-pump inhibitors and fundoplication, if needed. Adequate suppression of acid GER was ascertained by 24-h esophageal pH monitoring.
Main results: PFT results in all four patients stabilized or improved while their conditions were maintained with adequate treatment for acid GER. All patients were alive at the last follow-up, and none manifested an acute exacerbation of IPF or needed treatment for respiratory problems during this period. After maintaining 4 years of improved status (based on PFT and exercise testing findings) while adhering to treatment for acid GER, one patient's deterioration correlated with poor compliance to daily treatment during the fifth year, although the PFT results at the sixth year showed stabilization compared to baseline values. The condition of another patient was stabilized by adhering to anti-acid GER treatment after an initial period of deterioration that was associated with nonadherence
Conclusions: Future clinical studies are indicated to clarify the role of acid GER in IPF and to determine whether adequate treatment for increased acid GER in part improves the outcome of patients with IPF.