OBJECTIVES: Interstitial lung disease (ILD) is currently the main cause of death in systemic sclerosis (SSc) and has an unknown pathogenesis. Gastroesophageal reflux (GER) has been strongly implicated as a cause of ILD in several lung diseases, including SSc-ILD. This review summarizes clinical, radiologic, histopathologic, and treatment aspects of GER in SSc-ILD. METHODS: The PubMed database was searched using the following keywords: "systemic sclerosis, scleroderma, interstitial lung disease, and gastroesophageal reflux." The research was limited to English-language studies that included SSc patients with ILD. RESULTS: Pulmonary function tests were related with the presence of GER in several esophageal functional tests (esophageal endoscopy, pH monitoring, and manometric analysis). Regarding the histopathologic data, a pattern called centrilobular fibrosis was described in 21% of 28 lung biopsies, with a bronchocentric distribution and with an intraluminal content resembling gastric fluid. Radiologic evidence of esophageal dilation is very frequent in SSc patients, and consolidation with a patchy distribution was almost exclusively found in SSc patients with centrilobular fibrosis lung pattern. Furthermore, high levels of serum KL-6, a marker of epithelial injury, are indicative of active ILD in SSc disease. CONCLUSIONS: The association of GER with SSc-ILD is strongly supported by several studies. An aggressive treatment for reflux is recommended in all SSc patients with ILD; however, future studies need to be performed to prove a long-term benefit.