Gastroesophageal Reflux Disease and Connective Tissue Disorders: Pathophysiology and Implications for Treatment

Introduction It has been postulated that in patients with connective tissue disorders (CTD) and gastroesophageal reflux disease (GERD), esophageal function is generally deteriorated, often with complete absence of peristalsis. This belief has led to the common recommendation of avoiding antireflux surgery for fear of creating or worsening dysphagia. Methods We hypothesized that in most patients with CTD and GERD: (a) esophageal function is often preserved; (b) peristalsis is more frequently absent when end-stage lung disease (ESLD) is also present; (c) a tailored surgical approach (partial or total fundoplication) based on the findings of esophageal manometry allows control of reflux symptoms without a high incidence of postoperative dysphagia. Forty-eight patients with CTD were evaluated by esophageal manometry and 24-hour pH monitoring (EFT). Twenty patients (group A) had EFT because of foregut symptoms, and 28 patients with ESLD (group B) had EFT as part of the lung transplant evaluation. Two hundred and eighty-six consecutive patients with GERD by pH monitoring served as a control group (group C). A laparoscopic fundoplication was performed in two group A patients (total), eight group B patients (three patients total, five patients partial) and in all group C patients (total). Results Esophageal peristalsis was preserved in all patients with CTD and GERD. In contrast, peristalsis was absent in about half of patients when ESLD was also present. A tailored surgical approach resulted in control of reflux symptoms in all patients. One patient only developed postoperative dysphagia, which resolved with two Savary dilatations. Conclusion These data show that esophageal motor function is preserved in most patients with CTD, so that they should be offered antireflux surgery early in the course of their disease to prevent esophageal and respiratory complications. In patients with ESLD in whom peristalsis is absent, a partial rather than a total fundoplication should be performed, as it allows control of reflux symptoms while avoiding postoperative dysphagia.