Background/Purpose: Systemic sclerosis (SSc) patients frequently have upper gastrointestinal (GI) symptoms, with GI involvement being the leading cause of morbidity. Meanwhile, interstitial lung disease (ILD) and pulmonary hypertension (PH) are the leading cause of mortality in SSc. Reflux disease may play a role in ILD through chronic microaspiration and/or altered pulmonary mechanics in ILD can potentiate the impact of reflux. A potential causality between the two variables has yet to be established.

We investigated the prevalence of esophageal motility disorders and reflux in SSc patients and evaluated the relationship between GI characteristics and pulmonary outcomes.

Methods: This retrospective chart review was carried out at the University of California, Los Angeles (UCLA). Patients included in the study were diagnosed with systemic sclerosis and evaluated in SSc-GI clinic. Esophageal evaluation was carried out using at least one of the following modalities: high-resolution esophageal manometry (HREM), ambulatory pH testing and/or EGD. Data, collected within 1 year of the GI procedures, included: patient demographics, scleroderma characteristics, motility data, presence of ILD defined by HRCT, PH, oxygen requirement, and pulmonary function tests (PFTs) completed within one year of the index GI study. Statistical analyses included descriptive, ANOVA or Chi square testing as appropriate.

Results: Our study cohort included 122 patients, mean age± SD of 66± 9 at GI evaluation. 93% of patients were female, 68% had limited and 32% had diffuse cutaneous disease.

Most patients underwent HREM and EGD. Approximately 70% of the patients had absent contractility on manometry, while another 24% had ineffective esophageal motility. Hiatal hernia was found in one third of patients. Of those who underwent EGD, 37% had erosive esophagitis, while only 23% had a definitive diagnosis of GERD based on EGD. A portion of our cohort completed ambulatory pH testing, with 68% of studies demonstrating GERD. ILD was noted in 55% of patients.

We found no correlation between patient FEV1, FVC and/or DLCO and esophageal motility disorders based on HREM or the diagnosis of GERD based on EGD/pH studies. There were no significant differences in the PFTs and different HREM diagnoses, between patients with and without a diagnosis of GERD, or between patients with ILD with normal and abnormal motility. Our preliminary data did not reveal any correlation between esophagitis or hiatal hernia and ILD per HRCT findings.

Conclusion: Our study is one of the largest single-center cohorts of SSc patients characterizing esophageal motility, reflux and PFTs. The majority of our patients demonstrated abnormal motility, a finding believed to contribute to reflux. Over half of our cohort had lung involvement. There was no association found between esophageal dysmotility and/or GERD with PFTs. Similarly, preliminary data support a similar trend with HRCT findings of ILD. Given the lag time between discernable PFT changes and radiographic findings on HRCT, additional characterization of GI disease and early radiographic findings of ILD and PFT changes overtime is helpful and underway.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-gastroesophageal-disease-in-systemic-sclerosis-and-its-impact-on-lung-disease-fact-or-fiction/