Relationship Between Esophageal Abnormalities on Fluoroscopic Esophagram and Pulmonary Function Testing in Juvenile Systemic Sclerosis

Objective Juvenile systemic sclerosis (SSc) is a disabling autoimmune condition that affects multiple organs in addition to skin, notably the gastrointestinal and pulmonary systems. The relationship between esophageal abnormalities and pulmonary disease in juvenile SSc is not well understood. We describe associations between radiologic esophageal abnormalities and pulmonary function. Methods Clinical and radiographic data of children ages >18 years who fulfilled the 2007 Pediatric Rheumatology Provisional Classification Criteria for juvenile SSc between 1994 and 2016 were reviewed. Fluoroscopic upper gastrointestinal (UGI) studies, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs) within 12 months of presentation to Seattle Children's Hospital were extracted. Results Twenty-one children with juvenile SSc (67% female, ages 8-17 years) were studied. Esophageal abnormalities, defined as abnormal esophageal peristalsis and/or bolus clearance, were found in 12 patients. Abnormal esophagus on UGI tests was not associated with gastrointestinal or pulmonary symptoms, disease duration, use of medications (proton pump inhibitor or immunosuppressant), or specific autoantibodies. Compared with patients with a normal esophagus on UGI tests, children with an abnormal esophagus had decreased PFTs: mean forced expiratory volume in 1 second 96% versus 78% (P = 0.03), forced vital capacity 94% versus 76% (P = 0.02), and vital capacity 95% versus 76% (P = 0.02). Children with an abnormal esophagus on UGI tests had a larger mean esophageal diameter on HRCT (14.6 mm compared to 8.5 mm; P < 0.01). Conclusion There was an association between esophageal and pulmonary disease in children with juvenile SSc. Esophageal findings on UGI tests or HRCT, despite lack of symptoms, should raise concern for esophageal dysfunction and prompt heightened surveillance for concurrent lung disease.