Esophageal involvement in juvenile localized scleroderma: a pilot study.

Abstract OBJECTIVES: To evaluate the esophageal involvement in patients with juvenile localized scleroderma (JLS). METHODS: A cohort of patients with JLS underwent esophageal stationary manometry to evaluate esophageal motility and lower esophageal sphincter (LES) function, distal esophagus 24-hour pH-monitoring to detect gastroesophageal reflux (GER) and upper gastrointestinal (GI) endoscopy to evaluate the presence of esophagitis. RESULTS: Fourteen patients (10 female, mean age 13.3 yrs, mean disease duration 4.7 yrs), took part in the study. Ten had linear scleroderma, three deep morphea, and one generalized morphea. Esophageal abnormalities were found in 8/14 patients (57%): pathological acid exposure on 24-hour pH-monitoring was found in 7; non-specific esophageal motor abnormalities in 5 and endoscopy-proved esophagitis in 5 symptomatic patients. Interestingly, 5 out of 8 patients with esophageal abnormalities were found to be ANA positive, and 2 were also RF positive. CONCLUSION: Esophageal involvement is not unusual in patients with juvenile localized scleroderma, even in the absence of specific symptoms. These preliminary findings, if confirmed in a larger cohort of patients, may support the indication for an extensive GI evaluation especially in presence of positive autoantibodies or specific GI symptoms.