Clinical Image: Esophageal dilation and stenosis in systemic sclerosis

The patient, a 59-year-old woman, presented to the emergency department with progressively worsening dyspnea and upper abdominal distension over the past 12 days. For five years, she had been experiencing progressive skin tightening, burning substernal pain, and shortness of breath due to systemic sclerosis (SSc). On physical examination, the skin was significantly thickened and hardened, and the “salt-and-pepper” sign was evident on her upper chest. She weighed 34 kg (75 lb) and had grade 3 muscle strength. (A) Iodine–water esophagography demonstrated esophageal lumen dilation and high-grade stenosis, likened to a bird's beak between the lower end of the esophagus and the stomach. Chest high-resolution computed tomography scan revealed a markedly dilated esophagus. (B) On axial images, the esophageal diameter was greater than that of the trachea, (C) whereas the sagittal view showed signs of tracheal compression. A diagnosis of SSc with esophageal dilation and stenosis was established. Esophageal involvement is a severe manifestation of SSc and is associated with anti-Topo1 antibodies, possibly resulting from esophageal smooth muscle fibrosis and nerve injury.1, 2 Severe esophageal disorders often indicate a poor prognosis and higher mortality risk.1, 2 Unfortunately, this patient succumbed to malnutrition and respiratory failure caused by her esophageal involvement and respiratory muscle weakness related to SSc. Disclosure Form: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.