A 68-year-old man with a history of small bowel obstruction and chronic airflow obstruction in follow-up by Pneumology.
A computed tomography (CT) scan of the chest revealed a significant dilatation of the middle and distal third of the esophagus, a dilated esophagus at the cardia level with a mid-third air-fluid level.
The patient was admitted with digestive symptoms.
She complained of heartburn and nocturnal irritative cough for years.
Physical examination revealed malar teleangiectasia.
Analyses revealed mild normocytic anemia and elevated CRP.
During the follow-up consultation the patient presented several episodes of vomiting after coffee. Several endoscopies were performed, finding a marked dilatation of the esophagus, a permanently opened cardia and erosive lesions in the esophageal mucosa with acid reflux.
Three months later, the patient developed a pseudoaneurysm requiring surgery, which ruled out mechanical obstruction.
The barium-esophageal-gastroduodenal study showed a hypotonic and relaxed esophagus and marked dilation of the duodenum and small intestine.
The patient did not tolerate esophageal maniere and the functional study was completed with an isotopic esophageal transit that showed a severe transit delay with a pattern of esophageal body adynamia and presence of severe gastroesophageal reflux.
The antitransglutaminase IgA, ANA, anti-DNA, AML, ANCA antibodies were negative.
Anticentromere antibody titers of 1/320 were positive.
We again questioned the patient who referred a clinical picture compatible with Raynaud's phenomenon in all fingers of both hands after exposure to cold in the last 3 years and we referred him to the Coldermal Diseases Unit.
