Connective tissue disorder which is associated with gastroesophageal reflux is -

Scleroderma Is a systemic disease accompanied by oesophagal abnormalities in approximately 80% of patients? The oesophagal lesion consists of atrophy of smooth muscles, manifested by weakness in the lower two-thirds of the oesophagal body and incompetence of the LES. Gastroesophageal reflux d/t incompetent LES combined with poor oesophagal clearance d/t decreased motility leads to severe reflux esophagitis and stricture formation. The patient complains of dysphagia and symptoms of gastro-oesophageal reflux. The dysphagia in scleroderma is characteristic. Patients have dysphagia for liquids while recumbent but not upright. The dysphagia for solids is unrelated to posture. The Barium studies show a dilated oesophagus, stomach and duodenum or a hiatal hernia with distal oesophagal stricture and proximal dilation (a Hiatal hernia is the result of oesophagal shoening due to stricture which pulls the cardio up into the thorax) The diagnosis of scleroderma is made by manometry study which shows : normal peristalsis in the proximal striated oesophagus, with absent peristalsis in the distal smooth muscle poion (as there is smooth muscle atrophy in scleroderma) the LES pressure is decreased but sphincter relaxation to deglutition is normal. Ref : Schwaz 9/e 846