A Case of Systemic Sclerosis Mimicking Achalasia

Abstract

Systemic sclerosis (SS) may be associated with GERD symptoms and dysphagia related to esophageal dysmotility or a peptic stricture in 50 - 90% of patients. HRM may show esophageal aperistalsis of the smooth muscle with a low LESp. We present 2 cases of SS presenting with HRM findings characteristic of type I Achalasia; onepatient with symptomatic dysphagia and the other patient with symptomatic GERD and endoscopic esophagitis. Both patients have diffuse SS, Raynaud's phenomenon, interstitial lung disease, GERD and positive SS serologies. Case 1 is a 66 year-old female who developed dysphagia 8 years following SS diagnosis. BaS noted esophageal atony, no strictures and a patulous GEJ, with a normal EGD. GERD symptoms improved with PPIs but she developed progressive solid-food dysphagia without weight loss. Re-evaluation 4 years later revealed a normal EGD while HRM showed aperistalsis, normal LESp with a ?IRP mimicking type I achalasia. Timed BaS demonstrated mild mid-esophageal dilation (3 cm), tapering at the distal esophagus with minimal barium retention at 5 minutes (Fig. 1). Repeat EGD for possible pneumatic dilation, demonstrated a mildly patulous LES that was easily distended following air insufflation and no resistance w ith endoscopic passage through the GEJ. Case 2 is a 54 year old patient with an 11 year history of SSc and GERD but no dysphagia with a mildly dilated esophagus with intraluminal debris (Fig. 2). HRM showed aperistalsis, normal LESp, and elevated IRP (Fig 3). Most recent EGD revealed LA Class B esophagitis. Caution should be exercised in making the diagnosis of achalasia in SS patients. Full evaluation must include a timed barium study, and EGD to be certain that one is dealing with GEJ obstruction. Measurement of the LESp and relaxation are dependent upon: (1) the crural diaphragm which does not relax during swallowing, (2) the intrabolus pressure generated by the fluid passing through the GEJ, and (3) the cephalad movement of esophagus related to longitudinal muscle contraction which shortens the esophagus and momentarily pulls the stomach into the GEJ during swallowing. It is possible that in SS with a dilated esophagus and no muscle contraction, the esophagus did not shorten and LES remained stationary at the level of the crural diaphragm. Few cases exists of SS having HRM criteria for achalasia. These 2 cases presents a hypothesis for further research in SS patients having HRM criteria for achalasia.1722_A Figure 1. Mild esophageal dilation with distal esophageal tapering and minimal barium retention in case 11722_B Figure 2. Mild esophageal dilation with intraluminal debris in case 21722_C Figure 3. Representative esophageal topography of elevated IRP in case 2