Scleroderma esophagus.

ABSTRACT

A 40 year-old female presented with gradual onset generalised swelling, followed by thickening of skin for one year. She had symmetrical polyarthritis and firm, shiny skin with areas of depigmentation and absence of normal skin wrinkling. She had flexion contracture of fingers, masked facies, difficulty in opening mouth and Raynoud’s phenomenon. She had dysphagia to solid for last two months with recurrent heartburn. ESR was 120mm/1st hr. ANF was positive. X-ray hands showed periarticular osteopenia. Barium swallow X-ray showed dilated distal esophagus with loss of peristaltic contractions and a stricture at lower end of esophagus. Fundic gas was visible (Fig. 1). Upper GI endoscopy excluded any malignancy but showed features of reflux esophagitis with a stricture at lower esophagus causing difficulty in passing the endoscope through it. The esophagus is involved in 50-90% of patients of scleroderma.1 Smooth muscle atrophy and fibrosis lead to thinness and weakening in lower two-third of esophagus and incompetence of lower esophageal sphincter (LES). The proximal one third with its striated muscle is spared. The commonest manifestation is reflux esophagitis, which may be complicated by peptic stricturing near the junction with stomach. Dysphagia occurs due to esophageal dysmotility or stricture. Barium swallow X-ray shows dilatation and loss of peristaltic contractions in lower esophagus. LES is patulous. Esophageal mucosal ulceration and stricture may be visible. Manometry shows decreased amplitude or absence of peristaltic waves in lower esophagus. Resting pressure of LES is subnormal, but sphincter relaxation is normal. The differential diagnosis, achalasia shows esophageal dilatation with persistent beaklike narrowing of terminal esophagus due to non-relaxing LES. Chest X-ray shows absence of gastric air bubble and an air-fluid level in mediastinum on erect posture. Manometry shows normal or elevated resting pressure of LES and elevated resting pressure in esophageal body. Management of scleroderma esophagus includes proton pump inhibitors and dietary adjustments with soft foods. Bougie dilatation may be required for stricture. REFERENCE Kahan A, Menkes CJ. Gastrointestinal involvement in systemic sclerosis.Clin Dermatol 1994;12259-65.