A case-controlled study of the pathology of oesophageal disease in systemic sclerosis (scleroderma)

Caroline G.P. Roberts ¹, Laura K. Hummers ¹, William J. Ravich ¹, Fredrick M. Wigley ^1* and Grover M. Hutchins ¹

¹ Johns Hopkins University, United States

^* To whom correspondence should be addressed. E-mail: fwig{at}jhmi.edu.

Accepted 1 March 2006

Abstract

Objective: Atrophy of the smooth muscle layers of the muscularis propria characterizes esophageal involvement in systemic sclerosis (scleroderma). The etiology of this atrophy and of the resultant esophageal dysfunction is unknown. We examined esophageal tissue for evidence of fibrosis, vascular disease, inflammatory reactions, and neural abnormalities to determine possible causes of this disease process.

Methods: A case-controlled survey was conducted using esophageal tissue from 74 scleroderma cases and 74 age, race, and sex-matched controls from our autopsy files. Histological evidence of esophageal muscle atrophy was correlated with the degree of vascular changes, inflammatory infiltration, fibrosis, abnormalities of the myenteric plexus and reduction of interstitial cells of Cajal (ICC) using a pre-designed semi-quantitative descriptive method.

Results: Smooth muscle atrophy was found in 94% of scleroderma cases, and in 5% of controls (p< 0.0001). There was atrophy in the circular smooth muscle in 93% of cases, and in the longitudinal smooth muscle in 66% of cases. Intimal proliferation of arterioles was found in 38% of cases and 5% of controls (p< 0.0001), but was not associated with smooth muscle atrophy (p= 0.29). Despite these vascular changes there was no evidence of compromised perfusion, such as findings suggestive of acute ischemic necroses. Minimal cellular infiltrates were seen within the myenteric plexus in 82% of cases and in 92% of controls (p= 0.091). Interstitial cells of Cajal were found in fewer numbers in areas of atrophic smooth muscle compared to adjacent normal smooth muscle in selected scleroderma cases.

Conclusion: The pathological findings of esophageal lesions in scleroderma appear inconsistent with either an ischemic or an inflammatory process. The loss of circular and longitudinal smooth muscle in the distal scleroderma esophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion.

Keywords: esophageal disease, interstitial cells of Cajal, scleroderma, smooth muscle atrophy

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