Ultrastructure of the Small Intestinal Mucosa in Progressive Systemic Sclerosis (Pss)

Abstract

The present study deals with ultrastructural changes of jejunal absorptive epithelium in mucosal biopsy specimens from eleven patients with progressive systemic sclerosis (PSS). Epithelial cells showed cytoplasmic condensations with cholesterol clefts and fat droplets. Intercellular spaces of the epithelium were dilated with deposits of fat droplets, while tight junctions and villi preserved their normal structures. Goblet cells and entero‐endocrine cells showed no changes. In the lamina propria, fat droplets were observed as well. Vessels were strongly dilated and in 2 patients contained conglomerates of thrombocytes. Perivascular cell infiltrates consisted of lymphocytes, macrophages and mast cells, while plasma cells were scarcely seen; in 2 patients, plasma cells were abundant. Collagen fibrils, elastic fibres, and nerves showed no definite changes. The findings of epithelial cell degeneration and fat droplet deposits indicate dysfunction of jejunal absorptive epithelial cells in PSS.

Determining the Risk Factors and Clinical Features Associated With Severe Gastrointestinal Dysmotility in Systemic Sclerosis

“…Although our retrospective review did not allow us to determine the cause of myopathy in every case, it is interesting to speculate that autoantigens common to both skeletal and smooth muscle could be targeted by the autoimmune response . There is a precedent for this speculation in that the neurons that innervate smooth and skeletal muscle are similar, and if targeted in the bowel can cause the smooth muscle atrophy and bowel dysfunction observed in the SSc GI tract . As objective reports were not available in all patients to confirm definite myopathy, it is possible that the association between severe GI dysmotility and myopathy may also be driven by malnutrition.…”

Gastrointestinal Manifestations of Scleroderma: Recent Progress in Evaluation, Pathogenesis, and Management

“…Although severe GI manifestations seem to be mostly related to fibrosis [16], early neural disease without fibrosis can also lead to clinical manifestations. Some pathology reports have not found any fibrosis, even in symptomatic patients [15,17,18].…”

“…intestinal pathology [13][14][15]. Sjogren [14] proposed a fourstage process similar to that in the skin.…”

Current management of the gastrointestinal complications of systemic sclerosis

“…Whatever the aetiology of the disease, vascular instability is thought to progress to tissue ischaemia and subsequent defective immunity and tissue repair. Tissue repair in patients with systemic sclerosis is characterized by excessive collagen and collagen matrix deposition, resulting in fibrotic replacement of tissue in the skin and internal organs, including in the gastrointestinal tract [14] . Fibrosis involves both the gut smooth muscle and the smooth muscle of the gastrointestinal vasculature [15] .…”