Anti-vinculin autoantibodies in systemic sclerosis: a step toward a novel biomarker?

Excerpt

Systemic sclerosis (SSc) is an autoimmune systemic vasculopathy that leads to wide-spread organ fibrosis. Gastrointestinal (GI) involvement in SSc is common and often results in debilitating symptoms that can lead to malnutrition and in severe cases dependence on total parenteral nutrition (TPN). There are multiple causes of GI symptoms in SSc, including dysfunction of the lower esophageal sphincter which can lead to chronic gastrointestinal reflux disease (GERD) and strictures, GI dysbiosis [1] including small intestinal bowel overgrowth (SIBO), food intolerance (e.g., fructose, lactose) [2, 3], and dysmotility of the GI tract (i.e., esophagus, stomach, small and large bowel). Our understanding of GI dysbiosis in medicine, and particularly in SSc, is still evolving, and the relationship between transit abnormalities and dysbiosis is still poorly understood. While gastric emptying tests are widely available, other more specialized imaging tests to evaluate for dysmotility throughout the GI tract are limited in availability and not well-studied in SSc. Serum biomarkers to diagnose and monitor GI disease in SSc are lacking, especially biomarkers that can reliably detect early GI disease activity. In addition, questions remain about how to identify subgroups at high risk for GI progression and whether the early application of promotility agents or immunomodulation prior to the development of progressive smooth muscle atrophy and GI fibrosis is beneficial for such patients. …