Abstract
Superior mesenteric artery syndrome (SMAS) is a rare gastrointestinal disorder characterised by vascular compression of the third part of the duodenum, in the angle between the superior mesenteric artery (SMA) and the abdominal aorta. It presents as an uncommon cause of upper gastrointestinal obstruction. In patients with systemic sclerosis (SSc), gastrointestinal involvement may result in oesophageal dysmotility, gastroesophageal reflux disease (GERD), gastroparesis, small intestinal bacterial overgrowth (SIBO), chronic intestinal pseudoobstruction (CIPO), and fecal incontinence. Malnutrition may thus result in weight loss and reduced mesenteric and retroperitoneal adipose tissue, decreasing the angle between the SMA and aorta causing SMAS. Enteral or parenteral feeding can potentially reverse SMAS in SSc. We report a case of SMAS in an elderly female with SSc and concurrent gastrointestinal involvement, and discuss the important management considerations and potential adverse outcomes when untreated.
Highlights
Systemic sclerosis (SSc) is a systemic autoimmune rheumatic disease where gastrointestinal involvement occurs in more than 90% of patients, more often in diffuse cutaneous SSc rather than the limited forms
Gastrointestinal involvement may result in oesophageal dysmotility, gastroesophageal reflux disease (GERD), gastroparesis, small intestinal bacterial overgrowth (SIBO), chronic intestinal pseudoobstruction (CIPO), and fecal incontinence [1, 2]
A 64-year-old Chinese female was diagnosed with diffuse cutaneous SSc (dcSSc) in 2011 at the age of 59 when she presented with diffuse skin thickening, Raynaud’s phenomenon, and arthritis of the small joints of both hands
Summary
Systemic sclerosis (SSc) is a systemic autoimmune rheumatic disease where gastrointestinal involvement occurs in more than 90% of patients, more often in diffuse cutaneous SSc (dcSSc) rather than the limited forms. It is characterised by three key pathophysiological processes: inflammation, vasculopathy, and fibrosis. E four key pathophysiological mechanisms causing gastrointestinal dysfunction in patients with SSc appear to be as follows: infiltration of immune cells (via humoral and cell-mediated immunity) into gut smooth muscle; fibrosis of gut smooth muscle; labile vascular tone of the submucosal arterioles and venules; and dysfunction of the enteric nervous system (ENS) and smooth muscle [1]. Case Reports in Rheumatology compression of the third part of the duodenum, in the angle between the superior mesenteric artery (SMA) and the abdominal aorta. is is most often a result of significant weight loss and reduced mesenteric and retroperitoneal adipose tissue, decreasing the usual angle of 38–65 degrees between the SMA and aorta, to less than 25 degrees causing SMAS [6]
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