A 47-year-old woman with MCTD presents from home with fever and dyspnea. In the emergency room, she is found to have hypoxemia with a room air saturation of 87% and a right upper lobe opacity on chest x-ray. Her oxygenation improves with supplemental oxygen and then she is transported to the CT scanner. There she acutely decompensated with worsened hypoxemia and hypotension prompting endotracheal intubation, presumably after a gastric aspiration event.
Which is the most common site of gastrointestinal involvement of MCTD?
A. EsophagusCorrect Answer: A
MCTD, also known as Sharp syndrome, is considered an “overall syndrome” and shares clinical similarities with SLE, SS, and PM. MCTD is characterized by positive anti-U1-RNP antibody. Aside from the musculoskeletal system, the cardiovascular system, gastrointestinal tract, lungs, hematologic system, renal system, and central nervous system may all be involved.
Among gastrointestinal manifestations, approximately 85% involve the esophagus. Of all patients with MCTD, 45% to 85% may experience esophageal dysmotility, which may be subclinical at the time of diagnosis. As in SS, esophageal studies including manometry or barium swallow may show reduced peristalsis of the lower third of the esophagus and deceased pressure of the lower esophageal sphincter. Whether esophageal dysmotility and subsequent aspiration of gastric contents in MCTD is related to the manifestation of ILD is an area of debate. In MCTD patients with normal esophageal motility, only 20% experience dyspnea, versus over 70% of those with moderately reduced esophageal peristalsis or aperistalsis.
Although esophageal involvement is the most common, MCTD may also affect nearly every component of the GI system, including delayed gastric emptying, slow intestinal transit, malabsorption, and colonic pseudodiverticula and colonic perforations.
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