Superior Mesenteric Artery Syndrome: A Potentially Fatal but Reversible Gastrointestinal Manifestation of Systemic Sclerosis

Table 2

Summary of case reports and series on superior mesenteric artery syndrome in patients with rheumatological conditions.

Author	Title	Publication	Description
Gondos [22]	Duodenal compression defect and the “superior mesenteric artery syndrome (SMAS)”	Radiology 123(3): 575–580	SMAS-like manifestation described in 42 cases of systemic sclerosis in 1978. In this case series, all patients with duodenal dilatation had a compression defect at the site where the SMA crossed the duodenum. It was described that the compression defect and duodenal dilatation may spontaneously disappear and recur. However, the author attributed the compression produced by the SMA to dilatation and loss of muscle tone of the duodenum instead and did not accord a diagnosis of SMAS to the cases.
Bedaiwi et al. [23]	Superior mesenteric artery syndrome and intraabdominal compartment syndrome in systemic lupus erythematosus	Lupus 23(2): 194–196	SMAS was described in a 32-year-old female with newly diagnosed systemic lupus erythematosus (SLE) presenting with serositis, lupus enteritis, nephritis, and cerebritis. She developed SMAS following a significant weight loss of 10 kg over three months at the initial presentation. SMAS resolved with fasting and gastrointestinal decompression with an NGT, together with total parenteral nutrition (TPN) for three weeks followed by cyclic parenteral nutrition. Repeat CT abdomen-pelvis showed that the SMA-aortic angle, aortomesenteric, and fat volume estimation increased to 36.9 degrees, 3.4 mm, and 6082.460 cm^3, respectively, upon recovery.
Padhan et al. [24]	Superior mesenteric artery syndrome in an adult rheumatoid arthritis patient	International Journal of Rheumatic Diseases 15(1):e4-5	SMAS was described in an adult with rheumatoid arthritis who developed cachexia following a two-year history of persistent nausea and vomiting after meals, with a weight loss of 12 kg in a year. There was CT reduction of the aortomesenteric distance to 6.3 mm and aortomesenteric angle of 17 degrees with paucity of abdominal fat. This resolved with small frequent meals containing high calories and protein that resulted in a 5 kg weight gain over six months.
Chua et al.	Superior mesenteric artery syndrome: a potentially fatal but reversible gastrointestinal manifestation of systemic sclerosis	Current article	SMAS was described in an adult with diffuse cutaneous systemic sclerosis who developed recurrent nonbilious, nonbloody vomiting with high nasogastric tube aspirates and aspiration pneumonia. She lost about 10 kg over four years. During fluoroscopic guidance for nasojejunal tube insertion, the stomach and proximal duodenum were noted to be grossly dilated with fluid. A transition point was encountered at the mid third part of the duodenum with a vertically oriented obstruction suggestive of extrinsic compression by the SMA. Subsequent CT abdo-pelvis revealed narrowed aortomesenteric angle of approximately 23 degrees (Figure 1) and an aortomesenteric distance of approximately 7 mm. Patient declined TPN and eventually demised from acute myocardial infarction and pneumonia.