Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE

Abstract

Lupus mesenteric vasculitis (LMV) is a unique clinical entity found in patients who present with gastrointestinal manifestations of systemic lupus erythematosus, and is the main cause of acute abdominal pain in these patients. LMV usually presents as acute abdominal pain with sudden onset, severe intensity and diffuse localization. Other causes of abdominal pain, such as acute gastroenteritis, peptic ulcers, acute pancreatitis, peritonitis, and other reasons for abdominal surgery should be ruled out. Prompt and accurate diagnosis of LMV is critical to ensure implementation of appropriate immunosuppressive therapy and avoidance of unnecessary surgical intervention. The pathology of LMV comprises immune-complex deposition and complement activation, with subsequent submucosal edema, leukocytoclastic vasculitis and thrombus formation; most of these changes are confined to small mesenteric vessels. Abdominal CT is the most useful tool for diagnosing LMV, which is characterized by the presence of target signs, comb signs, and other associated findings. The presence of autoantibodies against phospholipids and endothelial cells might provide information about the likelihood of recurrence of LMV. Immediate, high-dose, intravenous steroid therapy can lead to a favorable outcome and prevent serious complications such as bowel ischemia, necrosis and perforation.

A patient with systemic lupus erythematosus who developed massive small intestinal hemorrhaging during treatment for chronic lupus peritonitis

“…The main pathologic lesions in LMV occur in small vessels in the bowel wall. Variable symptoms are caused by ischemia that involves four different layers of the bowel wall, as follows: serosa-ascites; muscular-pseudo-obstruction, dilatation, fluid accumulation; submucosa-submucosal edema, pseudotumors; mucosa-hemorrhaging, ulceration, diarrhea [5]. It is suggested from our patient's angiography findingsthat she had bleeding from multiple pseudo-microaneurysms that may have developed because of weakness of the vessel wall caused by vasculitis [11].…”

“…APS might be associated with mesenteric ischemia; the microvasculopathy in LMV results in worsening cascades of vasculitis and thrombosis, which are associated with circulating antiphospholipid antibodies [5]. Immediate and aggressive anti-inflammatory immunosuppressive therapy for LMV must include intravenous pulses of mPSL and IVCY [5,12]. However, in the present patient, the vasculitis might gradually progress despite the aggressive therapy with intravenous pulses of mPSL and IVCY, because this treatment is insufficient to halt such progress.…”

“…LMV was the main reported cause of acute abdomen in patients with SLE, in whom LMV develops insidiously [5]. Severe LMV can result in life-threatening complications, such as extensive gastrointestinal hemorrhaging, bowel perforation, and bowel infarction.…”

“…Additionally, extensive mucosal damage caused by lupus enteritis may have exacerbated the massive small intestinal hemorrhaging. APS might be associated with mesenteric ischemia; the microvasculopathy in LMV results in worsening cascades of vasculitis and thrombosis, which are associated with circulating antiphospholipid antibodies [5]. Immediate and aggressive anti-inflammatory immunosuppressive therapy for LMV must include intravenous pulses of mPSL and IVCY [5,12].…”

“…Patients with SLE sometimes develop small intestinal hemorrhaging as the result of a variety of causes, including enteritis, vasculitis, drug side effects, and infection. Lupus mesenteric vasculitis (LMV) is one of the most serious causes of such hemorrhaging [5]. We describe herein a patient with SLE complicated with chronic lupus peritonitis who developed massive small intestinal hemorrhaging that appeared to have been caused by LMV.…”