A 36-year-old lady presented acutely with abdominal pain, weight loss and joint pains. She had recently been diagnosed with rheumatoid arthritis based on arthralgia, and a positive rheumatoid factor. She became increasingly breathless, rapidly deteriorated and suffered a PEA cardiac arrest. A large pericardial effusion was diagnosed and treated.
Lupus is the unifying diagnosis which has been successfully treated resulting in resolution of both her gastrointestinal and cardiac symptoms.
A previously fit and well 36-year-old lady presented twice as a surgical emergency with epigastric pain. Rheumatoid arthritis had been diagnosed 18 months earlier on the basis of painful, swollen interphalangeal joints, a possible small erosion at the base of the proximal phalanx of the left ring finger and a positive rheumatoid factor.
She was taking meloxicam 15mg once daily.
Abdominal pain started two weeks before her first admission and had initially responded to lansoprazole. The pain then worsened, radiating through to her back necessitating admission.
Serum amylase, full blood count and liver function tests and abdominal ultrasound were normal. Her symptoms settled. Gastroscopy shortly after discharge was normal.
She was readmitted 2 weeks later, again with abdominal pain, this time in the right iliac fossa region accompanied by diarrhea, vomiting and vaginal discharge. Pelvic ultrasonography was reported normal and she was allowed home with a working diagnosis of bacterial vaginosis.
The next day she was readmitted as a medical emergency with diarrhea and vomiting, abdominal pain and pyrexia. She was found to have mouth ulcers, weight loss of over 2 stone in the previous 6 months but no blood or mucus in the stool.
Inflammatory bowel disease was suspected and she was commenced on intravenous hydrocortisone, and intravenous broad spectrum antibiotics in case of underlying sepsis.
Cardiomegaly was noted on her admission chest x-ray and an echocardiogram was booked.
Unfortunately the patient deteriorated rapidly overnight with increasing shortness of breath and hypotension. She developed a severe metabolic acidosis and was transferred to the intensive care unit. Here she experienced 2 cardiac arrests from which she was successfully resuscitated.
An emergency echocardiogram revealed a large pericardial effusion with cardiac tamponade. Immediate pericardiocentesis was carried out draining 500mls of straw-colored fluid. She became hemodynamically stable.
Her recovery on the intensive care unit was initially complicated by massive elevation in alanine transaminase, which slowly returned to normal with supportive management. This was thought to be related to ischemic hepatitis.
A CT scan of her abdomen demonstrated ascites, small bowel edema, and hepatomegaly (Figures 1 and 2).
Further test results became available later from the rheumatology clinic of a local teaching hospital where she had been seen 3 weeks earlier. There was a strongly positive anti-nuclear antibody (1 in 2560) and a positive lupus anticoagulant.
Figure 1 CT scan of the patient's abdomen.
Figure 2 CT scan of the patient's abdomen.A final diagnosis of lupus serositis was made. This had involved the pericardium giving rise to cardiac tamponade. Her abdominal pain is likely to have arisen from a combination of serositis affecting her small bowel, hepatic congestion secondary to right heart failure and possibly mesenteric ischemia due to vasculitis.
She received intravenous methylprednisolone followed by oral prednisolone and azathioprine. She was extubated 2 days after her arrest. She has made an excellent recovery, was allowed home 2 weeks later, and returned to her job as a full time charity worker. She remains well after 8 months follow up. As she has no evidence of renal dysfunction her long term prognosis is excellent.
Such an acute presentation of systemic lupus erythematosis (SLE), particularly with a significant pericardial effusion and abdominal pain, is unusual but has been previously reported. Studies of SLE patients ill enough to warrant intensive care have described a mortality rate of over 50% in such patients .
Patients with SLE tend to be female (female:male, 9:1) but usually present younger (mean 18 years), often with a previous diagnosis of antiphospholipid syndrome. Joints are affected in most patients (95%) and up to 44% have some cardiac involvement, often pericarditis; complication by tamponade is unusual.
Gastrointestinal manifestations occur in approximately 20%, although mouth ulcers are relatively common and are included in the American College of Rheumatology criteria for Systemic Lupus Erythematosus .
Our patient had been previously misdiagnosed with rheumatoid arthritis following her initial joint symptoms and positive rheumatoid factor, although 13% of patients with SLE will have a positive rheumatoid factor  possibly associated with a more benign course of the disease .
Abdominal pain in patients with SLE can be classified into 3 etiological groups; those with pain related to their medication (most commonly non steroidal anti inflammatory medication), pain from other completely unrelated gastroenterological problems, for example, acute appendicitis, and pain related to the direct effects of SLE.
In a retrospective series of 175 patients with SLE 22% of them presented with an acute abdomen, lupus enteritis being the final diagnosis in most of them. The jejunum and ileum were the sites most commonly involved and all the patients initially responded well to steroids . Patients presenting with symptoms and signs of peritonism have undergone laparotomy for suspected perforation, only to find that the diagnosis is acute serositis .
Our patient was thought to be suffering from SLE-induced serositis, affecting both bowel and pericardial sac leading to pericardial effusion. Hepatic congestion with or without mesenteric ischemia may have contributed to her abdominal pain.
CT scanning of the abdomen in such patients has been better evaluated in its role to assess the renal vasculature in suspected renal vein thrombosis, where prompt treatment can preserve renal function; however incidental findings such as serositis , bowel wall thickening and hepatic enlargement have been described . Our patient's CT scan revealed ascites, hepatomegaly and thickened small bowel wall, some of which could have been explained by acute hepatic congestion.
Studies have shown good resolution of ascites with treatment directed at SLE, often in response to corticosteroids and azathioprine .
Abdominal pain can be a presenting symptom of unusual multi-system disorders. Some such conditions are treatable, and must not be over-looked.
This article was first published on GastroHep.com on 19 June 2008.
Thy Do, Amanda Beale, Miles Allison and Philip Freeman
Royal Gwent Hospital
Dr Thy Do
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