Lupus Enteritis

09 APR 2014

Lupus Enteritis- A rare complication of Systemic Lupus Erythematous

DR LAJEESH JABBAR- PGY1 MEM, MIMS CALICUT

DR RAMINIDHARAN NARAYANAN, FACULTY EMERGENCY MEDICINE

DR VENUGOPALAN P P, CHIEF OF EMERGENCY MEDICINE

Emergency room presentation

A 26 year old female who is a diagnosed case of Systemic Lupus Erythematoses since 3 years with recently diagnosed Lupus Nephritis on Homeo treatment was brought to the Emergency Department with complains of abdominal pain, abdominal discomfort and multiple episodes of vomiting since 1 day. Her initial vitals revealed a heart rate of 110/min and a blood pressure of 90/60 mm of Hg. Pain score was 8/10 and was afebrile. Clinical examination revealed presence of pallor, pedal oedema and facial puffiness. Her abdomen was distended with diffuse tenderness and presence of free fluid. Her cardiovascular, neurological and respiratory examination were normal. She was treated with analgesics (morphine), antiemetics, proton pump inhibitors and intra venous fluids. She was given a dose of steroid injection (hydrocortisone) in view of persistent hypotension. CT abdomen showed Pleural effusion and Ascites and a sub capsular haematoma in the lower pole of left kidney. Echocardiogram showed a thin layer of pericardial effusion.

Course in the hospital-

She was admitted in the Medical Intensive Care Unit. Investigations showed neutrophilic leukocytosis. On further evaluation she had active SLE with grade IV Lupus Nephritis, Polyserositis and Autoimmune Haemolytic Anemia(Coombs Test +ve) and was in sepsis. Ascitic fluid study revealed high total WBC count. She was started on broad spectrum antibiotics ,steroids and immunosuppressive treatment by which she improved dramatically. Her APLA were positive with low complement counts. Rheumatology, Gastro and Nephro consultations were done. She improved symptomatically and was discharged.

Discussion

Lupus enteritis, also termed as mesenteric arteritis, lupus mesenteric vasculitis, lupus arteritis, lupus vasculitis, is a potentially severe complication of SLE, stressing the need for swift diagnosis and adequate management. In the BILAG 2004, lupus enteritis is defined as either vasculitis or inflammation of the small bowel, with supportive image and/or biopsy findings, thus a poorly defined cause of abdominal pain in SLE . Mean age at diagnosis of lupus enteritis reported in literature is 32.5 years, with the youngest patient being 13 and the oldest being 72 years old and male–female ratio of 1:14 . Abdominal pain is the most common presenting symptom amongst other non-specific manifestations like ascites, nausea, vomiting, diarrhea and fever and has been shown to be the most common cause of abdominal pain in SLE patients seeking emergency room treatment . Typically, the abdominal pain described in lupus enteritis is diffuse in pattern and in some cases accompanied by rebound tenderness and abdominal muscle guarding . Other than a drop in white blood cell count and complement titres, which may correlate with the occurrence of lupus enteritis, none of the laboratory indices have been found to be useful to establish the diagnosis . The presence of autoantibodies against phospholipids and endothelial cells might provide information about the likelihood of recurrence of lupus mesenteric vasculitis. CT scanning has become the investigation of choice for diagnosis of lupus enteritis . Features of lupus enteritis described include focal or diffuse bowel-wall thickening, bowel dilation, target sign, comb sign (considered to be an early sign), increased attenuation of mesenteric fat and ascites. Target Sign is “thickened bowel wall with enhancing outer (muscularis propria/serosa) and inner (mucosa) layers and a hypoenhancing middle layer due to submucosal edema.”

There are no randomized control trials on treatment of this entity. Lupus enteritis has been found in several reports to be generally reversible and steroid responsive, thus considered as the first line therapy. High dose prednisolone, Methylprednisolone pulse have been used according to individual preferences with favourable responses along with supportive measures which include bowel rest, IV fluids, proton pump inhibitors and Heparin might be added in presence of ApL (Antiphospholipid antibody) antibodies and suspicion of Antiphoshpholipid syndrome . Cyclophosphamide or Mycophenolate may be added in case of resistance to corticosteroids or when warranted by other organ involvement.

Lesson learnt

1. Diagnosis of Lupus Enteritis should be always kept in mind SLE patients coming with abdominal pain

2. These patients recover substantially if proper treatment is given at the right times

3. CT Abdomen is the gold standard for diagnosis of Lupus Enteritis

   References

1. Orphanet Journal of Rare Disease

2. Lupus Enteritis : an uncommon manifestation of SLE, Smith LW, Petri M

Journal of Clinical Rheumatology; 2013 Mar 19 (2): 84 – 6

doi : 10.1097 / RHU. 0601383/ 8284794e

3. Annals of Rheumatic Disease, volume 61, Issue 6