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CECT Abdomen showing bulky pancreas with mild peripancreatic fluid collection suggestive of acute pancreatitis. 

CECT Abdomen showing bulky pancreas with mild peripancreatic fluid collection suggestive of acute pancreatitis. 

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Acute pancreatitis as an initial presentation of SLE in paediatric age group is extremely rare and needs high level of suspicion to make timely diagnosis as results may be fatal when not treated on time.

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... of abdomen showed a bulky, hypo echoic pancreas with presence of free fluids in peritoneal cavity. Contrast enhanced computed tomography (CECT) abdomen confirmed diagnosis of acute pancreatitis (Fig 2). The connective tissue workup showed raised Anti-double-standard DNA antibody positivity (56.07) with elevated antinuclear antibody titre (>159 U/L) with low C3 level (75.70) supporting the diagnosis of SLE. ...

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... Still, our child has not had any such effect, which could have also contributed to the excellent prognosis. [17] Early diagnosis, suspected in front of any abdominal pain and appropriate treatment with high-dose corticosteroids and immunosuppressive therapy has shown improvement in prognosis. According to International Study Group of Pediatric Pancreatitis: In search for a cure (INSPPIRE) standardised definitions, pancreatitis in SLE patients is mostly an acute subtype with rare recurrence or progression to chronic damage. ...
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Systemic lupus erythematosus (SLE) is a multi-system and chronic inflammatory autoimmune disease characterised by the presence of autoantibodies and the creation of immune complexes, coming up with protean manifestations. Its occurrence in children is rare and acute pancreatitis as the presenting manifestation of childhood SLE is exceptional. Diagnosis of lupus pancreatitis is clinical, biological and radiological. Here, we report an 8 year 8-month-old young girl who presented to our hospital with abdominal pain, nausea and vomiting. She also had a fever, alopecia, oral ulceration and glossitis. Her investigations showed elevated serum amylase and lipase levels, suggestive of acute pancreatitis. Other investigations revealed pancytopenia, low complement, microalbuminuria and high antinuclear antibodies level, indicative of SLE. As a result, the diagnosis of lupus pancreatitis was retained. Her clinical symptoms and signs markedly improved after a course of glucocorticoid and immunosuppressive therapy. The child is now being brought for regular follow-up and is doing well.
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Pancreatitis is uncommon in systemic lupus erythematosus (SLE) and is rarely reported in children, possibly being related to macrophage activation syndrome (MAS). The incidence of MAS in children with lupus pancreatitis is unknown, as is their prognosis. In this case-based review, we report a pediatric patient with SLE complicated with pancreatitis and MAS, and performed a literature review. We report an 11-year-old girl with SLE and MAS who developed pancreatitis on the second day of methylprednisolone pulse therapy (500 mg/day). We continued methylprednisolone pulse therapy, and performed three rounds of DNA-immunoadsorption and three rounds of hemoperfusion. A second course of methylprednisolone pulse therapy was initiated 9 days later. The patient received a monthly cyclophosphamide pulse therapy (10 mg/kg/day, 2 consecutive days every month) for 6 months, after which she was treated with mycophenolate mofetil 20 mg/kg/day. The condition of the patient gradually improved, her blood amylase and lipase decreased. She was in a stable condition during 13-month follow-up period. Review of the literature of pediatric patients with SLE and pancreatitis showed that there are 127 cases that have been reported in the past 30 years, 40 cases were excluded in our study because of inadequate information. Of the 87 patients included in our literature review, the mortality rate was 33.33%, and 52.86% of the patients with pancreatitis had MAS at the same time. Pancreatitis is uncommon in SLE, but must be suspected if a patient with SLE develops digestive symptoms. Patients with SLE with pancreatitis have a high incidence of MAS and high mortality rate; however, early recognition and effective treatment can relieve the disease symptoms.