Huge water-balloons in the belly: multiple pancreatic pseudocysts in systemic lupus erythematosus.

Abstract

A 28-year-old woman presented with recurrent epigastric pain and abdominal distention for 6 months. Her symptoms first occurred on the first day of admission due to SLE flare (fever, polyarthritis, anaemia, leukocytopenia, proteinuria, low C3, positive tests on antinuclear, anti-double-stranded DNA, anti-Smith and anti-cardiolipin antibodies), when no treatment was initiated. Elevated blood amylase and CT (Fig. 1A) indicated acute pancreatitis and other common causes were excluded. Thrombotic microangiopathy (TMA) was then suspected because of renal, neuropsychiatric and haematological involvements. However, abdominal distention and peripancreatic effusions perpetuated though SLE, TMA and secondary infection remitted after using glucocorticoid, immunosuppressive agents, plasma exchange and supportive therapies. Therefore, drainage was performed a month before this presentation and CT (Fig. 1B) showed improvement. During this admission, emergency drainage was performed due to reappearing ascites. Analysis of chylous ascites identified extremely elevated amylase and lipase with Enterococcus faecium as pathogen. Massive ascites were encapsulated with a fistula connected to the newly emerged pseudocyst (Fig. 1C and D). Drainage was not removed until radiographic remission 7 months later (Fig. 1E). SLE flare and abdominal symptoms have not occurred up to the last follow-up 41 months after this admission. This case illustrates SLE-associated acute pancreatitis can present with atypical features [1, 2] and prolonged courses due to ‘add-on’ effects of SLE flare, infection, coagulopathy (thrombocytopenic purpura, antiphospholipid syndrome) and medication. Therefore, a tailored management strategy is required.