Background and aims Acute pancreatitis is a rare initial presentation of systemic lupus erythematosus.

Methods We report two cases of Malay patients who presented with fever, abdominal pain, ascites and high level of pancreatic enzymes. They were diagnosed with acute pancreatitis associated with systemic lupus erythematosus.

Results First patient is a Malay male patient presented to us late. He had severe acute pancreatitis with severe hyperbilirubinemia, lupus nephritis and autoimmune haemolytic anaemia. He died despite maximal therapy was provided. The second case is a Malay lady who came to us early responded well to high dose corticosteroids and discharged well.

Conclusions The mortality rate of lupus-associated pancreatitis is higher than in non-lupus associated pancreatitis. Early awareness of lupus-associated pancreatitis as an initial manifestation of systemic lupus erythematosus is crucial in managing and improving outcome in lupus patients.