Chronic Pancreatitis Associated with Systemic Lupus Erythematosus in a Young Girl

Systemic lupus erythematosus (SLE) is an uncommon etiology of pancreatic disease. Up to now, only 3 cases of chronic pancreatitis associated with SLE have been reported in adults. We report the case of a 14-year-old girl with SLE and calcifying chronic pancreatitis. At the age of 4 she was diagnosed with SLE. She presented with several acute exacerbations of SLE that were managed with prednisone and azathioprine. At the age of 9, she was admitted with abdominal pain and elevation of serum amylase and lipase levels; no gallstones were found on ultrasound, and treatment with azathioprine was withdrawn. Thereafter, she developed numerous episodes of acute pancreatitis. Later, an ERCP showed pancreatic calcifications and distortion of the main pancreatic duct, both findings consistent with established chronic pancreatitis. At the age of 14, her condition worsened progressively, and a surgical procedure (corporo-caudal spleno-pancreatectomy) was performed. The pathology specimen showed acinar atrophy and intense fibrosis. After surgery, the patient has remained pain-free and is enjoying a normal life.