Autoimmune pancreatitis as the initial presentation of systemic lupus erythematosus.

Pancreatitis is a relatively rare complication in systemic lupus erythematosus (SLE). Here we present a case of SLE associated with autoimmune pancreatitis. A 37-year old woman was admitted to our hospital because of fever, skin rash, proteinuria and abdominal pain. A diagnosis of SLE was made based on her clinical, laboratory and renal histological findings showing diffuse proliferative lupus nephritis. Elevated serum amylase, typical radiographic findings and selective increase in serum IgG4 all suggested that the patient also had autoimmune pancreatitis. Systemic administration of glucocorticoid successfully induced remission of pancreatitis and nephritis along with the reduction of IgG4. Autoimmune pancreatitis is a newly recognized type of pancreatitis, in which IgG4 immune response is thought to participate pathophysiologically. Although the disease has been observed to develop in patients having various connective tissue diseases, our report is the first to describe IgG4-related autoimmune pancreatitis in a patient with SLE.