Autoimmune chronic pancreatitis relapsing despite the maintenance dose of steroid

Autoimmune chronic pancreatitis is a disease characterized by diffuse swelling of the pancreas, irregular narrowing of the main pancreatic duct and elevated levels of serum IgG, and lymphoplasmacytic infiltration is observed on histologic examination. Steroid therapy can dramatically reverse the clinical symptoms and the histologic and radiologic findings. It is known that recurrence is very rare after successful steroid treatment. Furthermore, there have not yet been any reports about a case that relapsed during maintenance therapy with low dose steroid. We experienced a rare case of autoimmune chronic pancreatitis that relapsed despite maintenance therapy with low-dose steroid, and the patient again responded to high-dose steroid.