Autoimmune Pancreatitis without a Response to Steroid Therapy: A Case which Met Criteria after Withdrawal of Steroid

Autoimmune pancreatitis (AIP) sometimes forms a pancreatic mass lesion, which is often difficult to distinguish from pancreatobiliary malignancy, however it generally responds to steroid therapy. A 70-year-old man was referred to our institute with the suspected diagnosis of pancreatic cancer due to a mass lesion detected at the pancreatic head. Various images demonstrated an ill-defined mass at the enlarged pancreatic head with focal narrowing of the main pancreatic duct. Serum antinuclear antibody (ANA) was negative (x40 dilution) on the onset. Forceps biopsy from the narrowed pancreaticobiliary duct and fine-needle aspiration biopsy under endoscopic ultrasonography (EUS-FNAB) ruled out pancreatobiliary malignancy. Steroid therapy was started at 40 mg per day but was not effective according to subsequent image analyses. Repeated EUS-FNA from the pancreatic mass was performed but was again negative for carcinoma. Seven months later, under steroid-off condition, still no response was recognized in the clinical image but the titer of serum ANA was increased to be positive (x80), satisfying the criteria of AIP in Japan (2006). Although very rare, this is a case meeting Japanese criteria of AIP after withdrawal of steroid without response to steroid in the clinical images, suggesting the necessity of careful follow-up.