A Multisystem Illness Involving Pancreas, Bile Ducts, and Salivary Glands

Venu Julapalli, MD; Daniel Ball, MD; Ravi Shivshankar, MDSeries Editor: Richard Goodgame, MD


June 17, 2005


Autoimmune chronic pancreatitis is an increasingly recognized cause of pancreatitis. The history of autoimmune pancreatitis is well summarized in a recent review article.[1] Pancreatitis associated with hypergammaglobulinemia and not associated with alcohol consumption was first described by Sarles and colleagues in 1961. The term "autoimmune pancreatitis" was first coined by Yoshida and colleagues in 1995. Most of the literature on this disease has come from Japan, although this may represent a geographic difference in recognition rather than in prevalence.[1]

Our patient's clinical manifestations were quite consistent with those described in the literature. Most reported patients have been male. In the 17 Korean patients, the male:female ratio was 15:2.[4] The clinical manifestations are variable and reflect the anatomic targets of involvement, including the pancreatic parenchyma and duct, biliary duct, and extrapancreatic tissues. Most patients do not manifest recurrent acute pancreatitis. In addition, they have normal or only mildly elevated amylase and lipase levels.[4] The presence of a positive antinuclear antibody is widely variable, and ranges from 11% to 83%.[1] The key diagnostic test is the serum IgG4 level. A good case could be made for ordering this test in all of the following clinical situations: unusual CT and MRI appearance of the pancreas; suspected pancreatic cancer; chronic pancreatitis; or benign biliary strictures. On the basis of the pancreatic pathology, the disease is sometimes called lymphoplasmacytic pancreatitis. However, clinicians should be able to diagnose and treat this condition without biopsy. The dramatic response to corticosteroid therapy, as was seen in our patient, emphasizes the importance of proper and early diagnosis.


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