Chronic inflammatory sclerosis of the pancreas—An autonomous pancreatic disease?

A statistical study of 98 patients with pancreatitis leads us to think that the “monist” conception of “recurrent chronic pancreatitis”—according to which the noncancerous pathologic change is confined to a single affection that either may kill at the first attack (hemorrhagic pancreatitis) or be repeated and slowly lead to sclerosis and, later, endoductal calcification—neglected the true facts in favor of a too rigorous classification. Among the affections which the anatomicoclinical method has permitted us to isolate, a particularly serious form of chronic pancreatitis is to be noted: “primary inflammatory sclerosis.” It appears in later life (average age of onset, 61.4 years) and is found as often in women as in men; in most cases it progresses to cachexia and death. The relatively long duration of the evolution makes certain small, slowly developing duodenal or pancreatic cancers complicated by distal pancreatitis the only condition that need be considered in the differential diagnosis. Both clinical evolution and anatomic aspect are marked by significant signs of inflammation. Despite repeated attempts, no germ has been discovered. It is thus possible to put forward the hypothesis that this type of pancreatitis is an inflammatory, noninfectious disease that is caused by phenomena of self-immunization.