Chronic pancreatitis (CP) is a fibro-inflammatory syndrome associated with environmental, genetic, autoimmune, and metabolic factors. Although the main burden of CP is chronic pain and pancreatic insufficiency, epidemiological data indicate that CP patients are at significantly higher risk of developing pancreatic ductal adenocarcinoma (PDAC). CP is a disease that requires complex management based on reducing the severity of symptoms, as there is no curative treatment available. Idiopathic chronic pancreatitis (ICP) is diagnosed after ruling out other possible causes and remains the second most common type of CP—8%–30% of cases. A distinction is made between early-onset ICP (EO-ICP) and late-onset ICP (LO-ICP) based on the age of onset. ICP is characterized by a milder course than CP of other etiologies. In particular, considering the subtypes of ICP, there is a tendency that pain occurs significantly more frequently in EO-ICP compared to alcoholic CP (ACP) and LO-ICP. In turn, diabetes and exocrine pancreatic insufficiency seem to develop much faster in patients with LO-ICP than in patients with ACP and EO-ICP. However, in CP associated with genetic causes, especially mutations in the serine protease 1 ( PRSS1 ) and serine protease inhibitor Kazal type 1 ( SPINK1 ) genes, a significantly higher PDAC risk is observed than in both ICP and ACP. Moreover, patients with ICP are considerably less likely to require intensive pain treatment, including gabapentinoids and opioids. All this translates into a lower frequency of hospitalizations and need for interventional treatment, both endoscopic and surgical. Given that ICP represents a substantial proportion of CP cases, this review highlights possible factors underlying the idiopathic etiology, clinical presentation, and diagnostic features. It summarizes the clinical management of ICP, including the treatment of pain, steatorrhea, diabetes, as well as pancreatic cysts and the risk of PDAC, highlighting differences in treatment patterns and the relative use of specific therapeutic modalities in comparison to other types of CP.
Knopczyk et al. (Wed,) studied this question.