Challenges in the management of pancreatic exocrine insufficiency

Abstract

Pancreatic exocrine insufficiency (PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condition is associated with significant morbidity and reductions in quality of life, even in milder forms. The challenges in approaching this condition include the non-specific presentation of mild to moderate PEI, and the lack of a convenient, accurate diagnostic test in this cohort. Classical symptoms appear late in the disease, and the diagnosis should be considered before steatorrhoea develops. Direct pancreatic function tests are the reference standard for diagnosis, but are invasive and not widely available. The faecal elastase-1 (FE-1) stool test is widely available and has been shown to be as effective as the ¹³C-mixed triglyceride breath test in more advanced disease. We recommend a pragmatic diagnostic approach that combines clinical history, assessment of nutritional status and measurement of FE-1. The critical first step is to consider the diagnosis. Once the diagnosis is confirmed, pancreatic enzyme replacement therapy should be initiated. The variety of enzyme preparations and recommended dosing regimens can present a challenge when selecting an adequate initial dose. Non-response should be actively sought and addressed in a systematic manner. This article discusses these challenges, and presents a practical approach to the diagnosis and management of PEI.

Keywords: Pancreatic exocrine insufficiency, Chronic pancreatitis, Steatorrhoea, Pancreatic function tests, Pancreatic enzyme replacement therapy

Core tip: Pancreatic exocrine insufficiency (PEI) is common, and the prevalence is likely to increase in line with global trends in associated conditions (notably increasing age and diabetes mellitus). The classical symptom of steatorrhoea is a late presentation of PEI. The diagnosis should be considered far earlier, based on risk factors and clinical history. A current, pragmatic approach to diagnosis combines clinical history, assessment of nutritional status and measurement of faecal elastase-1. Treatment with pancreatic enzyme replacement therapy (PERT) is safe and effective. PERT must be adequately dosed, monitored, and optimized to ensure its benefits are realized.