Table 1.

Conditions/therapies associated with pancreatic exocrine insufficiency

Type of issue	Specific conditions	Prevalence
Intrinsic pancreatic disease	Chronic pancreatitis neoplasia	94% within 10 years of onset of chronic pancreatitis.18 66%–94% of patients with unresectable pancreatic cancer.19 20
Reduced CCK secretion	Coeliac disease	4%–80% in untreated coeliac disease (measured by FE-1).21–26 12% in patients with chronic diarrhoea on a gluten-free diet (based on pancreatic testing or trial of PERT) 18% (based on steatorrhoea and trial of PERT).27 28
Congenital disease	Pancreaticum divisum cystic fibrosis	48% based on faecal fat excretion.29 85% before the age of 1 year.14
Inflammatory bowel disease	Crohn’s disease	14%–30%.11 30
	Ulcerative colitis	Up to 22% using FE.11 Up to 50% using a secretin-cerulein test and 74% using para-aminobenzoic acid test.31 32
Bile acid malabsorption	May coexist27	No data but should be considered.13
Small intestinal bacterial overgrowth	May coexist27	Consider antibiotic therapy in those not responding to/not tolerating PERT.33
HIV disease	Due to disease34 35 or secondary to antiretroviral medication particularly Didanosine31 36	Up to 54% with improvements in faecal fat loss following institution of PERT.37 38
Diabetes mellitus	Type 1 diabetes Type 2 diabetes	Up to 6% in type 1 diabetics with diarrhoea39 and 26–44% otherwise.40–43 12%–20%40–43. Inadequate data whether there is any symptom improvement with PERT in those without diarrhoea.44
Oncological therapies	Tyrosine kinase inhibitors Checkpoint inhibitors Somatostatin analogues	7% in those treated with sorafenib.45 1% after nivolumab46 47 and reported after pembrolizumab.48 49 Chronic use can affect up to 38%.50

CCK, cholecystokinin; FE-1, faecal elastase-1; PERT, pancreatic enzyme replacement therapy.