Pancreatitis, Chronic

BASICS

DESCRIPTION

Long-standing and progressive destruction of the pancreas due to persistent inflammation
Results in exocrine and/or endocrine insufficiency
Major features
- Pain
- Malabsorption
- Diabetes mellitus (type 3)
- Increased risk of pancreatic cancer

EPIDEMIOLOGY

Incidence
Predominant age

35 to 45 years (usually related to alcohol)
Predominant sex: male > female
Hospitalization more common for blacks than whites

Prevalence
8/10,000
Genetics
Hereditary pancreatitis is a rare condition with an autosomal dominant inheritance pattern. Mutations in PRSSI gene and SPINKI gene

GENERAL PREVENTION

Avoid tobacco.
Avoid excess alcohol.

DIAGNOSIS

As with acute pancreatitis, symptoms and objective findings (imaging, amylase/lipase) do not always directly correlate.

HISTORY

Epigastric pain, often radiating to the back
Flare-ups may mimic acute pancreatitis.
Persistent nausea and vomiting
Chronic vague abdominal pain; may worsen postprandially or with alcohol consumption
Alcohol use
Cigarette smoking
Steatorrhea and/or diarrhea
Weight loss
Children
- Recurrent postprandial epigastric pain
- Family history of chronic pancreatitis
- Growth failure
- Diabetes

PHYSICAL EXAM

Acute superimposed on chronic pancreatitis
- See "Pancreatitis, Acute "
- Epigastric tenderness
- Loss of bowel sounds
- Fever
- Tachycardia
- Hypotension/shock
- Jaundice
- Rales/percussive dullness
Chronic pancreatitis
- Mild, diffuse tenderness
- Ascites

DIAGNOSTIC TESTS & INTERPRETATION

Interpret laboratory and radiographic findings in chronic pancreatitis within the context of each patient presentation due to common false-positive and false-negative findings.
Initial Tests (lab, imaging)
Features and considerations

Amylase and lipase usually normal or near normal
Hyperglycemia
Steatorrhea (fecal fat >7 g/day on 100 g of fat per day diet), with other malabsorptive consequences such as low B12 level
Elevated alkaline phosphatase and bilirubin imply obstruction of the intrapancreatic common bile duct.
Autoimmune pancreatitis: elevated serum IgG4 (type I, not type II), autoantibodies to lactoferrin and carbonic anhydrase (1)
Pancreatic insufficiency: fecal elastase-1 <200 ¼g/g in moderate to severe pancreatic insufficiency
Plain film of abdomen: pancreatic calcification if severe
Ultrasound (US): helps assess common bile duct diameter, less useful for visualizing pancreas
CT scan of abdomen: pseudocysts, pancreatic duct dilation, and calcifications, which are most commonly associated with alcohol, cigarette smoking, and hereditary pancreatitis and may take 5 to 10 years to develop (1)
Magnetic resonance cholangiopancreatography (MRCP): pancreatic ductal deformities/strictures (with or without pancreatic ductal stones), retained common bile duct stones
Endoscopic US (EUS): might help identify pancreatic cancer. Fine needle aspiration may be added if autoimmune pancreatitis is suspected (2)[B].

Follow-Up Tests & Special Considerations
Disorders that alter results for amylase or lipase

Biliary tract disease
Penetrating peptic ulcer
Intestinal obstruction, ischemia, or infarction
Ruptured ectopic pregnancy
Renal insufficiency
Burns
Macroamylasemia; macrolipasemia

DIFFERENTIAL DIAGNOSIS

Pancreatic cancer
Lymphoma
Other malabsorptive processes, such as bacterial overgrowth or celiac disease

TREATMENT

MEDICATION

First Line

Analgesics (no consensus on specific medications or doses)
- Tramadol 50 mg q6h PO is commonly used as a first-line.
- Chronic opioid (morphine, fentanyl) therapy may be required in some patients.
- Gabapentin, pregabalin, SNRIs, or TCAs may be used as adjunctive therapy.
- Traditional pancreatic enzyme supplements are microencapsulated and do not require acid inhibition for protection. Dosage is 25,000 to 40,000 IU at the beginning of the meal.
- Uncoated enzymes (Viokase) may be more efficacious for pain control (when given with proton pump inhibitors [PPIs] to protect their integrity) compared with coated enzymes (3,4). There is a theoretical benefit to using PPIs, even with coated enzymes, in patients with vitamin deficiency to allow faster release of enzymes into the proximal duodenum where fat soluble vitamin absorption occurs (1).
- Octreotide 25 to 50 ¼g/hr IV infusion can be supplemental therapy for pancreatic ductal fistulae, which can later be changed to long-acting SC administration.
- Individualized doses of corticosteroids for autoimmune pancreatitis

GENERAL MEASURES

Discontinue tobacco use, which can exacerbate chronic pancreatitis (5).
Analgesia: Consider pain management consultation for chronic opioid management.
Exocrine and endocrine replacement therapy (enzymes and insulin)
Consider more advanced therapy for pain: celiac ganglion block via EUS and endoscopic or surgical decompression of partially obstructed pancreatic duct.

SURGERY/OTHER PROCEDURES

Pseudocyst drainage
- Conservative approach if asymptomatic
- Endoscopic (via EUS) or surgical approach if mature wall
- Percutaneous approach if rapidly enlarging or if thin wall
- Endoscopic drainage and percutaneous drainage have similar clinical success rates, but percutaneous drainage requires more reintervention and longer hospital stay (6)[C].
Pancreatic ascites with disruption of the main pancreatic duct
- Endoscopic placement of pancreatic ductal stent is preferred, if possible.
- Lateral pancreaticojejunostomy if endoscopic therapy is not possible
Biliary obstruction (secondary to chronic pancreatitis, not choledocholithiasis)
- Placement of retrievable metal stent or multiple plastic stents, if possible
- Choledochojejunostomy if endoscopic therapy is not possible
Pancreatic ductal obstruction by stone
- Endoscopic pancreatic sphincterotomy with stone extraction
Total pancreatectomy and islet autotransplantation
- Favorable outcomes with pain reduction
- Indication for timing of procedure uncertain
Lithotripsy for chronic calcific pancreatitis may improve pain and decrease dependence on narcotic analgesics (7)[B].

INPATIENT CONSIDERATIONS

Discharge Criteria

Pain control
Resolution of problems secondary to ductal disruption, if present
Alcohol rehab and smoking cessation, if needed

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Depends on the source of the pain and whether a ductal disruption exists

DIET

Small meals high in protein, ~20 g/day of fat; adjust if diabetes is present. Patients with decreased endocrine function from chronic pancreatitis can be prone to hyper- or hypoglycemia (8)[C].
Pancreatic enzyme replacement therapy (coated/microencapsulated for pancreatic insufficiency but noncoated with PPI for pancreatic pain)
Vitamin A and vitamin E deficiency are not very common " levels should be checked and supplements provided only if deficiencies are noted (8)[C].

PROGNOSIS

Patients with recurrent episodes of acute pancreatitis are more likely to develop chronic pancreatitis.
May "burn out " with resolution of symptoms
Narcotic addiction occurs frequently.
Pancreatic exocrine and/or endocrine insufficiency may occur years later.
Patients with chronic pancreatitis have an increased mortality, particularly from pancreatic cancer (9).
25% of patients with chronic pancreatitis have osteoporosis. Perform routine bone health assessment (10)[A].

REFERENCES

11 Forsmark CE. Management of chronic pancreatitis. Gastroenterology. 2013;144(6):1282.e3 " 1291.e3.22 Iwashita T, Yasude I, Doi S, et al. Use of samples from endoscopic ultrasound-guided 19-gauge fine-needle aspiration in diagnosis of autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2012;10(3):316 " 322.33 Slaff J, Jacobson D, Tillman CR, et al. Protease-specific suppression of pancreatic exocrine secretion. Gastroenterology. 1984;87(1):44 " 52.44 Puylaert M, Kapural L, Van Zundert J, et al. 26. Pain in chronic pancreatitis. Pain Pract. 2011;11(5):492 " 505.55 Yadav D, Hawes RH, Brand RE, et al. Alcohol consumption, cigarette smoking, and the risk of recurrent acute and chronic pancreatitis. Arch Intern Med. 2009;169(11):1035 " 1045.66 Akshintala VS, Saxena P, Zaheer A, et al. A comparative evaluation of outcomes of endoscopic versus percutaneous drainage for symptomatic pancreatic pseudocysts. Gastrointest Endosc. 2014;79(6):921 " 928.77 Seven G, Schreiner MA, Ross AS, et al. Long-term outcomes associated with pancreatic extracorporeal shock wave lithotripsy for chronic calcific pancreatitis. Gastrointest Endosc. 2012;75(5):997.e1 " 1004.e1.88 Duggan S, Conlon K. A practical guide to the nutritional management of chronic pancreatitis. Pract Gastroenterol. 2013;37:24 " 32.99 Bang UC, Benfield T, Hyldstrup L, et al. Mortality, cancer, and comorbidities associated with chronic pancreatitis: a Danish nationwide matched-cohort study. Gastroenterology. 2014;146(4):989 " 994.1010 Duggan SN, Smyth ND, Murphy A, et al. High prevalence of osteoporosis in patients with chronic pancreatitis: a systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2014;12(2):219 " 228.

ADDITIONAL READING

Bornman PC, Botha JF, Ramos JM, et al. Guideline for the diagnosis and treatment of chronic pancreatitis. S Afr Med J. 2010;100(12 Pt 2):845 " 860.
Bramis K, Gordon-Weeks AN, Friend PJ, et al. Systematic review of total pancreatectomy and islet autotransplantation for chronic pancreatitis. Br J Surg. 2012;99(6):761 " 766.
Feurer M, Adler D. Evaluation and management of pancreatic pseudocysts. Pract Gastroenterol. 2014;38:12 " 25.
Giuliano CA, Dehoorne-Smith ML, Kale-Pradhan PB. Pancreatic enzyme products: digesting the changes. Ann Pharmacother. 2011;45(5):658 " 666.
Niemann T, Madsen LG, Larsen S, et al. Opioid treatment of painful chronic pancreatitis. Int J Pancreatol. 2000;27(3):235 " 240.
Yadav D, O 'Connell M, Papachristou GI. Natural history following the first attack of acute pancreatitis. Am J Gastroenterol. 2012;107(7):1096 " 1103.

CODES

ICD10

K86.1 Other chronic pancreatitis
K86.0 Alcohol-induced chronic pancreatitis

ICD9

577.1 Chronic pancreatitis

SNOMED

235494005 Chronic pancreatitis (disorder)
235952002 Alcohol-induced chronic pancreatitis (disorder)
235954001 Obstructive chronic pancreatitis (disorder)

CLINICAL PEARLS

EUS can help differentiate chronic pancreatitis from pancreatic cancer.
Chronic pain management is an essential element of managing chronic pancreatitis.
Pancreatic enzyme replacement therapy and the avoidance of tobacco and alcohol are cornerstone therapies for chronic pancreatitis.

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