Pancreatitis

This guideline covers managing acute and chronic pancreatitis in children, young people and adults. It aims to improve quality of life by ensuring that people have the right treatment and follow-up, and get timely information and support after diagnosis.

# Recommendations
People have the right to be involved in discussions and make informed decisions about their care, as described in NICE's information on making decisions about your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off-label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

# Information and support

## Patient information
Give people with pancreatitis, and their family members or carers (as appropriate), written and verbal information on the following, where relevant, as soon as possible after diagnosis:
pancreatitis and any proposed investigations and procedures, using diagrams
hereditary pancreatitis, and pancreatitis in children, including specific information on genetic counselling, genetic testing, risk to other family members, and advice on the impact of their pancreatitis on life insurance and travel
the long-term effects of pancreatitis, including effects on the person's quality of life
the harm caused to the pancreas by smoking or alcohol.
Advise people with pancreatitis where they might find reliable high-quality information and support after consultations, from sources such as national and local support groups, regional pancreatitis networks and information services.
Give people with pancreatitis, and their family members or carers (as appropriate), written and verbal information on the following about the management of pancreatitis, when applicable:
why a person may be going through a phase where no treatment is given
that pancreatitis is managed by a multidisciplinary team
the multidisciplinary treatment of pain, including how to access the local pain team and types of pain relief
nutrition advice, including advice on how to take pancreatic enzyme replacement therapy if needed
follow-up and who to contact for relevant advice, including advice needed during episodes of acute illness
psychological care if needed, where available (see the NICE guideline on depression in adults)
pancreatitis services, including the role of specialist centres, and primary care services for people with acute, chronic or hereditary pancreatitis
welfare benefits, education and employment support, and disability services.
For more guidance on giving information, including providing an individualised approach and helping people to actively participate in their care, see the NICE guideline on patient experience in adult NHS services.
Explain to people with severe acute pancreatitis, and their family members or carers (as appropriate), that:
a hospital stay lasting several months is relatively common, including time in critical care
for people who achieve full recovery, time to recover may take at least 3 times as long as their hospital stay
local complications of acute pancreatitis may resolve spontaneously or may take weeks to progress before it is clear that intervention is needed
it may be safer to delay intervention (for example, to allow a fluid collection to mature)
people who have started to make a recovery may have a relapse
although children rarely die from acute pancreatitis, approximately 15% to 20% of adults with severe acute pancreatitis die in hospital.
Tell adults with pancreatitis that there is a NICE guideline on patient experience in adult NHS services that will show them what they can expect about their care.

## Passing information to GPs
Ensure that information passed to GPs includes all of the following, where applicable:
detail on how the person should take their pancreatic enzyme replacement therapy (including dose escalation as necessary)
that the person should be offered HbA1c testing at least every 6 months and bone mineral density assessments every 2 years.

## Lifestyle interventions: alcohol
Advise people with pancreatitis caused by alcohol to stop drinking alcohol.
Advise people with recurrent acute or chronic pancreatitis that is not alcohol-related, that alcohol might exacerbate their pancreatitis.
For guidance on alcohol-use disorders, see the NICE guidelines on the diagnosis and management of physical complications of alcohol-use disorders and the diagnosis, assessment and management of harmful drinking and alcohol dependence.

## Lifestyle interventions: smoking cessation
Be aware of the link between smoking and chronic pancreatitis and advise people with chronic pancreatitis to stop smoking in line with the NICE guideline on tobacco.

# Acute pancreatitis
People with acute pancreatitis usually present with sudden-onset abdominal pain. Nausea and vomiting are often present and there may be a history of gallstones or excessive alcohol intake. Typical physical signs include epigastric tenderness, fever and tachycardia. Diagnosis of acute pancreatitis is confirmed by testing blood lipase or amylase levels, which are usually raised. If raised levels are not found, abdominal CT may confirm pancreatic inflammation.

## Identifying the cause
Do not assume that a person's acute pancreatitis is alcohol-related just because they drink alcohol.
If gallstones and alcohol have been excluded as potential causes of a person's acute pancreatitis, investigate other possible causes such as:
metabolic causes (such as hypercalcaemia or hyperlipidaemia)
prescription drugs
microlithiasis
hereditary causes
autoimmune pancreatitis
ampullary or pancreatic tumours
anatomical anomalies (pancreas divisum).

## Preventing infection
Do not offer prophylactic antimicrobials to people with acute pancreatitis.

## Fluid resuscitation
For guidance on fluid resuscitation, see the NICE guidelines on intravenous fluid therapy in adults in hospital and in children and young people in hospital.

## Nutrition support
Ensure that people with acute pancreatitis are not made 'nilbymouth' and do not have food withheld unless there is a clear reason for this (for example, vomiting).
Offer enteral nutrition to anyone with severe or moderately severe acute pancreatitis. Start within 72 hours of presentation and aim to meet their nutritional requirements as soon as possible.
Offer anyone with severe or moderately severe acute pancreatitis parenteral nutrition only if enteral nutrition has failed or is contraindicated.

## Managing complications
Offer people with acute pancreatitis an endoscopic approach for managing infected or suspected infected pancreatic necrosis when anatomically possible.
Offer a percutaneous approach when an endoscopic approach is not anatomically possible.
When deciding on how to manage infected pancreatic necrosis, balance the need to debride promptly against the advantages of delaying intervention.
For guidance on managing pseudocysts, see the recommendations in the section on pseudocysts in managing complications of chronic pancreatitis.
For guidance on managing pancreatic ascites and pleural effusion secondary to pancreatitis, see the recommendation in the section on pancreatic ascites and pleural effusion in managing complications of chronic pancreatitis.
For guidance on managing type 3c diabetes secondary to pancreatitis, see the recommendations in the section on type 3c diabetes in managing complications of chronic pancreatitis.

## Referral for specialist treatment
If a person develops necrotic, infective, haemorrhagic or systemic complications of acute pancreatitis:
seek advice from a specialist pancreatic centre within the referral network and
discuss whether to move the person to the specialist centre for treatment of the complications.
When managing acute pancreatitis in children:
seek advice from a paediatric gastroenterology or hepatology unit and a specialist pancreatic centre and
discuss whether to move the child to the specialist centre.

# Chronic pancreatitis
People with chronic pancreatitis usually present with chronic or recurrent abdominal pain. This guideline assumes that people with chronic abdominal pain will already have been investigated using CT scan, ultrasound scan or upper gastrointestinal endoscopy to determine a cause for their symptoms. The guideline committee looked at evidence on diagnosing chronic pancreatitis, and the evidence review can be found in the full guideline. We have made a recommendation for research on the most accurate diagnostic test to identify whether chronic pancreatitis is present in the absence of a clear diagnosis following these tests.

## Investigating upper abdominal pain
Think about chronic pancreatitis as a possible diagnosis for people presenting with chronic or recurrent episodes of upper abdominal pain and refer accordingly.

## Identifying the cause
Do not assume that a person's chronic pancreatitis is alcohol-related just because they drink alcohol. Other causes include:
genetic factors
autoimmune disease, in particular IgG4 disease
metabolic causes
structural or anatomical factors.

## Nutrition support
Be aware that all people with chronic pancreatitis are at high risk of malabsorption, malnutrition and a deterioration in their quality of life.
Use protocols agreed with the specialist pancreatic centre to identify when advice from a specialist dietitian is needed, including advice on food, supplements and long-term pancreatic enzyme replacement therapy, and when to start these interventions.
Consider assessment by a dietitian for anyone diagnosed with chronic pancreatitis.
For guidance on nutrition support for people with chronic alcohol-related pancreatitis, see the section on enzyme supplementation for chronic alcohol-related pancreatitis in the NICE guideline on alcohol-use disorders.
For guidance on nutrition support, see the NICE guideline on nutrition support for adults.

## Managing complications
Consider surgery (open or minimally invasive) as first-line treatment in adults with painful chronic pancreatitis that is causing obstruction of the main pancreatic duct.
Consider extracorporeal shockwave lithotripsy for adults with pancreatic duct obstruction caused by a dominant stone if surgery is unsuitable.
Offer endoscopic ultrasound (EUS)-guided drainage, or endoscopic transpapillary drainage for pancreatic head pseudocysts, to people with symptomatic pseudocysts (for example, those with pain, vomiting or weight loss).
Consider EUS-guided drainage, or endoscopic transpapillary drainage for pancreatic head pseudocysts, for people with non-symptomatic pseudocysts that meet 1 or more of the following criteria:
they are associated with pancreatic duct disruption
they are creating pressure on large vessels or the diaphragm
they are at risk of rupture
there is suspicion of infection.
Consider surgical (laparoscopic or open) drainage of pseudocysts that need intervention if endoscopic therapy is unsuitable or has failed.
For adults with neuropathic pain related to chronic pancreatitis, follow the recommendations in the NICE guideline on neuropathic pain in adults.
Consider referring a person with pancreatic ascites and pleural effusion for management in a specialist pancreatic centre.
Assess people with type 3c diabetes every 6 months for potential benefit of insulin therapy.
For guidance on managing type 3c diabetes for people who are not using insulin therapy, see the NICE guidelines on type 2 diabetes in adults and diagnosing and managing diabetes in children and young people.
For guidance on managing type 3c diabetes for people who need insulin, see:
the recommendations on insulin therapy and insulin delivery (including rotating injection sites within the same body region) in the NICE guidelines on type 1 diabetes in adults and diagnosing and managing diabetes in children and young people
NICE's technology appraisal guidance on continuous subcutaneous insulin infusion for the treatment of diabetes mellitus. 
For guidance on education and information for people with pancreatitis and type 3c diabetes requiring insulin, see the recommendations on education and information in the NICE guideline on diagnosing and managing type 1 diabetes in adults, and diagnosing and managing diabetes in children and young people.
For guidance on self-monitoring blood glucose for people with pancreatitis and type 3c diabetes requiring insulin, see the recommendations on blood glucose management in the NICE guideline on diagnosing and managing type 1 diabetes in adults, and blood glucose monitoring in the NICE guideline on diagnosing and managing diabetes in children and young people.

## Follow-up investigation
Offer people with chronic pancreatitis monitoring by clinical and biochemical assessment, to be agreed with the specialist centre, for pancreatic exocrine insufficiency and malnutrition at least every 12 months (every 6 months in under 16s). Adjust the treatment of vitamin and mineral deficiencies accordingly.
Offer adults with chronic pancreatitis a bone density assessment every 2 years.
Be aware that people with chronic pancreatitis have an increased risk of developing pancreatic cancer. The lifetime risk is highest, around 40%, in those with hereditary pancreatitis.
Consider annual monitoring for pancreatic cancer in people with hereditary pancreatitis.
Be aware that people with chronic pancreatitis have a greatly increased risk of developing diabetes, with a lifetime risk as high as 80%. The risk increases with duration of pancreatitis and presence of calcific pancreatitis.
Offer people with chronic pancreatitis monitoring of HbA1c for diabetes at least every 6 months.

# Terms used in this guideline

## Moderately severe acute pancreatitis
Moderately severe acute pancreatitis is characterised by organ failure that resolves within 48 hours (transient organ failure), or local or systemic complications in the absence of persistent organ failure (as defined by the revised Atlanta classification published in GUT).

## Severe acute pancreatitis
Severe acute pancreatitis is characterised by single or multiple organ failure that persists for more than 48 hours (persistent organ failure; as defined by the revised Atlanta classification).

## Type 3c diabetes
Diabetes mellitus secondary to pancreatic disease. When this is associated with pancreatitis, the primary endocrine defect is insufficient insulin secretion (the abnormality in type 1 diabetes) rather than insulin resistance (which is characteristic of type 2 diabetes).# Putting this guideline into practice
We have produced NICE tools and resources to help you put this guideline into practice.
Some issues were highlighted that might need specific thought when implementing the recommendations. These were raised during the development of this guideline. They are:
Models where local centres interact and collaborate with a regional specialist centre for acute pancreatitis are only currently established in some regions. Therefore, this model will need to be implemented across the country to enable the recommendations on specialist referral to be followed.
Networks of dietitians and specialist dietitians need to be established to support the production and dissemination of protocols to identify when advice from a specialist dietitian is needed.
Putting recommendations into practice can take time. How long may vary from guideline to guideline, and depends on how much change in practice or services is needed. Implementing change is most effective when aligned with local priorities.
Changes recommended for clinical practice that can be done quickly  like changes in prescribing practice  should be shared quickly. This is because healthcare professionals should use guidelines to guide their work  as is required by professional regulating bodies such as the General Medical and Nursing and Midwifery Councils.
Changes should be implemented as soon as possible, unless there is a good reason for not doing so (for example, if it would be better value for money if a package of recommendations were all implemented at once).
Different organisations may need different approaches to implementation, depending on their size and function. Sometimes individual practitioners may be able to respond to recommendations to improve their practice more quickly than large organisations.
Here are some pointers to help organisations put NICE guidelines into practice:
. Raise awareness through routine communication channels, such as email or newsletters, regular meetings, internal staff briefings and other communications with all relevant partner organisations. Identify things staff can include in their own practice straight away.
. Identify a lead with an interest in the topic to champion the guideline and motivate others to support its use and make service changes, and to find out any significant issues locally.
. Carry out a baseline assessment against the recommendations to find out whether there are gaps in current service provision.
. Think about what data you need to measure improvement and plan how you will collect it. You may want to work with other health and social care organisations and specialist groups to compare current practice with the recommendations. This may also help identify local issues that will slow or prevent implementation.
. Develop an action plan, with the steps needed to put the guideline into practice, and make sure it is ready as soon as possible. Big, complex changes may take longer to implement, but some may be quick and easy to do. An action plan will help in both cases.
. For very big changes include milestones and a business case, which will set out additional costs, savings and possible areas for disinvestment. A small project group could develop the action plan. The group might include the guideline champion, a senior organisational sponsor, staff involved in the associated services, finance and information professionals.
. Implement the action plan with oversight from the lead and the project group. Big projects may also need project management support.
. Review and monitor how well the guideline is being implemented through the project group. Share progress with those involved in making improvements, as well as relevant boards and local partners.
NICE provides a comprehensive programme of support and resources to maximise uptake and use of evidence and guidance. See NICE's into practice pages for more information.
Also see Leng G, Moore V, Abraham S, editors (2014) Achieving high quality care  practical experience from NICE. Chichester: Wiley.# Context
Acute pancreatitis is acute inflammation of the pancreas and is a common cause of acute abdominal pain. The incidence in the UK is approximately 56 cases per 100,000 people per year. Around 50% of cases are caused by gallstones, 25% by alcohol and 25% by other factors. In 25% of cases, acute pancreatitis is severe and associated with complications such as respiratory or kidney failure, or the development of abdominal fluid collections. In these more severe cases, people often need critical care and a prolonged hospital stay, and the mortality rate is 25%. The overall mortality rate in acute pancreatitis is approximately 5%.
Chronic pancreatitis is a continuous prolonged inflammatory process of the pancreas that results in fibrosis, cyst formation and stricturing of the pancreatic duct. It usually presents with chronic abdominal pain but it can sometimes be painless. The clinical course is variable but most people with chronic pancreatitis have had 1 or more attacks of acute pancreatitis that has resulted in inflammatory change and fibrosis. In some people, however, chronic pancreatitis has a more insidious onset. The intensity of pain can range from mild to severe, even in people with little evidence of pancreatic disease on imaging.
The annual incidence of chronic pancreatitis in western Europe is about 5 new cases per 100,000 people, although this is probably an underestimate. The male to female ratio is 7:1 and the average age of onset is between 36 and 55 years. Alcohol is responsible for 7080% of cases of chronic pancreatitis. Although cigarette smoking is not thought to be a primary cause in itself, it is strongly associated with chronic pancreatitis and is thought to exacerbate the condition. Chronic pancreatitis may be idiopathic or, in about 5% of cases, caused by hereditary factors (in these cases there is usually a positive family history). Other causes include hypercalcaemia, hyperlipidaemia or autoimmune disease.
Chronic pancreatitis causes a significant reduction in pancreatic function and the majority of people have reduced exocrine (digestive) function and reduced endocrine function (diabetes). They usually need expert dietary advice and medication. Chronic pancreatitis can also give rise to specific complications including painful inflammatory mass and obstructed pancreatic duct, biliary or duodenal obstruction, haemorrhage, or accumulation of fluid in the abdomen (ascites) or chest (pleural effusion). Managing these complications may be difficult because of ongoing comorbidities and social problems such as alcohol or opiate dependence. Chronic pancreatitis significantly increases the risk of pancreatic cancer. This risk is much higher in people with hereditary pancreatitis.# Recommendations for research
The guideline committee has made the following recommendations for research. The committee's full set of recommendations for research are detailed in the full guideline.

# Diagnosis of chronic pancreatitis
In people with suspected (or under investigation for) chronic pancreatitis, whose diagnosis has not been confirmed by the use of 'firstline' tests (for example, CT scan, ultrasound scan, upper gastrointestinal  endoscopy or combinations of these), what is the accuracy of magnetic resonance cholangiopancreatography (MRCP) with or without secretin and endoscopic ultrasound to identify whether chronic pancreatitis is present?

## Why this is important
People with chronic pancreatitis usually present with chronic abdominal pain. However, there are many other causes of chronic abdominal pain (for example, peptic ulcer disease, gallstone disease, gastric cancer, pancreatic cancer and abdominal aortic aneurysm). First-line tests to exclude these other causes include abdominal ultrasound, upper GI endoscopy and abdominal CT scan. Where the diagnosis has still not been confirmed following these first-line tests, it is important to have a clinical algorithm of specialist tests to be able to identify people with chronic pancreatitis. Appropriate management options can then be offered. A diagnostic cohort study is needed to determine the accuracy of MRCP with or without secretin and endoscopic ultrasound in diagnosing chronic pancreatitis.

# Speed of intravenous fluid resuscitation for people with acute pancreatitis
What is the most clinically effective and cost-effective speed of administration of intravenous fluid for resuscitation in people with acute pancreatitis?

## Why this is important
There is clinical uncertainty about the optimal rate of fluid for resuscitation in severe acute pancreatitis. Severe acute pancreatitis causes the depletion of body fluids and reduction of the intravascular volume severe enough to cause hypotension, acute renal failure and pancreatic hypoperfusion, aggravating the damage to the pancreas. In addition, there is conflicting evidence about the effect of aggressive or conservative fluid management on outcomes in other conditions with a pathophysiology.
Current guidelines recommend using goal-directed therapy for fluid management, but do not recommend a particular type of fluid. A randomised controlled trial is needed to determine whether aggressive rates of intravenous fluid administration for the initial period of fluid resuscitation are more clinically or cost effective than conservative rates in people with acute pancreatitis.

# Pain management: chronic pancreatitis
Is the long-term use of opioids more clinically effective and cost effective than non-opioid analgesia (including non-pharmacological analgesia) in people with chronic pain due to chronic pancreatitis?

## Why this is important
Chronic pancreatitis is a complex condition needing biopsychosocial management. The pain is varied in nature, intensity, duration and severity, along with acute exacerbations. It is also multifactorial, making it difficult to have a standard regimen that can work for everyone. Some people also develop psychosocial factors such as reduction in quality of life, relationship issues, addiction to painkillers and financial difficulties.
Chronic pancreatitis is usually managed pharmacologically with a combination of opioids and other interventions. However, the use of opioids in managing chronic pancreatitis is known to cause serious side-effects  including tolerance, addiction, tiredness and constipation. These side-effects are frequently worse than the disease itself. Therefore, the whole rationale for the use of opioids in chronic pancreatitis is questionable. A cohort study is needed to determine how effective long-term opioid use is in this population compared with non-opiate pain management strategies, including analgesia and psychological therapies.

# Pain management: small duct disease
What is the most clinically effective and cost-effective intervention for managing small duct disease (in the absence of pancreatic duct obstruction, inflammatory mass or pseudocyst) in people with chronic pancreatitis presenting with pain?

## Why this is important
Chronic pancreatitis with small duct disease is more difficult to treat than without the disease because there is no anatomically correctable pancreatic abnormality  for example, pancreatic duct obstruction, inflammatory mass or pseudocysts. A randomised controlled trial study is needed to determine what the most effective intervention is for treating small duct disease. The following interventions should be compared with each other and with no treatment: surgery (partial resection, total resection with or without islet transplant, or drainage), endoscopic treatment, or standard care (for example, pharmacological treatment only, enzyme replacement therapy, nerve blocks).

# Management of type 3c diabetes
What is the most clinically effective and cost-effective insulin regimen to minimise hypo- and hyperglycaemia for type 3c diabetes secondary to pancreatitis?

## Why this is important
Type 3c diabetes is associated with metabolic instability and risk of decompensation leading to severe hypoglycaemia and ketoacidosis, in addition to poor quality of life. However, there is no evidence available in this population to inform practice. Therefore, research specifically on type 3c diabetes is essential to inform future updates of key recommendations in this guideline. National adoption of evidence-based insulin management in type 3c diabetes has the potential to cost effectively improve health and wellbeing, reducing the incidence of acute and long-term complications of poorly controlled glucose levels in chronic pancreatitis. A randomised controlled trial is needed to determine the most effective insulin therapy regimen in this population, comparing twice daily insulin injections, an insulin analogue multiple daily dose basal bolus regimen, and insulin pump therapy.