Chronic pancreatitis overview: Difference between revisions
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==Overview== | ==Overview== | ||
Chronic [[pancreatitis]] is a long-standing [[inflammatory]] disease of the [[pancreas]] characterized by irreversible changes | Chronic [[pancreatitis]] is a long-standing [[inflammatory]] disease of the [[pancreas]] characterized by irreversible changes in [[pancreatic]] structure and function leading to [[inflammation]] and [[fibrosis]]. The concept of [[pancreas]] and [[pancreatic duct]] was first described by Johannes Wirsung of Padua in 1642. The classification systems that have been used up til now for [[chronic pancreatitis]] include; Marseille, Marseille-Rome, Cambridge, TIGAR-O, ABC grading system, and Manchester system. The pathogenesis is still unclear and multifactorial but decreased [[bicarbonate]] secretion and intrapancreatic activation of digestive enzymes is thought to play an important role in the pathogenesis of chronic pancreatitis. Other factors that are thought to play an important role in the pathogenesis of chronic pancreatitis may include intraductal plugging and obstruction, direct injury to [[pancreatic]] cells induced by [[toxins]] and toxic metabolites, [[antioxidants]], [[ischemia]], [[autoimmune disorders]], the sentinal acute pancreatitis event (SAPE) hypothesis, [[necrosis]] and [[Fibrosis|fibrosis.]] [[Genes]] involved in the pathogenesis of chronic pancreatitis include [[CFTR|CFTR-cystic fibrosis gene mutation]], SPINK-1, which encodes for [[trypsin inhibitor]], PRSS-1 gene linked to [[hereditary pancreatitis]], claudin-2 (CLDN2), and carboxypeptidase A1 (CPA1) genes. TIGAR-O may be used for the etiologic classification of chronic pancreatitis. Chronic pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, [[diarrhea]], and [[weight loss]]. The [[incidence]] of chronic pancreatitis and the number of hospital admissions secondary to chronic pancreatitis is increasing in most countries worldwide. Alcoholic pancreatitis is more prevalent in developed or industrialized countries. [[Idiopathic]] and tropical pancreatitis is more prevalent in developing and underdeveloped countries. The M-ANNHEIM multiple risk factor classification of chronic pancreatitis includes multiple risk factors such as alcohol consumption, nicotine consumption, nutritional factors, hereditary factors, [[Efferent ducts|efferent duct]] factors, [[immunological]] factors, rare [[metabolic]] factors, and miscellaneous factors. The risk of progression of chronic pancreatitis to [[pancreatic cancer]] is approximately 4% at 20 years. Common complications of chronic pancreatitis usually include [[pseudocyst]] formation and mechanical obstruction of the [[duodenum]] and [[common bile duct]]. The diagnostic study of choice for chronic pancreatitis is [[magnetic resonance cholangiopancreatography]] (MRCP). The diagnosis is difficult to establish as the laboratory results and the imaging may be normal. Patients with chronic pancreatitis usually present with persistent [[abdominal pain]] with episodic flares that may or may not be associated with food intake, [[steatorrhea]], pancreatic [[diabetes]], [[nausea]] and [[weight loss]]. A [[secretin]] stimulation test is considered the gold standard functional test for diagnosis of chronic pancreatitis. Fecal tests may include sudan staining of feces, 72-hour quantitative fecal fat (gold standard), and faecal elastase measurement (test of choice). CT scan findings suggestive of chronic pancreatitis may include dilatation of the main pancreatic duct, [[Calcification|calcifications]], pancreatic gland enlargement, changes in pancreatic size, shape, and contour, and pancreatic [[Pancreatic pseudocyst|pseudocysts]]. [[MRI]] findings may include [[parenchymal]] [[atrophy]] or enlargement, [[pseudocyst]] formation, dilatation and beading of the [[pancreatic duct]] with intraductal [[Calcification|calcifications]] giving an appearance of 'chain of lakes'. [[Endoscopic retrograde cholangiopancreatography|ERCP]] findings diagnostic of chronic pancreatitis may include beaded appearance of the main [[pancreatic duct]] and ectatic side branches from the main pancreatic duct. Presence of stones is the most predictive finding seen on [[Endoscopic ultrasound|endoscopic ultrasonography]]. Other diagnostic studies may include [[genetic testing]] such as [[CFTR (gene)|CFTR-Cystic fibrosis gene mutation]], SPINK-1, which encodes for trypsin inhibitor and PRSS-1 gene linked to [[hereditary pancreatitis]]. Medical therapy includes pancreatic enzyme supplementation, [[analgesics]] and [[antioxidants]]. Specialized approaches include celiac nerve block, [[endoscopic]] therapy, extracorporeal shock wave [[lithotripsy]] (ESWL), and [[radiation]]. [[Steatorrhea]] can be managed by dietary modification, [[lipase]] supplementation, [[vitamin]] supplementation, and [[medium chain triglycerides]] (MCTs). Surgery for chronic pancreatitis tends to be divided into two resectional and drainage procedures. Dilated [[pancreatic duct]] may be managed with lateral [[pancreaticojejunostomy]] (LPJ) and lateral [[pancreaticojejunostomy]] with localized pancreatic head resection. Nondilated [[pancreatic duct]] is usually manages with [[pancreaticoduodenectomy]], duodenal-preserving pancreatic head resection (DPPHR), distal [[pancreatectomy]] (DP) and [[total pancreatectomy]] (TP). | ||
==Historical Perspective== | ==Historical Perspective== | ||
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==Pathophysiology== | ==Pathophysiology== | ||
Chronic pancreatitis is a progressive [[inflammatory]] process leading to irreversible structural damage to pancreas resulting in [[exocrine]] and [[endocrine]] dysfunction. The pathogenesis is still unclear and multifactorial but decreased [[bicarbonate]] secretion and intrapancreatic activation of [[digestive enzymes]] is thought to play an important role in the pathogenesis of chronic pancreatitis. Other factors that are thought to play an important role in the pathogenesis of chronic pancreatitis may include intraductal plugging and obstruction, direct injury to pancreatic cells induced by toxins and toxic metabolites, [[antioxidants]], [[ischemia]], [[autoimmune disorders]], the sentinal acute pancreatitis event (SAPE) hypothesis, [[necrosis]] and [[fibrosis]]. [[Genes]] involved in the pathogenesis of chronic pancreatitis include [[Cystic fibrosis transmembrane conductance regulator|CFTR-]]<nowiki/>cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor, PRSS-1 gene linked to [[hereditary pancreatitis]], claudin-2 (CLDN2), and carboxypeptidase A1 (CPA1) genes. | Chronic pancreatitis is a progressive [[inflammatory]] process leading to irreversible structural damage to pancreas resulting in [[exocrine]] and [[endocrine]] dysfunction. The pathogenesis is still unclear and multifactorial but decreased [[bicarbonate]] secretion and intrapancreatic activation of [[digestive enzymes]] is thought to play an important role in the pathogenesis of chronic pancreatitis. Other factors that are thought to play an important role in the pathogenesis of chronic pancreatitis may include intraductal plugging and obstruction, direct injury to pancreatic cells induced by toxins and toxic metabolites, [[antioxidants]], [[ischemia]], [[autoimmune disorders]], the sentinal acute pancreatitis event (SAPE) hypothesis, [[necrosis]] and [[fibrosis]]. [[Genes]] involved in the pathogenesis of chronic pancreatitis include [[Cystic fibrosis transmembrane conductance regulator|CFTR-]]<nowiki/>cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor, PRSS-1 gene linked to [[hereditary pancreatitis]], claudin-2 (CLDN2), and [[Carboxypeptidase A|carboxypeptidase A1]] (CPA1) genes. Associated conditions may include [[autoimmune]] conditions, [[primary biliary cirrhosis]], [[primary sclerosing cholangitis]], [[Sjögren's syndrome|sjögren syndrome]], and [[renal tubular acidosis]]. Gross pathology may include enlarged or [[atrophic]] pancreas, [[cysts]], [[Calcification|calcifications]], [[fibrosis]] and patchy focal [[necrosis]]. Microscopic histopathological analysis may show patchy focal disease characterized by a [[Mononuclear cells|mononuclear]] infiltrate. | ||
==Causes== | ==Causes== | ||
TIGAR-O may be used for the etiologic classification of chronic pancreatitis. [[Toxic]] causes may include [[alcohol]], [[tobacco]] smoking, [[hypercalcemia]], [[hyperlipidemia]], [[chronic renal failure]], [[medications]], and [[toxins]]. [[Genetic]] causes may include cationic trypsinogen (''PRSS1''), cystic fibrosis transmembrane conductance regulator gene (''CFTR''), [[calcium-sensing receptor]] (''CASR''), chymotrypsin C gene (''CTRC'') and pancreatic secretory trypsin inhibitor gene (''SPINK1''). Less common causes may include [[hypertriglyceridemia]], [[scorpion sting]], tropical pancreatitis and medications such as [[metronidazole]], ercaptopurine, [[valproate]], [[isoniazid]] and [[corticosteroids]]. | TIGAR-O may be used for the etiologic classification of chronic pancreatitis. [[Toxic]] causes may include [[alcohol]], [[tobacco]] smoking, [[hypercalcemia]], [[hyperlipidemia]], [[chronic renal failure]], [[medications]], and [[toxins]]. [[Genetic]] causes of chronic pancreatitis may include cationic trypsinogen (''PRSS1''), cystic fibrosis transmembrane conductance regulator gene (''CFTR''), [[calcium-sensing receptor]] (''CASR''), [[chymotrypsin]] C gene (''CTRC'') and pancreatic secretory trypsin inhibitor gene (''SPINK1''). Less common causes may include [[hypertriglyceridemia]], [[scorpion sting]], tropical pancreatitis and medications such as [[metronidazole]], ercaptopurine, [[valproate]], [[isoniazid]] and [[corticosteroids]]. | ||
==Differentiating {{PAGENAME}} from Other Diseases== | ==Differentiating {{PAGENAME}} from Other Diseases== | ||
Chronic pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, [[diarrhea]] and [[weight loss]]. | Chronic pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as [[abdominal pain]], [[diarrhea]] and [[weight loss]]. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The incidence of chronic pancreatitis and the number of hospital admissions is increasing in most countries worldwide. The incidence of chronic pancreatitis is approximately 1.6 to 23 cases per 100,000 individuals per year worldwide. A rising level of per capita alcohol consumption is correlated with an increasing prevalence of chronic pancreatitis. Changes in lifestyle modifications such as smoking and alcohol cessation may result in slowing the progression and reducing the incidence of chronic pancreatitis. Alcoholic pancreatitis is more prevalent in developed or industrialized countries. Idiopathic and tropical pancreatitis is more prevalent in developing and underdeveloped countries. | The incidence of chronic pancreatitis and the number of hospital admissions is increasing in most countries worldwide. The incidence of chronic pancreatitis is approximately 1.6 to 23 cases per 100,000 individuals per year worldwide. A rising level of per capita alcohol consumption is correlated with an increasing prevalence of chronic pancreatitis. Changes in lifestyle modifications such as [[Smoking cessation|smoking]] and alcohol cessation may result in slowing the progression and reducing the incidence of chronic pancreatitis. Alcoholic pancreatitis is more prevalent in developed or industrialized countries. [[Idiopathic|Idiopathi]]<nowiki/>c and tropical pancreatitis is more prevalent in developing and underdeveloped countries. | ||
==Risk Factors== | ==Risk Factors== | ||
The M-ANNHEIM multiple risk factor classification of chronic pancreatitis includes multiple risk factors, alcohol consumption, nicotine consumption, nutritional factors, hereditary factors, efferent duct factors, immunological factors, miscellaneous and rare metabolic factors. Common risk factors may include alcohol abuse, cigarette smoking, genetic mutations (PRSS1 mutations, SPINK1 mutations, CFTR mutations, and CTRC mutations), obstructive causes and hypercalcemia. Less common risk factors may include sphincter of Oddi dysfunction, hyperlipidemia, metabolic diseases such as branched-chain organic aciduria and chronic renal failure. | The M-ANNHEIM multiple risk factor classification of chronic pancreatitis includes multiple risk factors, alcohol consumption, [[nicotine]] consumption, nutritional factors, [[hereditary]] factors, [[Efferent ducts|efferent duct]] factors, [[immunological]] factors, miscellaneous and rare [[metabolic]] factors. Common risk factors may include alcohol abuse, [[cigarette smoking]], [[genetic mutations]] (PRSS1 mutations, SPINK1 mutations, [[Cystic fibrosis transmembrane conductance regulator|CFTR]] mutations, and CTRC mutations), obstructive causes and [[hypercalcemia]]. Less common risk factors may include [[sphincter of Oddi dysfunction]], [[hyperlipidemia]], [[metabolic diseases]] such as branched-chain organic aciduria, and [[chronic renal failure]]. | ||
==Screening== | ==Screening== | ||
Patients with chronic pancreatitis should be screened for diabetes annually and should be followed up with fasting glucose and HbA1c levels. | Patients with chronic pancreatitis should be screened for [[diabetes]] annually and should be followed up with [[Fasting plasma glucose|fasting glucose]] and [[HbA1c]] levels. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
The natural history of pain is highly variable and mostly involves intermittent pain attacks that decrease or resolve over 5-25 years while a few patients may have chronic pain. The risk of progression of chronic pancreatitis to pancreatic cancer is approximately 4% at 20 years. Common complications usually include pseudocyst and | The natural history of pain is highly variable and mostly involves intermittent pain attacks that decrease or resolve over 5-25 years while a few patients may have chronic pain. The risk of progression of chronic pancreatitis to [[pancreatic cancer]] is approximately 4% at 20 years. Common complications usually include [[pseudocyst]] and mechanical obstruction of the [[duodenum]] and [[common bile duct]]. Less common complications may include pancreatic [[ascites]], [[pleural effusion]], [[pseudoaneurysm]] formation, and [[splenic vein thrombosis]]. Prognostic factors associated with chronic pancreatitis may include the age at diagnosis, smoking status, continued use of alcohol, and presence of liver [[cirrhosis]]. In chronic pancreatitis, the overall survival rate is 70% at 10 years and the 10-year mortality rate is 30%. | ||
==Diagnosis== | ==Diagnosis== | ||
===Diagnostic study of choice:=== | ===Diagnostic study of choice:=== | ||
The diagnostic study of choice for chronic pancreatitis is magnetic resonance cholangiopancreatography (MRCP). The diagnosis is difficult to establish as the laboratory results and the imaging may be normal. Diagnostic findings confirmatory for chronic pancreatitis may include pancreatic calcifications seen on abdominal plain films or CT scan, beaded appearance of main pancreatic duct seen on pancreatogram, abnormal pancreatogram showing | The diagnostic study of choice for chronic pancreatitis is [[Magnetic resonance cholangiopancreatography|magnetic resonance cholangiopancreatography (MRCP)]]. The diagnosis is difficult to establish as the laboratory results and the imaging may be normal. Diagnostic findings confirmatory for chronic pancreatitis may include pancreatic [[Calcification|calcifications]] seen on abdominal plain films or [[CT scan]], beaded appearance of main [[pancreatic duct]] seen on pancreatogram, abnormal pancreatogram showing ecstatic side branches from the main [[pancreatic duct]] and abnormal pancreatic function tests (Secretin test). | ||
===History and Symptoms=== | === Diagnostic criteria: === | ||
Patients with chronic pancreatitis usually present with persistent [[abdominal pain]] with episodic flares that may or may not be associated with food intake [[steatorrhea]], pancreatic diabetes, [[nausea]] and [[weight loss]]. According to M-ANNHEIM clinical staging of chronic pancreatitis, it can be classified into asymptomatic and symptomatic chronic pancreatitis. M-ANNHEIM scoring system for the grading of clinical features of chronic pancreatitis include features such as pain control, surgical intervention, exocrine insufficiency, endocrine insufficiency, morphologic status on pancreatic imaging and severe organ complications. | The M-ANNHEIM diagnostic criteria for chronic pancreatitis can be used to identify and differentiate various forms of chronic pancreatitis. M-ANNHEIM pancreatic imaging criteria for US, CT, MRI/MRCP, and EUS based on imaging features as defined by the Cambridge classification includes normal, equivocal, mild, moderate and marked changes. | ||
===History and Symptoms:=== | |||
Patients with chronic pancreatitis usually present with persistent [[abdominal pain]] with episodic flares that may or may not be associated with food intake [[steatorrhea]], pancreatic diabetes, [[nausea]], and [[weight loss]]. According to M-ANNHEIM clinical staging of chronic pancreatitis, it can be classified into [[asymptomatic]] and [[symptomatic]] chronic pancreatitis. M-ANNHEIM scoring system for the grading of clinical features of chronic pancreatitis include features such as pain control, surgical intervention, [[exocrine]] insufficiency, [[endocrine]] insufficiency, morphologic status on [[pancreatic]] imaging and severe organ complications. | |||
===Physical Examination:=== | ===Physical Examination:=== | ||
Patients with chronic pancreatitis may assume a characteristic position in an attempt to relieve their abdominal pain such as lying on the left side, flexing the spine, drawing the knees up toward the chest. Patients with steatorrhea or advanced disease may present as loss of subcutaneous fat, temporal wasting and sunken supraclavicular fossa. Skin findings may include [[ | Patients with chronic pancreatitis may assume a characteristic position in an attempt to relieve their abdominal pain such as lying on the left side, flexing the [[spine]], drawing the knees up toward the chest. Patients with [[steatorrhea]] or advanced disease may present as loss of [[subcutaneous fat]], temporal wasting and sunken [[supraclavicular fossa]]. Skin findings may include [[jaundice]], [[pallor]], and [[bruises]]. | ||
===Laboratory Findings:=== | ===Laboratory Findings:=== | ||
The diagnosis of chronic pancreatitis is typically based on tests on pancreatic structure and function. Serum [[amylase]] and [[lipase]] are usually normal but may be slightly elevated. Serum calcium and triglyceride levels may be elevated in hypertriglyceridemia induced pancreatitis. A [[secretin]] stimulation test is considered the gold standard functional test for diagnosis of chronic pancreatitis. Fecal tests may include sudan staining of feces, 72-hour quantitative fecal fat ( | The diagnosis of chronic pancreatitis is typically based on tests on [[pancreatic]] structure and function. Serum [[amylase]] and [[lipase]] are usually normal but may be slightly elevated. Serum [[calcium]] and [[triglyceride]] levels may be elevated in [[hypertriglyceridemia]] induced pancreatitis. A [[secretin]] stimulation test is considered the gold standard functional test for diagnosis of chronic pancreatitis. Fecal tests may include sudan staining of feces, 72-hour quantitative fecal fat (gold standard), and faecal elastase measurement (test of choice). | ||
=== Abdominal X-ray: === | === Abdominal X-ray: === | ||
An x-ray may be helpful in the diagnosis of chronic pancreatitis. Finding on an x-ray suggestive of chronic pancreatitis includes [[pancreatic]] [[calcification]]. | |||
=== CT scan: === | === CT scan: === | ||
[[CT scan]] findings suggestive of chronic pancreatitis may include dilatation of the main [[pancreatic duct]], [[Calcification|calcifications]], pancreatic gland enlargement, changes in pancreatic size, shape, contour, and [[pancreatic pseudocyst|pancreatic pseudocysts]]. Contrast enhanced [[CT scan]] may be helpful in ruling out other diseases with similar presentation such as [[malignancy]] and [[Pseudocyst|pseudocysts]]. The sensitivity of CT scan is 75 to 90 percent and specificity is 85 percent. | |||
=== MRI: === | === MRI: === | ||
MRI findings for chronic pancreatitis may be classified as early and late findings. Early findings may include low-signal-intensity pancreas on T1-weighted fat-suppressed images, decreased and delayed enhancement after IV contrast administration and dilated side branches. Late findings may include parenchymal atrophy or enlargement, pseudocyst formation, dilatation and beading of the pancreatic duct with intraductal calcifications giving an appearance of 'chain of lakes'. | MRI findings for chronic pancreatitis may be classified as early and late findings. Early findings may include low-signal-intensity [[pancreas]] on T1-weighted fat-suppressed images, decreased and delayed enhancement after IV contrast administration and dilated side branches. Late findings may include [[parenchymal]] [[atrophy]] or enlargement, [[pseudocyst]] formation, dilatation and beading of the pancreatic duct with intraductal [[Calcification|calcifications]] giving an appearance of 'chain of lakes'. | ||
=== Ultrasound: === | === Ultrasound: === | ||
Ultrasound findings suggestive of chronic pancreatitis may include dilatation of the main pancreatic duct, calcifications, pancreatic gland enlargement, changes in pancreatic size, shape, and contour, pancreatic pseudocysts, hyperechogenicity suggesting fibrotic changes, pseudoaneurysms and ascites. The sensitivity of ultrasound is 60 to 70 percent and the specificity of ultrasound is 80 to 90 percent. The pancreas might appear atrophic, calcified or fibrotic (advanced stages). | Ultrasound findings suggestive of chronic pancreatitis may include dilatation of the main [[pancreatic duct]], [[Calcification|calcifications]], pancreatic gland enlargement, changes in pancreatic size, shape, and contour, [[Pancreatic pseudocyst|pancreatic pseudocysts]], hyperechogenicity suggesting fibrotic changes, [[Pseudoaneurysm|pseudoaneurysms]] and [[ascites]]. The sensitivity of [[ultrasound]] is 60 to 70 percent and the specificity of ultrasound is 80 to 90 percent. The pancreas might appear [[atrophic]], calcified or fibrotic (advanced stages). | ||
=== Other imaging findings: === | === Other imaging findings: === | ||
Other imaging findings may include [[MRCP]], [[ERCP]], and endoscopic ultrasonography. MRCP is the diagnostic study of choice. MRCP findings suggestive of chronic pancreatitis may include calcifications and pancreatic duct obstruction. ERCP findings diagnostic of chronic pancreatitis may include beaded appearance of the main pancreatic duct and ectatic side branches from the main pancreatic duct. Presence of stones is the most predictive finding seen on endoscopic ultrasonography. Other findings suggestive of chronic pancreatitis may include visible side branches, cysts, lobularity, an irregular main pancreatic duct, hyperechoic foci, dilation of the main pancreatic duct and hyperechoic margins of the main pancreatic duct. Pancreatic function tests may include direct tests such as secretin stimulation tests and indirect tests. | Other imaging findings may include [[MRCP]], [[ERCP]], and [[endoscopic]] [[ultrasonography]]. [[Magnetic resonance cholangiopancreatography|MRCP]] is the diagnostic study of choice. [[Magnetic resonance cholangiopancreatography|MRCP]] findings suggestive of chronic pancreatitis may include calcifications and [[pancreatic duct]] obstruction. [[Endoscopic retrograde cholangiopancreatography|ERCP]] findings diagnostic of chronic pancreatitis may include beaded appearance of the main [[pancreatic duct]] and ectatic side branches from the main [[pancreatic duct]]. Presence of stones is the most predictive finding seen on [[endoscopic]] [[ultrasonography]]. Other findings suggestive of chronic pancreatitis may include visible side branches, [[cysts]], lobularity, an irregular main [[pancreatic duct]], hyperechoic foci, dilation of the main [[pancreatic duct]] and hyperechoic margins of the main [[pancreatic duct]]. Pancreatic function tests may include direct tests such as secretin stimulation tests and indirect tests. | ||
===Other diagnostic studies:=== | ===Other diagnostic studies:=== | ||
Other diagnostic studies may include genetic testing such as CFTR- | Other diagnostic studies may include [[genetic testing]] such as [[Cystic fibrosis transmembrane conductance regulator|CFTR-cystic fibrosis gene mutation]], SPINK-1, which encodes for [[trypsin inhibitor]] and PRSS-1 gene linked to [[hereditary pancreatitis]]. Routine [[genetic testing]] is usually not recommended for [[Cystic fibrosis transmembrane conductance regulator|CFTR]] and SPINK-1 genes as it is expensive, do not alter the management and false positive results. | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
The goals of chronic pancreatitis management include pain control, management of pancreatic insufficiency by pancreatic enzyme replacement and management of complications. Pain is managed in a stepwise approach of general recommendations, pancreatic enzyme replacement, analgesics and invasive procedures. General recommendations usually include smoking cessation, cessation of alcohol intake, small meals and hydration. Medical therapy includes pancreatic enzyme supplementation, analgesics and antioxidants. Specialized approaches include celiac nerve block, endoscopic therapy, extracorporeal shock wave lithotripsy (ESWL), and radiation. Steatorrhea can be managed by dietary modification, lipase supplementation, vitamin supplementation, and medium chain triglycerides (MCTs). Diabetes is usually managed with a trial of oral hypoglycemic agents followed by insulin therapy. | The goals of chronic pancreatitis management include [[pain]] control, management of [[pancreatic insufficiency]] by pancreatic [[Enzyme replacement therapy|enzyme replacement]] and management of [[complications]]. Pain is managed in a stepwise approach of general recommendations, pancreatic [[Enzyme replacement therapy|enzyme replacement]], [[analgesics]] and invasive procedures. General recommendations usually include smoking cessation, cessation of alcohol intake, small meals and [[hydration]]. Medical therapy includes pancreatic enzyme supplementation, [[analgesics]] and [[antioxidants]]. Specialized approaches include celiac nerve block, [[endoscopic]] therapy, extracorporeal shock wave lithotripsy (ESWL), and radiation. [[Steatorrhea]] can be managed by dietary modification, [[lipase]] supplementation, [[vitamin]] supplementation, and [[medium chain triglycerides]] (MCTs). [[Diabetes]] is usually managed with a trial of oral [[Hypoglycemic agent|hypoglycemic agents]] followed by [[Insulin|insulin therapy]]. | ||
===Surgery=== | ===Surgery=== | ||
Surgery is usually considered when pain management fails with medical and endoscopic therapies. The goals of surgery include effective pain relief, and to preserve long-term pancreatic function. Surgery for chronic pancreatitis tends to be divided into two areas - resectional and drainage procedures. Dilated pancreatic duct may be managed with lateral pancreaticojejunostomy (LPJ) and lateral pancreaticojejunostomy with localized pancreatic head resection. Nondilated pancreatic duct is usually | Surgery is usually considered when pain management fails with medical and [[endoscopic]] therapies. The goals of surgery include effective pain relief, and to preserve long-term pancreatic function. Surgery for chronic pancreatitis tends to be divided into two areas - resectional and drainage procedures. Dilated [[pancreatic duct]] may be managed with [[lateral]] pancreaticojejunostomy (LPJ) and [[lateral]]<nowiki/>pancreaticojejunostomy with localized pancreatic head resection. Nondilated [[pancreatic duct]] is usually managed with [[pancreaticoduodenectomy]], duodenal-preserving pancreatic head resection (DPPHR), distal [[pancreatectomy]] (DP) and total [[pancreatectomy]] (TP). | ||
===Primary Prevention=== | |||
===Prevention=== | === Secondary Prevention === | ||
Effective measures for the secondary prevention of Hereditary pancreatitis include low-fat diet, multiple small meals, good hydration, antioxidants and cessation/abstinence from smoking and alcohol use. Patients should be managed appropriately and timely with [[glucocorticoids]] and [[immunomodulatory]] drugs to prevent complications. | |||
==References== | ==References== |
Latest revision as of 21:53, 3 February 2018
https://https://www.youtube.com/watch?v=met9SntRZe8%7C350}} |
Chronic pancreatitis Microchapters |
Diagnosis |
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Treatment |
Case Studies |
American Pancreatic Association Practice Guidelines |
Chronic pancreatitis overview On the Web |
American Roentgen Ray Society Images of Chronic pancreatitis overview |
Risk calculators and risk factors for Chronic pancreatitis overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]
Overview
Chronic pancreatitis is a long-standing inflammatory disease of the pancreas characterized by irreversible changes in pancreatic structure and function leading to inflammation and fibrosis. The concept of pancreas and pancreatic duct was first described by Johannes Wirsung of Padua in 1642. The classification systems that have been used up til now for chronic pancreatitis include; Marseille, Marseille-Rome, Cambridge, TIGAR-O, ABC grading system, and Manchester system. The pathogenesis is still unclear and multifactorial but decreased bicarbonate secretion and intrapancreatic activation of digestive enzymes is thought to play an important role in the pathogenesis of chronic pancreatitis. Other factors that are thought to play an important role in the pathogenesis of chronic pancreatitis may include intraductal plugging and obstruction, direct injury to pancreatic cells induced by toxins and toxic metabolites, antioxidants, ischemia, autoimmune disorders, the sentinal acute pancreatitis event (SAPE) hypothesis, necrosis and fibrosis. Genes involved in the pathogenesis of chronic pancreatitis include CFTR-cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor, PRSS-1 gene linked to hereditary pancreatitis, claudin-2 (CLDN2), and carboxypeptidase A1 (CPA1) genes. TIGAR-O may be used for the etiologic classification of chronic pancreatitis. Chronic pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, diarrhea, and weight loss. The incidence of chronic pancreatitis and the number of hospital admissions secondary to chronic pancreatitis is increasing in most countries worldwide. Alcoholic pancreatitis is more prevalent in developed or industrialized countries. Idiopathic and tropical pancreatitis is more prevalent in developing and underdeveloped countries. The M-ANNHEIM multiple risk factor classification of chronic pancreatitis includes multiple risk factors such as alcohol consumption, nicotine consumption, nutritional factors, hereditary factors, efferent duct factors, immunological factors, rare metabolic factors, and miscellaneous factors. The risk of progression of chronic pancreatitis to pancreatic cancer is approximately 4% at 20 years. Common complications of chronic pancreatitis usually include pseudocyst formation and mechanical obstruction of the duodenum and common bile duct. The diagnostic study of choice for chronic pancreatitis is magnetic resonance cholangiopancreatography (MRCP). The diagnosis is difficult to establish as the laboratory results and the imaging may be normal. Patients with chronic pancreatitis usually present with persistent abdominal pain with episodic flares that may or may not be associated with food intake, steatorrhea, pancreatic diabetes, nausea and weight loss. A secretin stimulation test is considered the gold standard functional test for diagnosis of chronic pancreatitis. Fecal tests may include sudan staining of feces, 72-hour quantitative fecal fat (gold standard), and faecal elastase measurement (test of choice). CT scan findings suggestive of chronic pancreatitis may include dilatation of the main pancreatic duct, calcifications, pancreatic gland enlargement, changes in pancreatic size, shape, and contour, and pancreatic pseudocysts. MRI findings may include parenchymal atrophy or enlargement, pseudocyst formation, dilatation and beading of the pancreatic duct with intraductal calcifications giving an appearance of 'chain of lakes'. ERCP findings diagnostic of chronic pancreatitis may include beaded appearance of the main pancreatic duct and ectatic side branches from the main pancreatic duct. Presence of stones is the most predictive finding seen on endoscopic ultrasonography. Other diagnostic studies may include genetic testing such as CFTR-Cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor and PRSS-1 gene linked to hereditary pancreatitis. Medical therapy includes pancreatic enzyme supplementation, analgesics and antioxidants. Specialized approaches include celiac nerve block, endoscopic therapy, extracorporeal shock wave lithotripsy (ESWL), and radiation. Steatorrhea can be managed by dietary modification, lipase supplementation, vitamin supplementation, and medium chain triglycerides (MCTs). Surgery for chronic pancreatitis tends to be divided into two resectional and drainage procedures. Dilated pancreatic duct may be managed with lateral pancreaticojejunostomy (LPJ) and lateral pancreaticojejunostomy with localized pancreatic head resection. Nondilated pancreatic duct is usually manages with pancreaticoduodenectomy, duodenal-preserving pancreatic head resection (DPPHR), distal pancreatectomy (DP) and total pancreatectomy (TP).
Historical Perspective
The concept of pancreas and pancreatic duct was first described by Johannes Wirsung of Padua in 1642. In 1761, Giovanni Morgagni described the clinical syndrome of severe upper abdominal pain, vomiting, and collapse. He is also credited with the earliest pathological recognition of cancer of the pancreas. In 1948, Eliason and Welty described distal pancreatectomy (DP). In 1980, Beger described duodenal-preserving pancreatic head resection (DPPHR) technique for chronic pancreatitis to decrease the morbidity of pancreatic head resection.
Classification
Chronic pancreatitis may be divided based upon underlying morphology into large-duct type or small-duct type with or without calcification. The classification systems that have been used up til now for chronic pancreatitis may include ; Marseille, Marseille-Rome, Cambridge, TIGAR-O, ABC grading system and Manchester system.
Pathophysiology
Chronic pancreatitis is a progressive inflammatory process leading to irreversible structural damage to pancreas resulting in exocrine and endocrine dysfunction. The pathogenesis is still unclear and multifactorial but decreased bicarbonate secretion and intrapancreatic activation of digestive enzymes is thought to play an important role in the pathogenesis of chronic pancreatitis. Other factors that are thought to play an important role in the pathogenesis of chronic pancreatitis may include intraductal plugging and obstruction, direct injury to pancreatic cells induced by toxins and toxic metabolites, antioxidants, ischemia, autoimmune disorders, the sentinal acute pancreatitis event (SAPE) hypothesis, necrosis and fibrosis. Genes involved in the pathogenesis of chronic pancreatitis include CFTR-cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor, PRSS-1 gene linked to hereditary pancreatitis, claudin-2 (CLDN2), and carboxypeptidase A1 (CPA1) genes. Associated conditions may include autoimmune conditions, primary biliary cirrhosis, primary sclerosing cholangitis, sjögren syndrome, and renal tubular acidosis. Gross pathology may include enlarged or atrophic pancreas, cysts, calcifications, fibrosis and patchy focal necrosis. Microscopic histopathological analysis may show patchy focal disease characterized by a mononuclear infiltrate.
Causes
TIGAR-O may be used for the etiologic classification of chronic pancreatitis. Toxic causes may include alcohol, tobacco smoking, hypercalcemia, hyperlipidemia, chronic renal failure, medications, and toxins. Genetic causes of chronic pancreatitis may include cationic trypsinogen (PRSS1), cystic fibrosis transmembrane conductance regulator gene (CFTR), calcium-sensing receptor (CASR), chymotrypsin C gene (CTRC) and pancreatic secretory trypsin inhibitor gene (SPINK1). Less common causes may include hypertriglyceridemia, scorpion sting, tropical pancreatitis and medications such as metronidazole, ercaptopurine, valproate, isoniazid and corticosteroids.
Differentiating Chronic pancreatitis overview from Other Diseases
Chronic pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, diarrhea and weight loss.
Epidemiology and Demographics
The incidence of chronic pancreatitis and the number of hospital admissions is increasing in most countries worldwide. The incidence of chronic pancreatitis is approximately 1.6 to 23 cases per 100,000 individuals per year worldwide. A rising level of per capita alcohol consumption is correlated with an increasing prevalence of chronic pancreatitis. Changes in lifestyle modifications such as smoking and alcohol cessation may result in slowing the progression and reducing the incidence of chronic pancreatitis. Alcoholic pancreatitis is more prevalent in developed or industrialized countries. Idiopathic and tropical pancreatitis is more prevalent in developing and underdeveloped countries.
Risk Factors
The M-ANNHEIM multiple risk factor classification of chronic pancreatitis includes multiple risk factors, alcohol consumption, nicotine consumption, nutritional factors, hereditary factors, efferent duct factors, immunological factors, miscellaneous and rare metabolic factors. Common risk factors may include alcohol abuse, cigarette smoking, genetic mutations (PRSS1 mutations, SPINK1 mutations, CFTR mutations, and CTRC mutations), obstructive causes and hypercalcemia. Less common risk factors may include sphincter of Oddi dysfunction, hyperlipidemia, metabolic diseases such as branched-chain organic aciduria, and chronic renal failure.
Screening
Patients with chronic pancreatitis should be screened for diabetes annually and should be followed up with fasting glucose and HbA1c levels.
Natural History, Complications, and Prognosis
The natural history of pain is highly variable and mostly involves intermittent pain attacks that decrease or resolve over 5-25 years while a few patients may have chronic pain. The risk of progression of chronic pancreatitis to pancreatic cancer is approximately 4% at 20 years. Common complications usually include pseudocyst and mechanical obstruction of the duodenum and common bile duct. Less common complications may include pancreatic ascites, pleural effusion, pseudoaneurysm formation, and splenic vein thrombosis. Prognostic factors associated with chronic pancreatitis may include the age at diagnosis, smoking status, continued use of alcohol, and presence of liver cirrhosis. In chronic pancreatitis, the overall survival rate is 70% at 10 years and the 10-year mortality rate is 30%.
Diagnosis
Diagnostic study of choice:
The diagnostic study of choice for chronic pancreatitis is magnetic resonance cholangiopancreatography (MRCP). The diagnosis is difficult to establish as the laboratory results and the imaging may be normal. Diagnostic findings confirmatory for chronic pancreatitis may include pancreatic calcifications seen on abdominal plain films or CT scan, beaded appearance of main pancreatic duct seen on pancreatogram, abnormal pancreatogram showing ecstatic side branches from the main pancreatic duct and abnormal pancreatic function tests (Secretin test).
Diagnostic criteria:
The M-ANNHEIM diagnostic criteria for chronic pancreatitis can be used to identify and differentiate various forms of chronic pancreatitis. M-ANNHEIM pancreatic imaging criteria for US, CT, MRI/MRCP, and EUS based on imaging features as defined by the Cambridge classification includes normal, equivocal, mild, moderate and marked changes.
History and Symptoms:
Patients with chronic pancreatitis usually present with persistent abdominal pain with episodic flares that may or may not be associated with food intake steatorrhea, pancreatic diabetes, nausea, and weight loss. According to M-ANNHEIM clinical staging of chronic pancreatitis, it can be classified into asymptomatic and symptomatic chronic pancreatitis. M-ANNHEIM scoring system for the grading of clinical features of chronic pancreatitis include features such as pain control, surgical intervention, exocrine insufficiency, endocrine insufficiency, morphologic status on pancreatic imaging and severe organ complications.
Physical Examination:
Patients with chronic pancreatitis may assume a characteristic position in an attempt to relieve their abdominal pain such as lying on the left side, flexing the spine, drawing the knees up toward the chest. Patients with steatorrhea or advanced disease may present as loss of subcutaneous fat, temporal wasting and sunken supraclavicular fossa. Skin findings may include jaundice, pallor, and bruises.
Laboratory Findings:
The diagnosis of chronic pancreatitis is typically based on tests on pancreatic structure and function. Serum amylase and lipase are usually normal but may be slightly elevated. Serum calcium and triglyceride levels may be elevated in hypertriglyceridemia induced pancreatitis. A secretin stimulation test is considered the gold standard functional test for diagnosis of chronic pancreatitis. Fecal tests may include sudan staining of feces, 72-hour quantitative fecal fat (gold standard), and faecal elastase measurement (test of choice).
Abdominal X-ray:
An x-ray may be helpful in the diagnosis of chronic pancreatitis. Finding on an x-ray suggestive of chronic pancreatitis includes pancreatic calcification.
CT scan:
CT scan findings suggestive of chronic pancreatitis may include dilatation of the main pancreatic duct, calcifications, pancreatic gland enlargement, changes in pancreatic size, shape, contour, and pancreatic pseudocysts. Contrast enhanced CT scan may be helpful in ruling out other diseases with similar presentation such as malignancy and pseudocysts. The sensitivity of CT scan is 75 to 90 percent and specificity is 85 percent.
MRI:
MRI findings for chronic pancreatitis may be classified as early and late findings. Early findings may include low-signal-intensity pancreas on T1-weighted fat-suppressed images, decreased and delayed enhancement after IV contrast administration and dilated side branches. Late findings may include parenchymal atrophy or enlargement, pseudocyst formation, dilatation and beading of the pancreatic duct with intraductal calcifications giving an appearance of 'chain of lakes'.
Ultrasound:
Ultrasound findings suggestive of chronic pancreatitis may include dilatation of the main pancreatic duct, calcifications, pancreatic gland enlargement, changes in pancreatic size, shape, and contour, pancreatic pseudocysts, hyperechogenicity suggesting fibrotic changes, pseudoaneurysms and ascites. The sensitivity of ultrasound is 60 to 70 percent and the specificity of ultrasound is 80 to 90 percent. The pancreas might appear atrophic, calcified or fibrotic (advanced stages).
Other imaging findings:
Other imaging findings may include MRCP, ERCP, and endoscopic ultrasonography. MRCP is the diagnostic study of choice. MRCP findings suggestive of chronic pancreatitis may include calcifications and pancreatic duct obstruction. ERCP findings diagnostic of chronic pancreatitis may include beaded appearance of the main pancreatic duct and ectatic side branches from the main pancreatic duct. Presence of stones is the most predictive finding seen on endoscopic ultrasonography. Other findings suggestive of chronic pancreatitis may include visible side branches, cysts, lobularity, an irregular main pancreatic duct, hyperechoic foci, dilation of the main pancreatic duct and hyperechoic margins of the main pancreatic duct. Pancreatic function tests may include direct tests such as secretin stimulation tests and indirect tests.
Other diagnostic studies:
Other diagnostic studies may include genetic testing such as CFTR-cystic fibrosis gene mutation, SPINK-1, which encodes for trypsin inhibitor and PRSS-1 gene linked to hereditary pancreatitis. Routine genetic testing is usually not recommended for CFTR and SPINK-1 genes as it is expensive, do not alter the management and false positive results.
Treatment
Medical Therapy
The goals of chronic pancreatitis management include pain control, management of pancreatic insufficiency by pancreatic enzyme replacement and management of complications. Pain is managed in a stepwise approach of general recommendations, pancreatic enzyme replacement, analgesics and invasive procedures. General recommendations usually include smoking cessation, cessation of alcohol intake, small meals and hydration. Medical therapy includes pancreatic enzyme supplementation, analgesics and antioxidants. Specialized approaches include celiac nerve block, endoscopic therapy, extracorporeal shock wave lithotripsy (ESWL), and radiation. Steatorrhea can be managed by dietary modification, lipase supplementation, vitamin supplementation, and medium chain triglycerides (MCTs). Diabetes is usually managed with a trial of oral hypoglycemic agents followed by insulin therapy.
Surgery
Surgery is usually considered when pain management fails with medical and endoscopic therapies. The goals of surgery include effective pain relief, and to preserve long-term pancreatic function. Surgery for chronic pancreatitis tends to be divided into two areas - resectional and drainage procedures. Dilated pancreatic duct may be managed with lateral pancreaticojejunostomy (LPJ) and lateralpancreaticojejunostomy with localized pancreatic head resection. Nondilated pancreatic duct is usually managed with pancreaticoduodenectomy, duodenal-preserving pancreatic head resection (DPPHR), distal pancreatectomy (DP) and total pancreatectomy (TP).
Primary Prevention
Secondary Prevention
Effective measures for the secondary prevention of Hereditary pancreatitis include low-fat diet, multiple small meals, good hydration, antioxidants and cessation/abstinence from smoking and alcohol use. Patients should be managed appropriately and timely with glucocorticoids and immunomodulatory drugs to prevent complications.