Acute Relapsing Pancreatitis - Touch Gastroenterology

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Biliary or pancreatic SOD are divided into the following three types: 8,10,11 • Type 1 corresponds to patients with biliary- or pancreatic-type pain, elevation of liver function tests or pancreatic hydrolases, and dilation of the common bile duct (>12mm in diameter) or the main pancreatic duct (>5mm in diameter). Type 1 is considered to be due to a chronic inflammatory process (probably secondary to passage of biliary lithiasis or microlithiasis through the sphincter), which becomes a fibrosis with subsequent stenosis of part of or the entire sphincter. • Type 2 is considered for patients with biliary- or pancreatic-type pain and only one other criterion (abnormal laboratory tests or ductal dilation). These patients are thought to suffer from a functional alteration of the physiological motility of the sphincter that causes some delay in the passage of biliary or pancreatic juices into the duodenum. • Type 3 is reported for patients with only typical clinical symptoms. Pancreas divisum (PD) is the most common congenital variant of the human pancreas occurring in 5% to 10% of Caucasian individuals. 12,13 Less than 5% of the population with PD ever develop pancreatic symptoms; subsequently, PD seems to have little clinical relevance. 2 Although controversial, it is postulated that a relative outflow obstruction at the site of the minor papilla overburdened by draining of the larger dorsal pancreas may be the mechanism of pancreatitis in some patients with PD and unexplained ARP. 4,14 However, the frequency of PD is similar in control patients, patients with chronic pancreatitis and patients with idiopathic pancreatitis. 12,15 Recruitment bias (greater frequency of PD diagnosis in patients referred after unsuccessful opacification of the pancreatic ductal system) may have resulted in an overestimation of the prevalence of PD in endoscopic retrograde cholangio-pancreatography (ERCP) studies investigating suspected idiopathic ARP. 16 It has been shown that a persistent dilation of the main pancreatic duct greater than 3mm at 10 minutes after secretin injection assessed during secretin-enhanced magnetic resonance cholangio-pancreatography (S-MRCP) is correlated with a clinical diagnosis of papillary stenosis. 15,17 However, the frequency of such a response to secretin suggesting a functional or organic stenosis of the major or minor papilla is the same in patients with or without PD. 15 Other abnormalities that have been associated with ARP include anomalous pancreaticobiliary union, choledochocele, duodenal duplication cyst and annular pancreas. 18 BUSINESS BRIEFING: EUROPEAN GASTROENTEROLOGY REVIEW 2005 Acute Relapsing Pancreatitis Figure 1: Coronal View on T2-weighted MRI, S-MRCP and ERCP of a 42-year-old Man with Prior Cholecystectomy having Presented with 10 Attacks of Acute Pancreatitis over Two Years A B C D A 42-year-old man with a history of cholecystectomy presented 10 attacks of acute pancreatitis over two years during which he was admitted to hospital five times. This coronal view on T2-weighted MRI (A) and S-MRCP (B) showed a 2cm communicating cytic lesion at the junction of body and tail of the pancreas. ERCP demonstrated a normal ductal system in the head of the pancreas (C) and a typical branch duct type of intraductal papillary mucinous tumour (IPMT) with intraductal filling defect corresponding to mucus (D). A segmental pancreatectomy was performed and pathology revealed a branch duct IPMT with in situ carcinoma. An isolated, unexplained pancreatic duct stricture discovered during the work-up of a first episode of acute pancreatitis in a patient over 35 years of age without predisposing cause requires exclusion of an underlying pancreatic carcinoma. 5 Intraductal papillary mucinous tumour (IPMT) is the most frequent neoplasm associated with ARP. It is an intraductal pre-malignant lesion producing mucin that blocks the pancreatic duct, thus impeding outflow and encouraging bouts of pancreatitis. IPMT is characterised by dilation and filling defects of the main pancreatic duct or of the branch duct system (see Figure 1). 19 Miscellaneous Causes Genetic diseases can be identified as rare causes of acute and recurrent pancreatitis mainly in subjects with early onset of pancreatitis and with a history of first- or second-degree relatives with unexplained pancreatitis. 4 These inherited causes are mainly related to cystic fibrosis transmembrane conductance regulator gene mutations, and trypsinogen-gene mutations. Autoimmune pancreatitis is also a rare entity characterised by mild pancreatitis associated with abnormal laboratory findings (e.g. elevated serum immunoglobulin (Ig)G4 levels and presence of autoantibodies), imaging studies showing a diffusely 2
3 Reference Section Figure 2: MRI and MRCP Images of a 51-year-old Woman having Presented with Seven Attacks of Acute Pancreatitis Since 1998 A B C A 51-year-old woman presented seven attacks of acute pancreatitis since 1998. She underwent a cholecystectomy in May 2000, but acute pancreatitis recurred, with four attacks during the last 12 months. In this case, there was no alcohol abuse, no smoking and no drugs involved. A genetic origin remained possible, as family history was positive for one third-degree relative with unexplained ARP. However, genetic testing was negative in the patient. The CT scan showed no calcifications. T2-weighted MRI (A) and MRCP before (B) and after (C) secretin injection that were performed one week after the last attack of pancreatitis demonstrated a normal pancreatic gland and a normal main pancreatic duct. The dynamic response to secretin was also normal with a normal duodenal filling (C). Endoscopic ultrasonography was also performed and was normal. A pancreatic SOD type 2 is suspected (based on pancreatic-type pain and increased pancreatic hydrolases without dilation of the main pancreatic duct). An ERCP with dual endoscopic sphincterotomy and transient main pancreatic duct stenting has been proposed to the patient if another attack occurs. swollen pancreas and irregular narrowing of the duct due to lymphoplasmacytic infiltration, responding favourably to steroid therapy and sometimes associated with other autoimmune disorders. 5 Finally, very rare miscellaneous causes of ARP include vascular disorders, infections (e.g. tuberculosis, virus and parasite), and tropical pancreatitis. 4 Among the patients without an obvious cause for ARP, this means that around 20% to 30% of all cases with ARP, SOD, PD and occult biliary stones and tumours are generally considered as aetiological associated factors in 30%, 20%, 20% and 10%, respectively, leaving only approximately 20% of ‘idiopathic’ ARP related to genetic, autoimmune, infectious and really unknown factors (see Figure 2). 20 Initial Evaluation Procedures History and physical examination are probably the most important parts of the initial evaluation to search evidence for alcohol abuse, drug-induced pancreatitis and a family history of pancreatitis. Routine blood tests should include liver function tests within 24 to 48 hours of onset of symptoms (a three- fold or greater increase in the alanine aminotransferase (ALT) level is generally regarded as the best indicator of gallstone-induced pancreatitis), and the serum levels of calcium and triglyceride soon after admission (because of the drop in calcium and triglyceride levels due to fasting and administration of intravenous fluids). 8 Abdominal U/S is routinely performed in patients without previous cholecystectomy in order to detect gallbladder stones (with an accuracy of >90%). Contrast-enhanced CT scan is usually performed either in all patients during their first episode to confirm the diagnosis of acute pancreatitis and to assess the severity, 8 or only when the attack is severe, when the course is complicated, when aetiology remains unclear or in the elderly. 2 Recently, the use of gadolinium-enhanced dynamic MRI was found to be comparable with contrastenhanced CT for the assessment of the severity of acute pancreatitis, while avoiding the use of iodinated contrast medium and radiation, and with the ability to identify bile duct stones more accurately than CT. 21 S-MRCP should be considered as the first-choice procedure in the diagnostic algorithm of ARP (see Figure 3), because its diagnostic accuracy in detecting the various aetiological lesions of ARP is similar to that of ERCP without carrying the risk of potential ERCP-related complications. This initial work-up during an attack of pancreatitis detects the causes of ARP in about 70% of cases. 2 Further Evaluation Procedures If the initial work-up following an attack of pancreatitis is negative and successive attacks occur, or if the first episode of pancreatitis is moderate to severe or occurs after the age of 40 years of age, a more extensive evaluation will reveal a diagnosis in around two-thirds of this group of patients. 2 Advanced laboratory analysis may include genetic testing in patients younger than 40 years of age, tumour markers (carbohydrate antigen (CA) 19–9) in patients older than 40 years of age with a positive family history or with tobacco use, and serological markers of autoimmune pancreatitis if imaging studies are compatible with this diagnosis. As an additional diagnostic procedure, endoscopic ultrasonography (EUS) is able to postulate a cause for pancreatitis in approximately one-third of the patients with ARP for whom conventional evaluation, including transabdominal U/S and pancreatic CT with contrast injection, is negative. 22 Due to its complication rate being lower than that of ERCP, EUS should be performed earlier in the BUSINESS BRIEFING: EUROPEAN GASTROENTEROLOGY REVIEW 2005
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Acute Relapsing Pancreatitis - Touch Gastroenterology

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