Original articleThe Spanish Pancreatic Club recommendations for the diagnosis and treatment of chronic pancreatitis: Part 1 (diagnosis)
Section snippets
Justification
CP is characterized by the development of deficiencies in both exocrine and endocrine function, with morphologic alterations affecting the parenchyma and the ducts of the pancreatic glands. This causes a great variation in the clinical manifestations of the disease. Its main symptom is pain that usually occurs in early stages when detectable functional and structural manifestations have not developed [1]. Recently, the advent of endoscopic ultrasound has allowed for the detection of minimal
Objective
For the above reasons, the Spanish Pancreatic Club held a consensus conference to guide the diagnostic and therapeutic approach of professionals who attend patients with CP.
Methodology
As in the previous consensus [7], [8], [9], the methodology used is a modification of the Consensus Development Conferences [10]. The sections of the conference were the panel of experts, the questions raised and the agenda. The responsibility for planning and managing the logistics of the consensus conference fell to the Pancreatic Pathology Unit of the University General Hospital of Alicante, Spain. The members of the panel of experts were chosen from amongst the faculty of various medical
What is chronic pancreatitis?
Despite extensive efforts over the past 50 years, there is no widely accepted clinical definition of CP. There have been several meetings of experts [12], [13], [14] with the aim of achieving a consensus. Each report that they have issued has based the definition of CP on the diagnostic methods available at that time, from histology to modern imaging techniques such as magnetic resonance imaging (MRI) [14].
From a general point of view, CP is defined as an inflammatory disease of the pancreas
Which non-endoscopic imaging techniques allow the diagnosis of chronic pancreatitis?
CP diagnosis by imaging techniques is based on the morphological changes of the gland that can be very evident in its advanced stages but difficult to detect in early stages [16], [17].
In plain abdominal radiography, the presence of calcifications in the pancreatic area with compatible clinical manifestations can be diagnostic of CP.
Transabdominal ultrasound only detects advanced stages of CP [18].
Computerized tomography (CT) is the best non-endoscopic imaging technique to diagnose and localize
Which endoscopic imaging techniques allow the diagnosis of CP?
Although ERCP has traditionally been considered the gold standard for morphological diagnosis, the emergence of new imaging methods, such as endoscopic ultrasound (EUS) and MRCP, along with the complications associated with ERCP, have relegated it to the background [22].
EUS is the most sensitive imaging method for CP diagnosis and allows for the targeted collection of samples [23]. Some criteria that characterize the disease have been defined and are divided amongst parenchymal and ductal
How is exocrine pancreatic insufficiency defined and diagnosed?
Based on the concept of insufficiency as the inability of an organ to perform its physiological function and taking into account the known functional reserve of the pancreas, exocrine pancreatic insufficiency (EPI) must refer exclusively to the situation in which the disturbance of pancreatic function is associated with an inability of the pancreas to facilitate normal digestion.
Currently, the gold standard for the diagnosis of EPI is the determination of the coefficient of fat absorption (CFA)
How is endocrine pancreatic insufficiency defined and diagnosed?
Diabetes mellitus secondary to CP (DM-CP), also classified as type 3c diabetes, is included in ‘other specific forms’ of diabetes in the etiological classification of DM of the American Diabetes Association and is defined as a group of metabolic diseases characterized by hyperglycaemia resulting from defects in insulin secretion and/or action secondary to processes that affect the pancreas diffusely [38].
For the diagnosis of DM-CP, it is recommended to determine fasting plasma glucose (FPG)
What is the etiology of chronic pancreatitis? What should be the initial etiologic study?
In 2001 [15] the etiologic classification system called TIGAR-O was published and subsequently modified [40]. This classification is based on the fact that in most cases, CP is the result of the interaction of multiple risk factors, although sometimes its etiology is unknown.
Today excessive alcohol consumption is considered the main cause of CP in industrialized countries, but it is estimated that there must be individual susceptibility (genetic basis combined with environmental co-factors) and
Are there different types of CP?
In general, the clinical, functional and morphological characteristics of patients with CP are similar [15]. However, certain etiological factors of the disease have well-differentiated behavior and histological features [51]. Therefore, CP can be classified according to clinical features, histology and response to treatment:
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Calcifying CP: characterized by abdominal pain, recurrent bouts of acute pancreatitis, development of calcification and the development of endocrine and exocrine pancreatic
When to request a genetic study of CP and how to interpret the results?
Hereditary CP is an autosomal dominant inherited disease with a penetrance of 80%. In 70% of hereditary CP patients, mutations of the protease, serine, 1 (trypsin 1) PRSS1 gene have been reported [53], [54]. Variants of the serine protease inhibitor Kazal type 1 (SPINK1) gene have also been associated with CP; SPINK1 blocks intrapancreatic trypsin activity to prevent additional activation of trypsinogen and limits further tissue damage [55]. The chymotrypsin C (CTRC) gene has low penetrance.
Autoimmune pancreatitis: how to diagnose it and how to treat it?
Autoimmune pancreatitis lacks specific symptomatology. The main differential diagnosis is pancreatic cancer, and autoimmune pancreatitis must be suspected when there is a pancreatopathy of unclear origin combined with autoimmune diseases or when confirmed after histological analysis [67]. Because clinical manifestations are not very sensitive or specific, diagnosis of autoimmune pancreatitis is based on radiological manifestations, laboratory test alterations and histological findings, although
What prognostic and developmental stage classification should be used?
Multiple classification systems of CP have been proposed; however, none of them has been extended to clinical practice or used as a standard for comparative studies.
The ABC system [85] divides patients according to the absence of abdominal pain (A), pain without complications (B) and pain with complications (C). The Japan Pancreas Society has proposed a classification that reflects the quality of life and can be used for assessments of clinical course and treatment effects [86]. The Manchester
What clinical and laboratory parameters should be used for the follow-up of patients with chronic pancreatitis?
The objective of monitoring CP is the early detection of endocrine and exocrine insufficiency and the presence of complications that can occur at any stage of the disease. These complications are pseudocysts, biliary obstruction, duodenal obstruction, bacterial overgrowth, pancreatic ascites, intraductal, retroperitoneal or intracystic hemorrhage, splenic and/or mesenteric thrombosis and pancreatic cancer. For monitoring CP, it is not well established how often follow-up should be performed and
In which CP patients, how and when should a pancreas cancer screening be performed?
The relationship between CP and pancreatic cancer has been confirmed in several epidemiological studies and cohort studies. However, these studies have obtained variable findings regarding risk quantification, depending on the methods and the type of CP. There should not be a temporal overlap between the diagnosis of CP and pancreatic cancer. Therefore, to be considered a true case of cancer in a patient with CP, there must be a minimum of 2 years of progression from the diagnosis of CP. A
Funding
The consensus has not received funding.
Conflicts of interest
Enrique de-Madaria, Enrique Domínguez-Muñoz, Julio Iglesias-García and José Lariño-Noia have been paid speakers by Abbott Laboratories. Enrique Domínguez-Muñoz is a Consultant for Abbott Laboratories and Pentax. Julio Iglesias-García is a Consultant for Cook Medical Company. Luis Gómez and Yolanda Sastre have been paid speakers by Mundipharma, Zambon, Ferrer Pharma and Grunenthal Pharma. José Ramón Aparicio is a Consultant for Boston Scientific.
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Cited by (28)
Recommendations from the United European Gastroenterology evidence-based guidelines for the diagnosis and therapy of chronic pancreatitis
2018, PancreatologyCitation Excerpt :The Harmonizing diagnosis and treatment of chronic pancreatitis across Europe (HaPanEU) initiative of United European Gastroenterology (UEG) aims to provide the community with evidence-based, state-of-the-art clinical guidelines to help in the management of patients with chronic pancreatitis (CP) [1]. The statements are based on the recent guidelines and recommendations published by the Australian [2], Belgian [3], German [4], Hungarian [5], Italian [6], Romanian [7], and Spanish [8,9] Societies of Gastroenterology and Pancreatology, as well as pertinent new literature. The recommendations format comprised the question, the statement, its level of evidence and strength of recommendation, and the percentage agreement of the global consensus group with the final version.
Diagnosis of exocrine pancreatic insufficiency in chronic pancreatitis: <sup>13</sup>C-Mixed Triglyceride Breath Test versus Fecal Elastase
2017, PancreatologyCitation Excerpt :The CFA is the proportion of dietary fat absorbed in the digestive tract. A CFA lower than 93% (that corresponds to a daily fat excretion higher than 7 g with a daily fat intake of 100 g) is characteristically indicative of EPI in clinical practice [10,15–17]. Unfortunately, the fecal fat assays are not easy to perform.
Chronic pancreatitis: An international draft consensus proposal for a new mechanistic definition
2016, PancreatologyCitation Excerpt :The workshop members therefore defined CP as “a continuing inflammatory disease of the pancreas, characterized by irreversible morphological change, and typically causing pain and/or permanent loss of function.” Other groups have adopted this, or similar definitions, including the recent Italian consensus guidelines (ICG) [20] and Spanish recommendations (SR) [21,22]. From 1991 to 1994 the Japanese Pancreas Society met to defined CP.
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On behalf of Spanish Pancreatic Club