Idiopathic Recurrent Pancreatitis Research Articles

Descriptive study for recurrent idiopathic acute pancreatitis cases admitted in Kasr El-Ainy endoscopy unit

Descriptive study for recurrent idiopathic acute pancreatitis cases admitted in Kasr El-Ainy endoscopy unit

Severity and outcome of a first episode of idiopathic acute pancreatitis is not more severe than pancreatitis of other etiologies

BackgroundWith respect to severity and outcome of an index episode of idiopathic acute pancreatitis the current literature reports conflicting retrospective results. One reason might be the retrospective study design precluding in depth analysis resulting in mixed etiologies and combination of index episode versus recurrent idiopathic acute pancreatitis. MethodsIn this retrospective monocentric cohort study, we retrieved all patients with a first acute pancreatitis episode treated between 2005 and 2021 at the LMU University Hospital from our clinical information system based on the respective ICD-10 codes. In an initial sample of 1390 presumed idiopathic pancreatitis patients we identified 68 confirmed idiopathic acute pancreatitis patients and compared those to 75 first-time alcohol-induced acute pancreatitis patients and 390 first-time biliary-induced acute pancreatitis patients. Clinical outcome (severity, SIRS, mortality, and re-admission rate) was set as outcome measures. Multinomial logistic regression analysis was performed. ResultsIn alcohol-induced acute pancreatitis moderate and severe courses occur significantly more often when compared to idiopathic acute pancreatitis (17.33 % vs. 10.29 %; multinomial logistic regression p = 0.0021). There were no significant differences in mortality between first-time alcoholic, idiopathic and biliary pancreatitis (p = 0.6328). Patients with idiopathic acute pancreatitis had significantly more hospital readmissions (within 30 days) compared to alcohol-induced pancreatitis patients (p = 0.0284). ConclusionIn the context of a first episode of acute pancreatitis, idiopathic acute pancreatitis remains a challenging diagnosis posing an increased risk of recurrence, but not an increased risk for a more severe disease course.

Current treatment of biliary Sphincter of Oddi Dysfunction.

The sphincter of Oddi is a delicate neuromuscular structure located at the junction of the biliary-pancreatic system and the duodenum. Sphincter of Oddi Dysfunction (SOD) can result in various clinical manifestations, including biliary-type pain and recurrent idiopathic pancreatitis. The management of SOD has been challenging. With the publication of the landmark Evaluating Predictors and Interventions in Sphincter of Oddi Dysfunction (EPISOD) trial and the Rome IV consensus, our clinical practice in the treatment of SOD has changed significantly in recent years. Currently, the management of type II SOD remains controversial and there is a lack of non-invasive therapy options, particularly for patients not responding to endoscopic treatment. In this mini review, we aimed to discuss the current knowledge on the treatment of biliary SOD.

Antioxidant therapy decreases pain frequency in patients with idiopathic recurrent acute pancreatitis

Antioxidant therapy decreases pain frequency in patients with idiopathic recurrent acute pancreatitis

A292 ACUTE PANCREATITIS AS THE FIRST PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS (GPA): A CASE REPORT

Abstract Background Granulomatosis with Polyangiitis (GPA) is a rare necrotizing ANCA-associated vasculitis characterized by inflammation in small-sized arteries. GPA often presents with a triad of a) upper (nasal obstruction, sinusitis, crusting rhinitis) and lower respiratory tract (lung nodules, alveolar hemorrhage) b) systemic vasculitis and c) kidney involvement (necrotizing glomerulonephritis). However, gastrointestinal involvement is exceedingly rare and only occurs in about 5-11% of GPA cases. Specifically, recurrent acute pancreatitis is even more uncommon. Purpose To raise awareness about systemic vasculitides in cases of idiopathic acute pancreatitis Method A 48-year-old female was seen in an outpatient gastroenterology clinic for recurrent idiopathic pancreatitis. She reported a six-month history of intermittent sharp epigastric pain associated with a rise in lipase. The patient noted having episodes of acute sinusitis shortly prior to the onset of her epigastric pain. Her initial labwork revealed normal creatinine and liver enzymes, elevated total bilirubin 24, lipase 316 and mildly high CRP 16.1 and ESR 27 which normalized on repeat blood work. Abdominal US was unremarkable with no gallstones, intra- or extrahepatic duct dilatation. An abdominal MRI revealed segmental enlargement of the distal tail/body of the pancreas consistent with resolving focal pancreatitis. A follow-up MRI 5 weeks later revealed progression of pancreatic swelling and intermittent narrowing of the pancreatic duct. These findings were suspicious for an inflammatory process such as autoimmune pancreatitis. The patient then underwent EUS-guided examination and FNA of her pancreas. Pathology revealed focal chronic pancreatitis with fibrosis and mild lymphocytic infiltrate, but was not suggestive of autoimmune pancreatitis. She was then treated empirically with a 3-month course of prednisone and had significant improvement in her symptoms and interval resolution of pancreatic inflammation on repeat MRI. After discontinuation of her steroids, her symptoms recurred with intermittent epigastric pain, facial pain and sinusitis. She was then seen by rheumatology and an autoimmune panel was ordered. Result(s) Autoimmune work-up revelead positive anti-PR3 (27 RU/ml), negative anti-MPO and normal IgG-4 levels. Ultimately a diagnosis of limited GPA, which spares the kidneys, was made given the patient’s clinical presentation and positive anti-PR3 antibody. Since there was no life-threatening organ involvement, she was elected to start methotrexate therapy and had significant improvement in her symptoms. The plan is to stay on methotrexate for at least 24 months and have regular follow-up to ensure clinical stability. Conclusion(s) Acute pancreatitis is a rare initial presentation of GPA vasculitis. It is important to consider autoimmune disease and systemic vasculitides in cases of idiopathic pancreatitis after ruling out common causes. Early diagnosis and therapy allow for high rates of remission and improved survival rates. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

CFTR-Related Metabolic Syndrome: Genetic Variants Increasing Pancreatitis Risk in the Pediatric Puerto Rican Population

CFTR-related metabolic syndrome (CRMS) is a novel diagnosis due to widespread use of and advances in the newborn screening (NBS) process for cystic fibrosis (CF) in the United States of America, allowing for the diagnosis of asymptomatic children with CF. Before 2015, a large Puerto Rican pediatric population was not screened for CF in the NBS test. Studies have shown that patients presenting with idiopathic recurrent or chronic pancreatitis have an increased frequency of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. We present a retrospective chart review of 12 pediatric cases (n = 12) that were presented to an outpatient community clinic with clinical manifestations associated with CF. The pancreatic insufficiency prevalence (PIP) score was calculated on CFTR mutations. The mutations considered for the calculation of the PIP score were: F508del (c.1521_1523del), V201M (c.601G > A), I507del (c.1519_1521del), and L1335P (c.4004T > C). V201M mutation was classified as mild in both PIP scores, and a correlation with pancreatitis was noted. Clinical manifestations vary in cases with the V201M variant (c.601G > A). One case was diagnosed with CFTR-related disorder (CRD) and recurrent pancreatitis. It is important to consider CRMS or CRD as a differential diagnosis in the pediatric population of Puerto Rico due to the implications and increased risk of pancreatitis and other CF-related complications.

Evaluating the role of endoscopic ultrasound in pancreatitis

ABSTRACT Introduction Endoscopic ultrasound (EUS) provides high-resolution images of both pancreatic parenchyma and duct and therefore is an integral component of evaluating and treating patients with pancreatitis and its complications. The development of enhanced EUS imaging techniques and newer EUS-specific accessories has expanded the diagnostic and therapeutic role of EUS in patients with acute and chronic pancreatitis (CP). Areas covered This review discusses the current diagnostic and therapeutic role of EUS in acute pancreatitis (AP), CP, and autoimmune pancreatitis (AIP). Expert opinion EUS plays a vital role in patients with AP by confirming the presence of common bile duct (CBD) stones in patients with acute biliary pancreatitis and intermediate probability of CBD stones. It plays an important role in the etiological evaluation of patients with idiopathic acute and recurrent pancreatitis. EUS is also an essential modality for diagnosing and managing pancreatico-biliary as well as gastroduodenal complications associated with CP. EUS-guided FNB using newer generation core biopsy needles has made possible accurate diagnosis of AIP by providing tissue samples with preserved architecture.

Роль однонуклеотидных полиморфизмов генов провоспалительных факторов как предикторов риска развития и тяжести течения острого панкреатита

A literature review has been conducted examining the role of single nucleotide polymorphisms of pro-inflammatory factor genes as predictors of risk of developing and severity of acute pancreatitis. The review was conducted on the following databases: Pub Med, Scopus for the period from 2009 to 2021 according to the following keywords: acute pancreatitis, hereditary pancreatitis, severe acute pancreatitis, gene polymorphism, single nucleotide genes, proinflammatory factors. A number of mutations and single nucleotide polymorphisms have been identified, which are the main triggers or cofactors of the initiation of both acute and chronic hereditary pancreatitis. The percentage of cases of idiopathic recurrent acute pancreatitis and idiopathic chronic pancreatitis associated with genetic mutations is 30-60% and 12-43%, respectively. The following genetic variants of the cationic trypsinogen (PRSS1) gene - CFTR, SPINK1 and chymotripsin C (CTRC) - are most often associated with the development of pancreatitis. Inherited pancreatitis associated with gene mutations has SPINK1 autosomal recessive inheritance pattern. When the mutant gene is expressed, the synthesis of a strong trypsin inhibitor prevents premature activation of immature trypsinogen. Data are obtained that IL-6, IL-8, FNOα can activate NF kB transcription factor, inducing, thus, increase in an expression 2 DRIED. It has been found that some COX 2 polymorphisms characterized by increased expression of this enzyme create prerequisites for the progression of the inflammation process, and are possibly predictors of the development of severe acute pancreatitis.

The role of endoscopic ultrasound and magnetic resonance cholangiopancreatography in the diagnosis of idiopathic recurrent acute pancreatitis: a narrative review

Objective: Our aim was to provide a qualitative synthesis of the literature to formulate an evidence-based approach to the diagnostic evaluation of patients with idiopathic recurrent acute pancreatitis (IRAP).

Impact of genetic testing and smoking on the distribution of risk factors in patients with recurrent acute and chronic pancreatitis

Objective The aim of the present study is to assess the impact of smoking dose and duration on the distribution of risk factor(s) in patients with RAP and CP, and the impact of genetic testing on the distribution of risk factor(s) in patients with idiopathic RAP and CP. Methods All adult patients with RAP and CP referred to a multidisciplinary pancreatitis clinic between 2010 and 2017 were evaluated. Risk factors included alcohol and smoking, hypertriglyceridemia, biliary, and other etiologies. Genetic testing was only pursued in patients with idiopathic RAP or CP. Results Among the 1770 patients evaluated, 167 had RAP and 303 had CP. After genetic testing and smoking, the most common risk factors for RAP and CP were pathogenic variant(s) (23%) and the combination of alcohol and smoking (23%), respectively. Genetic testing and smoking assessment decreased the proportion of patients with alcoholic RAP from 17% to 5%, alcoholic CP from 33% to 10%, idiopathic RAP from 49% to 12%, and idiopathic CP from 54% to 14%. Pathogenic CFTR variants were the most common variant in patients with RAP (51%) and CP (43%). Among the 68 patients with pancreas divisum, other risk factor(s) were identified in 72%. Conclusion Genetic testing and a detailed assessment of smoking dose and duration reduce the proportion of patients with alcoholic and idiopathic pancreatitis. Other risk factor(s) for pancreatitis are found in the majority of patients with pancreas divisum further questioning its role as an independent risk factor. 1. What is the current knowledge? Approximately 30% of patients with pancreatitis have no clear risk factor(s) and are categorized as having an idiopathic etiology. Pathogenic variant(s) as well as smoking dose and duration are well-established risk factors for recurrent acute and chronic pancreatitis but are not widely recognized or incorporated into clinical practice. 2. What is new here? Genetic testing and a detailed assessment of smoking dose and duration reduced the proportion of patients with alcoholic and idiopathic acute recurrent and chronic pancreatitis. Approximately three-fourths of patients with pancreas divisum have a risk factor for pancreatitis.

Idiopathic recurrent pancreatitis in a young woman: Unexpected clue

Idiopathic recurrent pancreatitis in a young woman: Unexpected clue

Comparison of idiopathic recurrent acute pancreatitis [IRAP] and recurrent acute pancreatitis with genetic mutations

BackgroundIdiopathic recurrent acute pancreatitis (IRAP) describes frequent episodes of pancreatitis without an etiology found using current testing. We compared the natural history of IRAP with recurrent acute pancreatitis with genetic mutations. MethodsRetrospective cohort of patients with recurrent acute pancreatitis (≥2 episodes) and negative conventional testing. All patients had ≥1 episode after cholecystectomy and completed genetic testing. Primary outcomes were chronic pancreatitis incidence, pancreatic cancer, and mortality. Secondary outcomes included opioid and ERCP utilization. Results128 patients met criteria for presumed IRAP. 35 patients met criteria for true IRAP. 12 patients had recurrent acute pancreatitis with gene mutations. Chronic pancreatitis developed in 27 (77.1%) IRAP patients over a median of 6 years. Chronic pancreatitis incidence was similar in IRAP and CFTR mutation carriers; but developed later in SPINK1 carriers. No patients developed pancreatic cancer or died from pancreatic-related causes. Patients were frequently treated with oral opioids and ERCP, without significant differences within or between groups. ConclusionIRAP and pancreatitis in mutation carriers is associated with chronic pancreatitis. Important differences in natural history were observed, but no association was found with cancer or pancreas-related mortality. Efforts to understand the genetic contributions to IRAP, minimize opioids and unnecessary ERCPs are encouraged.

Disfunción del esfínter de Oddi: un reporte de caso

La disfunción del esfínter de Oddi es un síndrome clínico causado por una enfermedad funcional (discinesia) o estructural (estenosis). La prevalencia estimada de disfunción del esfínter de Oddi en la población en general es del 1 %; aumentando a 20 % para pacientes con dolor persistente posterior a colecistectomía y a 70 % en pacientes con pancreatitis aguda recurrente idiopática. Se caracteriza clínicamente por la presencia de dolor abdominal, similar al cólico biliar o dolor tipo pancreático en ausencia de patología biliar orgánica; así como en pacientes con pancreatitis recurrente idiopática asociada con elevación de enzimas pancreáticas o hepáticas, y dilatación del conducto biliar o pancreático. El tratamiento para la disfunción del esfínter de Oddi tipo I se basa en la realización de esfinterotomía endoscópica, pero existe controversia en el manejo de la disfunción del esfínter de Oddi tipo II y III. En este artículo se presenta el caso clínico de una paciente de 67 años con antecedente de colecistectomía por laparotomía. Después del procedimiento quirúrgico refirió un dolor abdominal de predominio en el hipocondrio derecho tipo cólico asociado con emesis de características biliares. En el reporte de colangiorresonancia se encontró una ligera dilatación de la vía biliar intrahepática y gammagrafía con ácido iminodiacético hepatobiliar (HIDA) diagnóstica de disfunción del esfínter de Oddi. Se realizó una esfinterotomía endoscópica. En el seguimiento, dos años después, la paciente se encontraba asintomática con la disfunción del esfínter de Oddi resuelta.

Utility of endoscopic ultrasound in idiopathic acute recurrent pancreatitis

Idiopathic acute recurrent pancreatitis (IARP) is defined as at least two episodes of acute pancreatitis with the complete or near-complete resolution of symptoms and signs of pancreatitis between episodes, without...

S1414 Successful Endoscopic Management of Necrotizing Pancreatitis Due to Rare Anatomical Variants

INTRODUCTION: Pancreatic divisum (PD) is an embryological failure of fusion of the ventral and dorsal pancreatic ducts. Meandering main pancreatic duct (MMPD) is another rare developmental loop-type anomaly in the main duct. PD and MMPD are more prevalent in idiopathic recurrent acute pancreatitis (IRAP). We report a case of recurrent necrotizing pancreatitis in a patient with these anatomical variants, fascinatingly resulting in complete resolution after staged direct endoscopic necrosectomies. CASE DESCRIPTION/METHODS: A 51-year-old male presented with epigastric pain and vomiting. Physical examination, complete blood count, complete metabolic panel, lipase and lactic acid were unremarkable. Abdominal CT showed acute pancreatitis with 10% parenchymal necrosis and 7 × 8 × 15 cm peripancreatic walled-off necrosis. He was managed with fluids, pain and anti-emetics and tolerated diet advancement. The etiology of his pancreatitis was unclear given absence of alcohol abuse and biliary pathology and negative autoimmune work-up. He was discharged with plans to repeat imaging 6 weeks later at which time he was readmitted for fevers and pain recurrence. Abdominal CT showed increase in size of the walled-off necrosis to 15 × 10 × 20 cm (Figure 1). He was treated with antibiotics and underwent endoscopic cystogastrostomy. His clinical course was complicated by splenic vein and upper extremity deep venous thrombi and a pulmonary embolism requiring anticoagulation. While on anticoagulation, he underwent 14 careful staged endoscopic necrosectomies over two months until complete resolution (Figure 2). Subsequent ERCP demonstrated incomplete PD and reverse-Z MMPD (Figure 3). A stent was placed into the ventral pancreatic duct and he was managed with pancreatic enzyme replacement, remaining asymptomatic on 1-year follow-up. DISCUSSION: PD and MMPD are rare embryological errors, carrying ∼40% prevalence in cases of IRAP and are thought to play a role in disease onset. Necrosis complicates 20% of acute pancreatitis cases with a 25% mortality rate. While two thirds of cases resolve with conservative measures, the remainder require intervention with endoscopic necrosectomy to avoid open surgical approaches with high mortality. Large multi-center studies have demonstrated successful resolution with endoscopic necrosectomy in ∼90% of cases with significantly lower mortality. This case serves to illustrate the effectiveness of endoscopic necrosectomy even in the face of anticoagulation and to prompt physicians to consider PD and MMPD in IRAP.Figure 1.: CT showing necrotic collection.Figure 2.: Endoscopic necrosectomy.Figure 3.: ERCP showing PD and MMPD anatomical variants.

Idiopathic recurrent pancreatitis in a young woman: unexpected clue

The article presents a complex clinical case of idiopathic recurrent pancreatitis in a young woman, 23 years old. The main clinical manifestation of the disease was acute, intense, recurrent abdominal pain. Despite ongoing symptomatic therapy, the patient’s condition had been worsened, and the intensity of abdominal pain increased. Adequate dosage of the enzyme replacement therapy allowed increasing the duration of remission. Differential diagnosis, additional laboratory and instrumental studies allowed determining the cause of recurrent pancreatitis and confirming the diagnosis of cystic fibrosis with a primary lesion of the pancreas. A distinctive feature of this clinical case is the diagnosis of a hereditary cystic fibrosis disease in adulthood, the absence of clinical symptoms of lungs’ involvement in the pathological process, as well as the absence of underdevelopment, malnutrition. The diagnosis of cystic fibrosis is confirmed by German specialists; currently, the patient continues treatment in a German clinic.
 The article presents current data on idiopathic pancreatitis, discusses a diagnostic algorithm for identification of the cause of the disease. The etiology, pathogenetic mechanisms of cystic fibrosis occurrence are considered, the known types of CFTR gene mutations, causing the development of the disease, as well as the principles of replacement enzyme therapy in patients with cystic fibrosis are described. Evidence-based medicine data are presented, revealing the efficacy and safety of taking enzyme preparations in the correction of the exocrine pancreatic insufficiency.

A rare case of acute pancreatitis: a diagnostic conundrum and unusual complications

A 63-year-old man with a history of recurrent idiopathic acute pancreatitis (AP) was admitted to our surgical ward due to severe abdominal pain. He denied chronic excessive alcohol use. Other typical causes of AP, such as gallstones, hypertriglyceridemia, and trauma, were ruled out. After considering all possible etiologies, the most likely factor producing AP was medication that had been administered to him two weeks before the very first episode of the disease. Medication should always be considered as a possible trigger of AP, especially if the first episode occurs shortly after drug administration and the etiology is unclear. During patient’s hospitalization, laboratory reports revealed significant fluctuations in the serum levels of pancreatic enzymes, which can be attributed to recurrent bacteriemia. After the 30-day period of hospitalization and long-lasting antibiotic therapy, he was discharged in a good condition with normal levels of serum pancreatic enzymes.

A rare case of acute pancreatitis: a diagnostic conundrum and unusual complications.

A 63-year-old man with a history of recurrent idiopathic acute pancreatitis (AP) was admitted to our surgical ward due to severe abdominal pain. He denied chronic excessive alcohol use. Other typical causes of AP, such as gallstones, hypertriglyceridemia, and trauma, were ruled out. After considering all possible etiologies, the most likely factor producing AP was medication that had been administered to him two weeks before the very first episode of the disease. Medication should always be considered as a possible trigger of AP, especially if the first episode occurs shortly after drug administration and the etiology is unclear. During patient's hospitalization, laboratory reports revealed significant fluctuations in the serum levels of pancreatic enzymes, which can be attributed to recurrent bacteremia. After the 30-day period of hospitalization and long-lasting antibiotic therapy, he was discharged in a good condition with normal levels of serum pancreatic enzymes.

Acoustic radiation force impulse imaging of pancreas in patients with early onset idiopathic recurrent acute pancreatitis.

Patients with recurrent acute pancreatitis (RAP) may progress to chronic pancreatitis (CP). A critical step in this progression is the development of fibrosis. Elastography has been used to assess degree of fibrosis by measurement of shear wave velocity (SWV). The aim of this study was to measure the stiffness of pancreas as a surrogate for pancreatic fibrosis in patients with RAP and compare it with a group of individuals without any history of pancreatic disorders. Using acoustic radiation force impulse (ARFI) imaging SWV was calculated in 31 patients with idiopathic RAP having disease onset ≤30 years. A control group of 31 individuals with no history pancreatic disorder and no features of CP on endosonography was selected for comparison. ARFI imaging was done by a single radiologist who was blinded to the cases and controls RESULTS:: Mean age of patients with RAP was 24.8 (SD 9.86) years, disease duration was 3.45 (interquartile range 1-5) years, mean number of episodes 4.9 (SD 2.72). There was a significant difference in SWV between patients (1.27 ± 0.50 m/s) and controls (1.00 ± 0.17 m/s) (P = 0.001)). There was a positive correlation between SWV and number of pain episodes (P = 0.026) and negative correlation with BMI (P = 0.002). SWV was high in patients with RAP indicating a stiff pancreas. The stiffness increases with the number of episodes of pancreatitis.

Genetic Evaluation of Children with Idiopathic Recurrent Acute Pancreatitis.

Several genetic risk factors have been identified in adults with idiopathic acute recurrent pancreatitis (IARP). However, the literature regarding the genetics of IARP is sparse in children. In this study, we aimed to analyze the genetic risk factors in children with IARP. All children (< 18years) with ARP from January 2015 to May 2018 were prospectively enrolled in the study. Children with a known cause of ARP like obstructive, toxic/metabolic, and autoimmune were excluded from the final analysis. Children with IARP underwent genetic testing for mutations/polymorphisms in genes known to predispose to pancreatitis including cationic trypsinogen protease serine 1 (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1), cystic fibrosis transmembrane conductance regulator gene (CFTR), chymotrypsin C (CTRC), claudin-2 (CLDN2) and cathepsin B (CTSB). A total of 239 children (116 boys, 10.3 ± 3.7years) were enrolled during the study period. Of these, 204 (85.35%) children were identified as IARP. The mean age of symptom onset and the number of pancreatitis episodes were 8.3 ± 3.7years and 3.3 ± 1.8, respectively. A family history of pancreatitis was noted in 4.6% children. Mutations/polymorphisms in at least 1 gene were identified in 89.5% (129/144) children including SPINK1 in 41.9%, PRSS1 (rs10273639) in 58.2%, CTRC in 25.6%, CTSB in 54.9%, CLDN2 in 72.9%, and CFTR in 2.3%. There was no significant incidence of genetic mutations/polymorphisms in IARP with or without pancreas divisum (95.7 vs 88.4%; p = 0.467). Genetic alterations are present in the majority of the children with IARP. The incidence of genetic mutations is similar in children with or without pancreas divisum.