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Dermatomyositis (DM) is a relatively uncommon inflammatory myopathy that has been linked to cancer. We report the case of an 81-year-old woman with cecum adenocarcinoma presenting with antinuclear antibody (ANA) and anti-Mi-2-alpha antibody-positive DM. The patient complained of anorexia, symmetric proximal muscle weakness and skin rash and presented with elevated muscle enzymes. A skin and muscle biopsy supported the diagnosis of DM as did the limbs magnetic resonance imaging (MRI) and electromyography. A diagnosis of localized adenocarcinoma of the cecum was made through colonoscopy and the patient was successfully surgically managed, with decreasing muscle enzymes at discharge and gradual recovery of muscle strength. The presence of both ANA and anti-Mi-2 autoantibodies has classically been described as comprising a better prognosis with a lower risk of underlying malignancy. This case highlights the importance of pursuing a cancer diagnosis in elderly patients presenting with DM even in presence of less predisposing immunological profiles.

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Chronic pancreatitis (CP) is a complex disease that should be treated by experienced teams of gastroenterologists, radiologists, surgeons, and nutritionists in a multidisciplinary environment. Medical treatment includes lifestyle modification, nutrition, exocrine and endocrine pancreatic insufficiency correction, and pain management. Up to 60% of patients will ultimately require some type of endoscopic or surgical intervention for treatment. However, regardless of the modality, they are often ineffective unless smoking and alcohol cessation is achieved. Surgery retains a major role in the treatment of CP patients with intractable chronic pain or suspected pancreatic mass. For other complications like biliary or gastroduodenal obstruction, pseudocyst drainage can be performed endoscopically. The recommendations for CP were developed by Clube Português do Pâncreas (CPP), based on literature review to answer predefined topics, subsequently discussed and approved by all members of CPP. Recommendations are separated in two parts: "chronic pancreatitis etiology, natural history, and diagnosis," and "chronic pancreatitis medical, endoscopic, and surgical treatment." This abstract pertains to part II.

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Chronic pancreatitis (CP) is a heterogeneous disease, with different causes and often a long delay between onset and full classic presentation. Clinical presentation depends on the stage of the disease. In earlier stages, recurrent episodes of acute pancreatitis are the major signs dominating clinical presentation. As the inflammatory process goes on, less acute episodes occur, and pain adopts different aspects or may even disappear. After 10-15 years from onset, functional insufficiency occurs. Then, a classic presentation with pain and pancreatic exocrine and endocrine insufficiency appears. Diagnosis remains challenging in the early stages of the disease, as its initial presentation is usually ill-defined and overlaps with other digestive disorders. Computed tomography and magnetic resonance cholangiopancreatography should be the first choice in patients with suspected CP. If the results are normal or equivocal but still there is a high suspicion of CP, the next option should be endoscopic ultrasound. Endoscopic retrograde cholangiopancreatography is mainly a therapeutic technique, and for the diagnostic purpose should only be used when all other imaging modalities and pancreatic function tests have been exhausted. Indirect tests are used to quantify the degree of insufficiency in already-established late CP. Recommendations on CP were developed by Clube Português do Pâncreas (CPP), based on literature review to answer predefined topics, subsequently discussed and approved by all members of CPP. Recommendations are separated in two parts: "chronic pancreatitis etiology, natural history, and diagnosis," and "chronic pancreatitis medical, endoscopic, and surgical treatment." This abstract pertains to part I.

A pancreatite crónica (PC) é uma doença heterogénea, com diferentes etiologias, muitas vezes, com um longo período entre o início de sintomatologia e a apresentação clínica clássica. A clínica depende do estadio da doença, sendo que nos estadios iniciais, predominam episódios recorrentes de pancreatite aguda; com a progressão da doença, os episódios agudos tornam-se menos frequentes, e a dor adota padrões diferentes, podendo inclusive desaparecer; a insuficiência funcional desenvolve-se 10 a 15 anos após o início, assumindo-se então, a apresentação clássica com dor, insuficiência pancreática exócrina e endócrina. O diagnóstico pode ser desafiador nos estadios iniciais da doença, já que a apresentação inicial é geralmente mal definida e se sobrepõe a outros patologias gastrointestinais. A TAC e CPRM devem ser os primeiros métodos de imagem em doentes com suspeita de PC. Se os resultados forem normais ou ambíguos, a próxima opção deve ser a ecoendoscopia. A CPRE é uma técnica principalmente terapêutica, sendo que para fins de diagnóstico, deve ser reservada para quando todas os outros exames de imagem/testes de função pancreática forem inconclusivos. Testes indiretos de função pancreática devem ser usados para quantificação do grau de insuficiência pancreática em doentes com PC já estabelecida. As recomendações sobre PC foram desenvolvidas pelo Clube Português do Pâncreas (CPP), com base numa revisão da literatura para responder a questões predefinidas, posteriormente discutidos e aprovados por todos os membros do CPP. As recomendações encontram-se separadas em duas partes: "etiologia da pancreatite crónica, história natural e diagnóstico" e "tratamento médico, endoscópico e cirúrgico da pancreatite crónica." Este resumo corresponde à parte I.

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BACKGROUND: Acute pancreatitis (AP) is an aggressive and potentially fatal clinical condition. Although all age groups are at risk, the elderly may be a group of special concern. We aimed at evaluating clinical outcomes of patients with elderly-onset AP. MATERIALS AND METHODS: Using a single-center retrospective database, treatment and follow-up records of 550 patients admitted with AP were reviewed. Outcomes included mortality, admission to the ICU, need for interventional procedures, nutritional support, and length of hospital and ICU stay. Elderly-onset AP was defined as an episode of AP occurring in patients older than 65 years. RESULTS: A total of 263 patients were classified as having elderly-onset AP. There was an association between older age and higher Ranson and the bedside index of severity in AP scores, translating into longer lengths of hospital stay, higher requirements for ICU admission, interventional procedures, organ failure, persistent organ failure, and overall mortality. In multivariate analysis, age was an independent predictor of mortality in AP. CONCLUSIONS: Age was strongly associated with a more severe course of AP. Early recognition and prompt action are essential to improve outcomes in this population.

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BACKGROUND: Acute liver failure (ALF) induced by diffuse metastatic disease has rarely been reported. CASE PRESENTATION: We present a 51-years-old woman with relevant clinical history for breast cancer. The patient was admitted in the emergency department with jaundice, dark urine and pale stools. She was on the 10th day of hormonotherapy for recurrence of breast cancer, diagnosed 7 years previously. Usual causes of acute liver failure were excluded, all drugs were stopped and the imaging studies performed were positive only for steatosis. Nonetheless, ALF progressed and the patient died 4 days later. Autopsy demonstrated a massive intrasinusoidal infiltration of the liver by breast cancer cells. CONCLUSION: We highlight a rare cause of ALF. Although uncommon, physicians should be alert for this situation as the diagnosis can be challenging and the imaging studies can remain normal.

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Intraductal papillary mucinous neoplasm represents the most common pancreatic cystic neoplasm and harbours significant malignant potential. Duct obliteration can lead to recurrent episodes of pancreatic inflammation mimicking acute and chronic pancreatitis. We report a case of a patient with multiple episodes of idiopathic pancreatitis occurring over a period of three decades. Following admission with severe upper gastrointestinal bleeding, the patient was diagnosed as having invasive pancreatic adenocarcinoma. Examination of the surgical specimen confirmed an intraductal papillary mucinous neoplasm with multiple foci of invasive adenocarcinoma. This case is of significant interest, highlighting the long delay until diagnosis seen in this situation. Intraductal papillary mucinous neoplasm should be considered in the presence of recurrent acute or chronic pancreatitis of unknown aetiology.

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BACKGROUND: Acute pancreatitis (AP) represents a complex and potentially fatal disease with a highly variable clinical course. Three classification systems for assessing the severity in AP have been validated for clinical use. AIMS: The aim of the present study was to evaluate the performance of the Atlanta and Determinant-based classifications in predicting severe clinical outcomes in patients with AP. METHODS: In this retrospective study we reviewed the treatment and follow-up records of 525 patients with AP admitted to our unit between the years of 2003 and 2014. Outcomes included mortality, admission to the ICU, need for interventional procedures or nutritional support, and duration of hospital and ICU stay. RESULTS: The prevalence of organ failure and persistent organ failure in our cohort was 23.0 and 10.7%, respectively, and the mortality rate was 5.9%. Higher grades of severity were associated with worse outcomes in all classification systems. The revised Atlanta and Determinant-based classifications performed similarly in predicting outcomes, and both proved to be superior to the former classic Atlanta classification. CONCLUSION: Recent classifications proved to be more accurate in predicting important clinical outcomes in patients with AP.

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