Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organ...Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organs of AIP patients, AIP appears to be a pancreatic lesion reflecting a systemic "IgG4-related sclerosing disease". Clinically, AIP patients and patients with pancreatic cancer share many features, such as preponderance of elderly males, frequent initial symptom of painless jaundice, development of new-onset diabetes mellitus, and elevated levels of serum tumor markers. It is of uppermost importance not to misdiagnose AIP as pancreatic cancer. Since there is currently no diagnostic serological marker for AIP, and approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological, and histopathological features. Findings suggesting AIP rather than pancreatic cancer include:fluctuating obstructive jaundice; elevated serum IgG4 levels; diffuse enlargement of the pancreas; delayed en- hancement of the enlarged pancreas and presence of a capsule-like rim on dynamic computed tomography; low apparent diffusion coefficient values on diffusion-weighted magnetic resonance image; irregular narrowing of the main pancreatic duct on endoscopic retrograde cholangiopancreatography; less upstream dilatation of the main pancreatic duct on magnetic resonance cholangiopancreatography, presence of other organ involvement such as bilateral salivary gland swelling, retroperitoneal fibrosis and hilar or intrahepatic sclerosing cholangitis; negative work-up for malignancy including endoscopic ultrasound-guided fine needle aspiration; and steroid responsiveness. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection.展开更多
Pancreatic cancer is an aggressive and lethal disease that affects especially older population. Its more relevant tumor marker is CA 19-9 (carbohydrate antigen 19-9), although it can be elevated in others clinical s...Pancreatic cancer is an aggressive and lethal disease that affects especially older population. Its more relevant tumor marker is CA 19-9 (carbohydrate antigen 19-9), although it can be elevated in others clinical situations, like cholangitis and cholestasis. Otherwise, a small people subset, like our patient, do not produce this tumor marker, as on blood as in the tumor, because they are incapable to express the Lewis Antigen. Therefore, this case report is about a patient without Lewis Antigen express and CA 19-9 low levels. We will report a rapid disease progression, despite of low CA 19-9, comparing with available data that often show better prognosis in this setting. Conclusion: Low levels of CA 19-9 do not predict good response or better prognosis in patients that do not express Lewis Antigen.展开更多
文摘Autoimmune pancreatitis (AIP) is a newly described entity of pancreatitis in which the pathogenesis appears to involve autoimmune mechanisms. Based on histological and immunohistochemical examinations of various organs of AIP patients, AIP appears to be a pancreatic lesion reflecting a systemic "IgG4-related sclerosing disease". Clinically, AIP patients and patients with pancreatic cancer share many features, such as preponderance of elderly males, frequent initial symptom of painless jaundice, development of new-onset diabetes mellitus, and elevated levels of serum tumor markers. It is of uppermost importance not to misdiagnose AIP as pancreatic cancer. Since there is currently no diagnostic serological marker for AIP, and approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological, and histopathological features. Findings suggesting AIP rather than pancreatic cancer include:fluctuating obstructive jaundice; elevated serum IgG4 levels; diffuse enlargement of the pancreas; delayed en- hancement of the enlarged pancreas and presence of a capsule-like rim on dynamic computed tomography; low apparent diffusion coefficient values on diffusion-weighted magnetic resonance image; irregular narrowing of the main pancreatic duct on endoscopic retrograde cholangiopancreatography; less upstream dilatation of the main pancreatic duct on magnetic resonance cholangiopancreatography, presence of other organ involvement such as bilateral salivary gland swelling, retroperitoneal fibrosis and hilar or intrahepatic sclerosing cholangitis; negative work-up for malignancy including endoscopic ultrasound-guided fine needle aspiration; and steroid responsiveness. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection.
文摘Pancreatic cancer is an aggressive and lethal disease that affects especially older population. Its more relevant tumor marker is CA 19-9 (carbohydrate antigen 19-9), although it can be elevated in others clinical situations, like cholangitis and cholestasis. Otherwise, a small people subset, like our patient, do not produce this tumor marker, as on blood as in the tumor, because they are incapable to express the Lewis Antigen. Therefore, this case report is about a patient without Lewis Antigen express and CA 19-9 low levels. We will report a rapid disease progression, despite of low CA 19-9, comparing with available data that often show better prognosis in this setting. Conclusion: Low levels of CA 19-9 do not predict good response or better prognosis in patients that do not express Lewis Antigen.