Narrowing Of Main Pancreatic Duct Research Articles

Clinical characteristics, treatment and outcome of pembrolizumab-induced acute pancreatitis.

Acute pancreatitis (AP) is a rare adverse event of pembrolizumab with unclear clinical features. This study investigated the clinical features of pembrolizumab-induced AP to provide a reference for prevention and treatment. Case reports, case series and clinical studies of pembrolizumab-induced AP were collected by searching Chinese and English databases up to January 31, 2024. Thirty-one patients were included, with a median age of 59 years (range 39, 82). The median time from administration to onset of AP was 5.05 months (range 0.5, 16) and the median cycle was 7 cycles (range 1, 35). Twenty-two (71.0%) patients had elevated pancreatic amylase with a median value of 860 IU/L (range 105-12562), and 16 (51.6%) patients had elevated lipase with a median value of 282 IU/L (range 153-1034). Pancreatic biopsy showed neutrophil infiltration (9.7%) and lymphocyte infiltration (6.5%). Immunohistochemical staining showed CD8 dominated inflammatory infiltration (6.5%). The computed tomography showed diffuse enlargement (51.6%) and focal enlargement (51.6%) of the pancreas. Endoscopic ultrasound showed enlarged hypoechoic pancreas(16.1%). PET/CT showed increased FDG uptake (16.1%). The magnetic resonance cholangial pancreatography showed narrowing of main pancreatic duct (12.9%). AP symptoms and pancreatic enzymes improved after discontinuation of pembrolizumab and administration of steroids and infliximab. Clinicians should be aware that AP is a rare adverse reaction to pembrolizumab. Pembrolizumab induced AP can be initiated with steroids for control, and infliximab can be initiated with steroid-refractory AP.

Cholangiocarcinoma Masquerading as IgG4-related Sclerosing Cholangitis

IgG4-related sclerosing cholangitis (IgG4-SC) represents a rare but clinically challenging differential diagnosis in patients with biliary strictures which can be mistaken for cholangiocarcinoma. We present a case of a 73-year-old male presented with abdominal discomfort and weight loss. Biliary images showed long-segment luminal narrowing of extrahepatic bile duct associated with prominent enhanced wall thickening, but luminal patency was preserved. Pancreatic images revealed segmental irregular narrowing of main pancreatic duct without upstream duct dilatation. His liver function tests and CA19-9 level were normal. Putting all findings together, IgG4-SC associated with autoimmune pancreatitis was strongly suspected. However, endobiliary biopsy of extrahepatic bile duct revealed adenocarcinoma which was not resectable due to celiac axis involvement. Because there is an overlap in biliary imaging findings between IgG4-SC and cholangiocarcinoma, biopsy is essential for adequate differential diagnosis. We present a case of cholangiocarcinoma masquerading as IgG4-SC based on clinical and imaging findings.

Pancreatic Lymphoma Masquerading as Autoimmune Pancreatitis

최근 우리나라에서는 자가면역췌장염의 ì¦ë¡€ë³´ê³ ê°€ ëŠ˜ê³ ìžˆë‹¤. 자가면역췌장염의 ì •í™•í•œ 진단은 ìž„ìƒì ìœ¼ë¡œ 매우 중요한데, 이는 ìž„ìƒì ìœ¼ë¡œ ê·¸ë¦¬ê³ ì˜ìƒí•™ì ìœ¼ë¡œ ì·Œìž¥ì• ì„±ì¢ ì–‘ê³¼ì˜ 감별이 ì–´ë µê¸° 때문이다. 자가면역췌장염의 경우, ìŠ¤í Œë¡œì´ë“œì— 대하여 반응이 ì¢‹ê³ , ìˆ˜ìˆ ì  치료를 í”¼í• ìˆ˜ 있다는 ì ì—ì„œ ê·¸ 의의가 크다. 이번 ì¦ë¡€ë³´ê³ ì—ì„œ ì €ìžë“¤ì€ 영상검사에서 자가면역췌장염을 충분히 ì˜ì‹¬í• ë§Œí•œ 환자에서 생검상 ë¦¼í”„ì¢ ìœ¼ë¡œ 확진된 증례를 경험하였기에, 문헌 ê³ ì°°ê³¼ 함께 ë³´ê³ í•˜ëŠ” 바이다. Keywords: 자가면역췌장염, 말초Tì„¸í¬ë¦¼í”„ì¢ , 췌장 ì‹ ìƒë¬¼, 폐쇄성 췌장염, 급성 췌장염

Endoscopic approaches for the diagnosis of autoimmune pancreatitis.

Autoimmune pancreatitis (AIP) is characterized by diffuse pancreatic enlargement and irregular narrowing of the main pancreatic duct (MPD). Immunoglobulin (Ig)G4-related sclerosing cholangitis (IgG4-SC) associated with AIP frequently appears as a bile duct stricture. Therefore, it is important to differentiate AIP and IgG4-SC from pancreatic cancer and cholangiocarcinoma or primary sclerosing cholangitis, respectively. Endoscopy plays a central role in the diagnosis of AIP and IgG4-SC because it provides imaging of the MPD and bile duct strictures as well as the ability to obtain tissue samples for histological evaluations. Diffuse irregular narrowing of MPD on endoscopic retrograde cholangiopancreatography (ERCP) is rather specific to AIP, but localized narrowing of the MPD is often difficult to differentiate from MPD stenosis caused by pancreatic cancer. A long stricture (>1/3 the length of the MPD) and lack of upstream dilatation from the stricture (<5 mm) might be key features of AIP on ERCP. Some cholangiographic features, such as segmental strictures, stric tures of the lower bile duct, and long strictures with prestenotic dilatation, are more common in IgG4-SC than in cholangiocarcinoma. Endoscopic ultrasonography (EUS) reveals diffuse hypoechoic pancreatic enlargement, sometimes with hypoechoic inclusions, in patients with AIP. In addition, EUS-elastography and contrast-enhanced harmonic EUS have been developed with promising results. The usefulness of EUS-guided fine-needle aspiration has been increasingly recognized for obtaining adequate tissue samples for the histological diagnosis of AIP. Further improvement of endoscopic procedures and devices will contribute to more accurate diagnosis of AIP and IgG4-SC.

Role of EUS in Diagnosis of Autoimmune Pancreatitis

Context The diagnosis of autoimmune pancreatitis (AIP) is based on a combination of pancreatic histology, imaging, serology, other organ involvement, and response to steroids. In this setting, endoscopic ultrasound (EUS) is considered the procedure of choice to obtain pancreatic tissue in order to establish the diagnosis and/or to exclude malignancy. However, negative cytology or histology obtained by EUS-FNA does not exclude AIP. In order to further investigate these subgroups of patients, there are two promising imaging tools: real-time EUS elastography of the pancreas and contrast-enhanced EUS. Objective and methods To evaluate the diagnostic yield of EUS in detection of AIP by comparing EUS findings with standard radiological imaging (CT and/or MRI) in patients with suspected autoimmune disease referred to our tertiary center. Results Thirty-two patients with radiological suspicious of AIP underwent EUS. Focal enlargement of pancreatic parenchyma and focal narrowing of main pancreatic duct (MPD) were detected in the same way by MRI and EUS, being both techniques better that CT (12/21, 12/32, and 7/19, respectively). Diffuse pancreatic parenchyma enlargement and detection of multiple stricture of MPD were better identified by EUS (8/19 with CT, 3/21 with RMN, 12/32 with EUS; 1/19 with CT, 1/21 with RMN, 6/32 with EUS, respectively). Focal and diffuse AIP presented hypervascularization pattern after contrast agent injection in the majority of patients (10/13). At elastography, focal AIP presented with a pattern of small spotted mainly blue colour signals that are evenly spread, showing a different pattern from pancreatic cancer. In addition, when targeted masses were unclear on fundamental B-mode EUS imaging, elastography was useful to find the targeted area to biopsy. Conclusion We believe that EUS elastography and contrast-enhanced EUS alone or in combination are promising tools in the diagnostic approach of focal AIP. It seems that in the near future their use might be integrated in the diagnostic algorithms of AIP.

The differentiation of autoimmune pancreatitis and pancreatic cancer using imaging findings.

Differentiation of autoimmune pancreatitis (AIP) and pancreatic cancer (PC) is important to avoid unnecessary surgery. The aim of this study was to compare various image findings and facilitate the differentiation of these two diseases. The radiological features of 36 AIP patients and 36 patients with resected PC diagnosed at Samsung Medical Center from January 1991 to October 2010, were compared. Regarding CT/MRI findings, diffuse pancreas enlargement, capsule-like rim and delayed homogenous enhancement, were significantly more frequent in AIP. For cholangiopancreatography findings, main pancreatic duct (MPD) narrowing by ≥1/3 of the pancreatic length, skipped lesions of the MPD, the presence of side branches at the narrowed MPD portion, and smooth and straight intrapancreatic common bile duct stenosis were significantly more frequent in AIP. However, according to FDG-PET findings, SUVmax, uptake shape and pattern, and uptake by extrapancreatic lesions were not significantly different for AIP and PC. Diffuse pancreas enlargement, a capsule-like rim, delayed homogenous enhancement, MPD narrowing of ≥1/3 of the pancreatic length, skipped lesions and the presence of side branches at the narrow MPD portion were found to have high specificity for AIP. These findings have great power to differentiate AIP and PC.

Mis-diagnosis and mis-treatment of autoimmune pancreatitis: a clinical study of 17 cases

Objective To summarize the reasons of mis-diagnosis and mis-treatment of autoimmune pancreatitis (AIP). Methods Clinical data of 17 patients with AIP,who were admitted to the hospital from May 2005 to July 2010 and experienced mis-diagnosis and mis-treatment, were retrospectively analyzed. Results The main clinical manifestations included epigastric pain (13 cases),progressive obstructive jaundice (12 cases), fever (6 cases) and weight loss (9 cases). Fifteen patients had extrapancreatic organ involvemnet, including allergic rhinitis, swelling of lymphoglandulae submaxillares, swelling of submaxillary gland, allergic asthma, rheumatoid arthritis, Sjogren syndrome, diabetes mellitus, primary sclerosing cholangitis and autoimmune hepatitis. Of these 17 cases, 11 cases presented with high serum globulin, 14 cases with high serum IgG, 13 cases with high serum γ-globulin, 13 cases with positive anti-nuclear antibody and 2 cases with positive anti-insulin IgG antibody. The abdominal imaging demonstrated that 15 patients had diffuse enlargement of the pancreas with diffuse or segmental narrowing of main pancreatic duct, narrowing of the intrapancreatic common bile duct, dilation of the proximal biliary duct and gallbladder enlargement. Focal enlargement of the pancreas was found in 2 cases. Thirteen cases were misdiagnosed as pancreatic carcinoma. Among them, 4 cases underwent pancreaticoduodenectomy and 7 cases underwent choledochojejunostomy. Two cases were misdiagnosed as end stage of cancer that lost therapeutic chance. Another 4 cases were misdiagnosed as chronic pancreatitis. Steroid therapy was administered in all patients with satisfactory response. All patients were followed-up for 15 months (ranged from 6 months to 45 months), and recurrence was found in 4 cases. Satisfactory response was found in patients treated with steroid for the second time. No pancreatic cancer was found in these patients in the follow up period. Conclusion The main causes of mis-diagnosis and mis-treatment of AIP may be contributed by difficulty in differentiating AIP from pancreatic carcinoma based on clinical manifestations and inadequate knowledge of AIP as well as insufficient attention to AIP in China. Key words: Pancreatitis; Autoimmune diseases; Diagnostic errors; Therapeutic errors

Magnetic Resonance Cholangiopancreatography for the Diagnostic Evaluation of Autoimmune Pancreatitis

The aim of this study was to determine the role of magnetic resonance cholangiopancreatography (MRCP) for diagnosing autoimmune pancreatitis (AIP) and the accuracy of MRCP in depicting the main pancreatic duct (MPD) morphology of AIP using endoscopic retrograde cholangiopancreatography (ERCP) as the reference standard. Thirty-eight AIP patients, 40 pancreatic cancer patients, and 40 patients with normal pancreas were included. MRCP was interpreted in association with cross-sectional magnetic resonance images regarding MPD morphology, pancreatic parenchyma, and extrapancreatic abnormalities. Main pancreatic duct was interpreted as narrowed when a narrowed-appearing segment on MRCP was associated with upstream dilatation or pancreatic parenchymal abnormalities in the same location. Accuracy of MRCP for depicting MPD morphology of AIP (64.7% [22/34]) was lower than those for pancreatic cancer (88.5% [23/26]) (P = 0.041) or normal pancreas (100% [40/40]) (P < 0.0005). The inaccuracy in AIP was primarily (10/12) due to overestimation of MPD narrowing. Of various differing MRCP findings between AIP and pancreatic cancer, multiple MPD narrowing (AIP vs cancer, 27/38 vs 0/40) and upstream MPD dilatation greater than 5 mm in diameter (AIP vs cancer, 0/38 vs 10/40) could exclude pancreatic cancer and AIP, respectively. MRCP cannot replace ERCP for the diagnostic evaluation of AIP but may deserve to be used when ERCP has been unsuccessful or is difficult to perform.

Autoimmune pancreatitis: imaging findings on contrast-enhanced MR, MRCP and dynamic secretin-enhanced MRCP

This study retrospectively determined magnetic resonance (MR), MR cholangiopancreatography (MRCP) and secretin-MRCP findings in patients with autoimmune pancreatitis (AIP). The MR examinations of 28 patients with histopathologically proven AIP were reviewed. In 14 cases, secretin-enhanced MRCP was performed. The observers evaluated pancreatic parenchymal enlargement, signal intensity abnormalities, enhancement, vascular involvement, bile-duct diameter and main pancreatic duct (MPD) narrowing (diffuse/focal/segmental). After secretin administration, the presence of the "duct-penetrating" sign was evaluated. MR imaging showed diffuse pancreatic enlargement in 8/28(29%) cases, focal pancreatic enlargement in 16/28 (57%) cases and no enlargement in 4/28 (14%) cases. The alteration of pancreatic signal intensity was diffuse in 8/28 (29%) cases (eight diffuse AIP) and focal in 20/28 (71%) cases (20 focal AIP). Delayed pancreatic enhancement was present in all AIP, with peripheral rim of enhancement in 8/28 (29%) AIP (1/8 diffuse, 7/20 focal); vascular encasement was present in 7/28 (25%) AIP (1/8 diffuse, 6/20 focal); distal common bile duct narrowing was present in 12/28(43%) AIP (5/8 diffuse, 7/20 focal). MRCP showed MPD narrowing in 17/28 (61%) AIP (4/8 diffuse, 15/20 focal), MPD dilation in 8/28(29%) AIP (3/8 diffuse, 5/20 focal) and normal MPD in 1/8 diffuse AIP. Secretin-MRCP showed the duct-penetrating sign in 6/14(43%) AIP (one diffuse AIP with MPD segmental narrowing, five focal AIP with MPD focal narrowing), demonstrating integrity of the MPD. Delayed enhancement and MPD stenosis are suggestive for AIP on MR and MRCP imaging. Secretin-enhanced MRCP is a problem-solving tool in the differential diagnosis between focal AIP and ductal adenocarcinoma.

Autoimmune Chronic Pancreatitis

Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis that is characterized by swelling of the pancreas, narrowing of the main pancreatic duct (MPD), elevation of serum gamma globulin, or immunoglobulin G or presence of autoantibody, lymphoplasmacytic infiltration and dense fibrosis on histopathology. It is responsive to steroid therapy. The incidence of AIP can reach 5-6% of chronic pancreatitis. It can present as obstructive jaundice, body weight loss, and pancreas head mass mimicking pancreatic cancer. The recognition of AIP can avoid major surgery such as pancreatic resection. From May 2003 to July 2007, a total of 5 cases of AIP were reviewed retrospectively. The diagnosis was made on imaging study, serology, steroid response and/or histology if surgery was carried out. There were 2 male and 3 female patients, with a mean age of 61 (39-75) years. Atypical AIP was found in the first case and typical AIP in the remaining 4. The presenting clinical pictures were mild epigastric pain, obstructive jaundice, and loss of body weight in 4 cases, with associated autoimmune disease in 1. Diffuse or long segmental enlargement of the pancreas without peripancreatic fat infiltration was found in all patients except 1 who only had focal pancreatic head enlargement. Distal common bile duct (CBD) stricture was seen in 4 cases and the median CBD stricture length was 1.2 (0.5-2.5) cm. Multiple narrowing of the whole MPD was seen in 2 cases, focal narrowing of the MPD in 2 and long segmental narrowing of the MPD in 1. Serum immunoglobulin G tests were done in 4 cases and were elevated in all. Antinuclear antibody was positive in 3. The first case was operated on after a preoperative diagnosis of suspicious pancreatic head tumor. The subsequent 3 cases were diagnosed correctly as AIP. The last case presented with distal CBD stricture and hypoechoic lesion in the pancreas head on endoscopic ultrasound, with only borderline pancreatic enlargement on computed tomography, and he was operated on. Retrospective endoscopic retrograde pancreatogram review revealed MPD narrowing in the pancreatic body. Endoscopic retrograde brush cytology was performed and was negative for malignancy in 3 cases. Steroid therapy was given in 3 and was responsive, but there were 2 recurrences. AIP should be a differential diagnosis in distal CBD stricture and pancreatic head mass when the patient has: (1) diffuse or long segmental enlargement of the pancreas without peripancreatic fat infiltration, with multiple narrowing of the MPD without much upstream dilatation, or narrowing of the MPD not corresponding to the region of CBD stricture; and (2) abnormal immunoserologic tests.

Autoimmune Dorsal Pancreatitis: Involvement of the Pancreatic and Bile Ducts in Autoimmune Pancreatitis

Background and Aim: Typical cases of autoimmune pancreatitis (AIP) show a diffusely enlarged pancreas with a sausage-like appearance and diffuse irregular narrowing of the entire main pancreatic duct. A focal type of AIP, showing focal swelling with localized narrowing of the main pancreatic duct and upstream dilatation, is regarded as a variant of AIP. Stenosis of the bile duct frequently occurs with AIP. We examined the involvement of the pancreatic and bile ducts in patients with AIP, and proposed a new term of autoimmune dorsal pancreatitis. Patients and Methods: Clinical and cholangiopancreatographic findings of 32 patients (26 men and 6 women, average age 68.4) with AIP were evaluated. For the purposes of this study, the pancreatic duct system was divided into 3 portions: the ventral pancreatic duct, the head portion of the dorsal pancreatic duct, and the body and tail of the dorsal pancreatic duct. Results: Both the ventral and dorsal pancreatic ducts were involved in 27 patients, while in 5 patients only the dorsal pancreatic duct was involved. Segmental narrowing of the head of the dorsal pancreatic duct with normal ventral pancreatic duct was seen in 2 patients, and the other 3 patients showed segmental narrowing of the body and tail of the dorsal pancreatic duct. Marked stricture of the bile duct was detected in 23 patients and their initial symptom was obstructive jaundice. Six patients showed moderate stenosis to 30%-40% of the normal diameter, and the other 3 patients showed no stenosis of the bile duct. Although marked stricture of the bile duct was detected in 23 of the 27 patients (85%) who showed narrowing of both the ventral and dorsal pancreatic ducts, it was not observed in the 5 patients who showed only involvement of the dorsal pancreatic duct (P = 0.0006). Conclusion: Both the ventral and dorsal pancreatic ducts were involved in many patients with AIP, while in 5 patients only the dorsal pancreatic duct was involved. The pancreas develops by fusion of the dorsal and ventral pancreatic primordial buds. Although the pathogenesis of AIP is unclear, AIP might have occurred only in the dorsal pancreas in these 5 patients. We would propose a new term of autoimmune dorsal pancreatitis. Although obstructive jaundice due to stenosis of the bile duct commonly occurs with AIP, in this type of the disease, anatomic relationships between the dorsal pancreas and common bile duct suggest that obstructive jaundice rarely occurs, even if the pancreatic head is involved.

Autoimmune pancreatitis: Functional and morphological recovery after steroid therapy

Autoimmune pancreatitis, a recently recognized type of chronic pancreatitis, is not rare in Japan, but reports of it elsewhere are relatively uncommon. We report the first preoperatively diagnosed case of autoimmune pancreatitis in Hungary, which responded well to steroid treatment and provided radiographic and functional evidence of this improvement. A 62-year-old female presented with a 4-month history of recurrent epigastric pain and a 5-kg weight loss. The oral glucose tolerance test (OGTT) indicated diabetes mellitus and the result of the fecal elastase test was abnormal. Ultrasonography (US) and the CT scan demonstrated a diffusely enlarged pancreas, and endoscopic retrograde cholangiopancreatography (ERCP) an irregular main pancreatic duct with long strictures in the head and tail. Autoimmune pancreatitis was diagnosed. The patient was started on 32 mg prednisolone daily. After 4 wk, the OGTT and faecal elastase test results had normalized. The repeated US and CT scan revealed a marked improvement of the diffuse pancreatic swelling, while on repeated ERCP, the main pancreatic duct narrowing was seen to be ameliorated. It is important to be aware of this disease and its diagnosis, because AIP can clinically resemble pancreatobiliary malignancies, or chronic or acute pancreatitis. However, in contrast with chronic pancreatitis, its symptoms and morphologic and laboratory alterations are completely reversed by oral steroid therapy.

A case of pouchitis, duodenitis and pancreatitis showing diffuse irregular narrowing of main pancreatic duct after total colectomy for ulcerative colitis

A case of pouchitis, duodenitis and pancreatitis showing diffuse irregular narrowing of main pancreatic duct after total colectomy for ulcerative colitis

A CASE OF NEURINOMA OF THE PANCREAS IN A PATIENT WITH PRIMARY ALDOSTERONISM

Neurinoma of the pancreas is very rare lesion with only 11 cases having been reported to date in Japan. Here we report one such case that was found in a patient with primary aldosteronism. A 37-year-old woman was admitted to our hospital because of hypertension and hypokalemia along with an increased blood concentration of aldosterone (34ng/dl). The patient was definitely diagnosed as having primary aldosteronism due to a left adrenal adenoma by hormonal examinations and radiological imaging tests. During the work-up a mass lesion measuring 3cm in diameter on ultrasonography was incidentally detected in the pancreas haed. Computed tomography of he abdomen showed the tumor being located in the dorsal portion of the pancreas head. Celiac arteriography demonstrated a neovascularity of the tumor, though neigher irregularity of the vessels wall or tumor staining in the capillary phase was seen. Endoscopic retrograde pancreatography showed a mild degree of narrowing of main pancreatic duct in the pancreas head. No hormonal abnormalities other than then hyperaldosteronism were found. The patient underwent pancreaticoduodenectomy along with left adrenectomy under the preoperative diagnosis of primary aldosteronism and a non-functioning endocrine tumor in the pancreas head. Microscopic examination confirmed that the tumor was neurinoma arising form the pancreas head.