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• W1600171623 abstract "Immunoglobulin G4 (IgG4)-related systematic disease (IgG4-RSD) is a systemic fibroinflammatory condition characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs including pancreas, bile duct, gallbladder, salivary glands, kidney, lung, prostate, retroperitoneum and even the thyroid gland. It may affect one or two and sometimes more than three organs. Early diagnosis and treatment could not prevent sclerosis and permanent damage of the affected organ but also avoid other organ involvement. Here we present a rare case of IgG4-RSD affecting pancreas, biliary duct system and abdominal aorta. A review of the literature was also made mainly concerning IgG4-related sclerosing cholangitis (IgG4-SC) and periaortitis. A 45-year-old man complaining of painless jaundice and weight loss was admitted to the Department of Gastroenterology of the local hospital in June 2007. Initial laboratory results revealed cholestasis with normal serum carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA). Computed tomography (CT) showed significant enlargement of the pancreatic head and upstream dilatation of the main pancreatic duct (MPD) with a capsule-like low-density rim surrounding the whole pancreas, and also wall thickening and enhancement of the gallbladder and bile duct (Fig. 1). The patient was suspected as pancreatic cancer, and an exploratory surgery was performed. The surgery revealed dilatation of the extrahepatic bile duct with marked concentric wall thickening and stiffness of the bile duct and gallbladder, as well as the enlargement and stiffness of pancreatic head that encased the superior mesenteric vessels. Only cholecystectomy was performed and pathological analysis showed chronic transmural inflammation. The patient's jaundice resolved spontaneously after surgery. Initial computed tomography (June 2007) shows (a) significant enlargement of the pancreatic head with a capsule-like low-density rim and (b) wall thickening and enhancement of the gall bladder and bile duct. By May 2008, the patient was admitted to the Outpatient Center of Peking Union Medical College Hospital because of the recurrent jaundice. Laboratory examinations showed alanine aminotransferase (ALT) 99 U/L (normal < 40 U/L), aspartate aminotransferase (AST) 83 U/L (normal < 37 U/L), γ-glutamyltransferase (γ-GT) 556 U/L (normal 10–67 U/L), alkaline phosphatase (ALP) 359 U/L (normal 30–120 U/L), erythrocyte sedimentation rate (ESR) 51 mm/h (normal 0–15 mm/h), immunoglobulin G (IgG) 32.6 g/L (normal 7–17 g/L). Bilirubin levels, serum autoimmune antibodies and tumor markers were normal. Abdominal CT with three-dimensional (3-D) reconstruction (Fig. 2) showed (i) stenosis of the distal common bile duct (CBD) with diffusely distributed dilatation of the intrahepatic and extrahepatic bile duct; (ii) significant wall thickening of the bile duct with its inner wall smooth and greatly enhanced; (iii) similar pancreatic appearance as seen in the initial scan; (iv) ring-form wall thickening of the abdominal aorta. The abnormities were confirmed by endoscopic ultrasonography (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). Malignancy was excluded by wire-guided brushing cytology of the bile duct. Transampullary balloon dilation and CBD stent placement were performed during the ERCP. The patient was then diagnosed as autoimmune pancreatitis (AIP). Steroid therapy began with prednisolone 30 mg daily, and was tapered gradually and totally withdrawn 9 months later. Follow-up examinations including liver function tests, ESR, IgG and CT scan showed significant improvement. Abdominal computed tomography with three-dimensional reconstruction of the pancreas and the biliary duct system (May 2008) shows (a) stenosis in the distal common bile duct with dilatation of proximal bile ducts wall, thickening and inner wall enhancement of the bile ducts; (b) enlargement of pancreatic head and body with dilated main pancreatic duct; and (c) ring-form wall thickening of the abdominal aorta. However, the patient experienced recurrence in August 2010. Examinations revealed ALT 466 U/L, AST 369 U/L, γ-GT 1089 U/L, ALP 661 U/L, total bilirubin (TB) 75 µmol/L (normal 5.1–22.2 µmol/L), direct bilirubin (DB) 50.2 µmol/L (normal 0–8.6 µmol/L), ESR 49 mm/h, IgG 29 g/L, IgG4 78.59 g/L (tested by dilution, normal 0.01–2.91 g/L). Abdominal CT (Fig. 3) showed additional abnormities compared with the former one: (i) multiple stenosis of the bile duct; (ii) stricture of the splenic vein with low-density soft tissue around. The diagnosis of IgG4-SC with AIP and periaortitis was made and the patient was treated with steroids and a CBD stent. Four months after the treatment, liver function test, IgG and ESR returned to normal range with a slightly elevated IgG4 level. Repeated abdominal CT (Fig. 4) also showed improvement. Abdominal computed tomography with three-dimensional reconstruction of the biliary duct system (August 2010) shows (a) multistenosis of the right intrahepatic duct, distal common bile duct and common hepatic duct and (b) stricture of the splenic vein. Comparison of abdominal computed tomography shows (a) significant thickening and inner wall enhancement of the bile duct and capsule-like rim around the pancreas before treatment and (b) four months after stent placement and steroid therapy, the above abnormities significantly improved. AIP, first proposed by Yoshida et al.1 in 1995, is a unique form of chronic pancreatitis with a probable autoimmune etiology. As AIP has gradually been identified worldwide in recent years, different diagnostic criteria have been proposed by experts from Japan, Korea, the USA and Italy. The characteristics of AIP can be described as follows: (i) a male predominance with elder age; (ii) painless obstructive jaundice is the main presenting symptom; (iii) occasional association with various extrapancreatic lesions; (iv) typical radiological findings of diffuse enlargement of the pancreas with irregular narrowing of MPD; (v) elevated serum IgG or IgG4; (vi) histological findings of dense infiltration of T-lymphocytes and IgG4-positive plasma cells in the pancreas with fibrosis and obliterative phlebitis; (vii) remission or marked improvement of pancreatic and extrapancreatic manifestations with steroid therapy. The diagnosis of AIP is not difficult for cases with typical imaging findings. But for those with an atypical appearance such as focal enlargement of the pancreas, pancreatic cancer must be carefully excluded to avoid unnecessary surgery. It is reported that up to one-third of the AIP cases are diagnosed after the surgery for a suspected malignancy.2 Lack of awareness of the new entity is one of the reasons. Two strategies have been published, one from Japan3 and the other from the USA,4 both agree that the presence of elevated serum IgG4 is highly suggestive of AIP. The Japanese strategy is simpler for clinical practise because it placed more emphasis on CT and endoscopic retrograde pancreatography (ERP) findings. CT findings including enhancement of the enlarged pancreas, capsule-like rim and presence of extrapancreatic lesions, and ERP findings including a more than 3 cm narrowed MPD, skipped lesions of MPD and maximal upstream MPD diameter of less than 5 mm were defined as being suggestive of AIP rather than pancreatic cancer. The American strategy recommended a core biopsy of the pancreas, steroid trial or surgical resection as the final choice if a definite diagnosis cannot be reached by clinical, serological and radiological findings. As for this patient, AIP could be definitely diagnosed with clinical, radiological, serological findings and responsiveness to steroids. When the patient's abdominal CT serial was retrospectively analyzed, gallbladder and extrahepatic bile duct were found to be involved initially and progressed gradually. Under the background of markedly elevated serum IgG4 and definite diagnosis of AIP, IgG4-SC was highly suspected. IgG4-SC is included in IgG4-RSD and is frequently associated with AIP, with 92% of cases accompanied by AIP.5 The cholangiographic appearance of IgG4-SC is quite similar to that of primary sclerosing cholangitis (PSC), but unlike PSC it responds dramatically to steroid therapy and has a better prognosis. HISORt criteria for IgG4-SC proposed by Ghazale et al.5 include (i) histology: lymphoplasmacytic infiltration of the bile duct with extensive transmural fibrosis and IgG4 immunostaining showing infiltration of abundant IgG4-positive plasma cells in the bile duct wall [>10/high-power field (HPF)]; (ii) imaging: stricture(s) of intrahepatic, proximal extrahepatic or intrapancreatic ducts; (iii) serology: elevated serum IgG4 level; (iv) other organ involvement: frequently associated with AIP and occasionally with other sclerosing diseases such as sclerosing cholecystitis, sclerosing sialadenitis, sclerosing dacryoadenitis and retroperitoneal fibrosis; (v) response to steroid therapy: dramatic response to steroid treatment, and after 4-week steroid therapy markedly improved biliary strictures can allow stent removal, liver enzymes < twice the upper limit of normal and decrease of serum IgG4 and CA19-9. According to the HISORt criteria, the present patient could be diagnosed definitely as IgG4-SC. Currently no consensus has been reached on the dosage and duration of steroid trial for IgG4-SC. Since misdiagnosis and wrong therapy may lead to severe consequences, only short-term steroid trial should be performed and it must be monitored carefully by specialists. It is important to keep in mind that not all the IgG4-SC patients respond to steroid therapy. Advanced-stage IgG4-SC may have a poor response to steroid therapy because of its irreversible fibrosis.6 In patients with suspected diagnosis of IgG4-SC, PSC and cholangiocarcinoma must be excluded because the treatment and prognosis of them are quite different. The distinctive points between IgG4-SC and PSC include: (i) age of clinical onset: while most PSC patients present the symptoms at the age of 25–45 years, IgG4-SC patients are usually elder than 65 years; (ii) associated diseases: concomitant inflammatory bowel disease (IBD) occurs in approximately 62.5–90% of PSC patients, but only 0–6% of IgG4-SC patients;5, 7 and while 10–30% of PSC develop into cholangiocarcinoma, to date no case of cholangiocarcinoma as the complication of IgG4-SC has been reported.8 On the contrary, IgG4-SC is usually accompanied by other IgG4-related diseases; (iii) serum IgG4: elevated serum IgG4 level occurs in most IgG4-SC patients, but only 9% of PSC patients;9 (iv) cholangiographic features: long segmental strictures and strictures of the distal CBD were more common in IgG4-SC while band-like strictures, beaded or a pruned-tree appearance and diverticulum-like outpouching were more common in classic PSC.10, 11 Nakazawa et al.12 classified IgG4-SC into four types based on the region of strictures revealed by cholangiography. The cholangiographic findings of type 2 (stenosis throughout the intrahepatic and extrahepatic bile ducts) are sometimes misdiagnosed as PSC, and the other types as cholangiocarcinoma; (v) pathological findings13-15: the characteristic pathological findings are dense infiltration of lymphoplasmacytes and IgG4-positive plasma cells with extensive fibrosis and obliterative phlebitis in the bile duct wall and periportal area of the liver, whereas that of PSC is a fibro-obliterative process that may lead to an onion skin appearance of concentric fibrosis surrounding the medium-sized bile ducts; (vi) response to steroid therapy: IgG4-SC shows a good response to steroid therapy; however, except for autoimmune hepatitis (AIH)-PSC overlap syndrome, typical PSC does not respond to steroid or any other immunosuppressive agent. It is also difficult but important to discriminate between IgG4-SC and cholangiocarcinoma. Cholangiography and cross-sectional imaging with CT or magnetic resonance imaging (MRI) may be insufficiently sensitive for the detection of cholangiocarcinoma. In patients suspected of cholangiocarcinoma, ERCP with intraductal ultrasonography (IDUS), brush cytology and endobiliary biopsy would be helpful. But given the superficial nature of brush cytology and endoscopic biopsy, their diagnostic sensitivity are not high. Naitoh et al.16 reported that IDUS is a useful tool for differentiating IgG4-SC from cholangiocarcinoma. Bile duct wall thickness of more than 0.8 mm in areas without stenosis was regarded as the most characteristic IDUS finding of IgG4-SC. The current treatment regimen of IgG4-SC is mainly derived from the treatment experiences of AIP. Until now, there have been no published randomized controlled studies on the treatment of either AIP or IgG4-SC. A treatment approach for IgG4-SC proposed in 2008 by Björnsson17 has suggested corticosteroids as the first-line therapy with an initial dosage of prednisolone 40 mg daily. After 4 weeks the prednisolone can be tapered down over a period of 3–6 months by 5 mg/week. Steroids can be discontinued in patients with a good clinical and biochemical response. For those with a suboptimal response, the placement of a biliary stent during ERCP is suggested to hasten symptomatic improvement. However, the relapsing rate of AIP during or after steroid therapy is reported to be as high as 40–53%5, 18 and most patients who relapsed had IgG4-SC, especially those with proximal extrahepatic or intrahepatic ductal strictures.4 A maintenance therapy with steroid 2.5–5 mg/day for at least 3 years is recommended by the Research Committee for Intractable Pancreatic Disease and Japan Pancreas Society,18 which can effectively reduce the relapsing rate of AIP from 54% to 26%. For most relapsed AIP cases, the re-administration or a dose-up of steroids is still effective. Apart from steroids, immunosuppressive agents such as azathioprine or mycophenolate mofetil have also been administered as steroid-sparing agents in refractory or recurrent AIP.17 However, related data are few and limited. Indications for these drugs should therefore be assessed carefully. Despite treatment with steroids and immunosuppressive agents, some patients with AIP and IgG4-SC still have incomplete responses, relapse during long-term follow-up, refractory to steroid tapering or experience drug-related adverse effects. Recently, reports from Topazian et al.19 and Khosroshahi et al.20 showed that treatment with rituximab could lead to a prompt clinical and serological improvement in these patients. However, large prospective studies are needed to verify its effectiveness. Our patient had a good response to initial steroid therapy for 9 months, but relapsed 17 months after steroid withdrawal. Re-administration of the steroid seemed to be effective by evaluation of his clinical manifestations, biochemical blood tests and imaging findings. With the experience of relapse after steroid withdrawal, long-term maintenance therapy with a low-dose steroid should be administered, and a longer duration of follow-up is needed to avoid a second relapse. Recent work in the last few years has revealed that some kinds of inflammatory aortic diseases are, in fact, manifestations of IgG4-RSD, namely IgG4-related aortic disease.21 This includes non-infectious thoracic aortitis, inflammatory abdominal aortic aneurysms (IAAA) or abdominal periaortitis and retroperitoneal fibrosis. The cases of IgG4-related IAAA or abdominal periaortitis were first reported in 2008 by Kasashima et al.22 After that, a single-center study consisted of 13 cases from Japan concerning the clinical characteristics of IgG4-related IAAA was published. Compared with non-IgG4-related IAAA and conventional atherosclerotic abdominal aortic aneurysms, IgG4-related IAAA were found to be particularly characterized by the frequency of complications of allergic and autoimmune diseases, low incidence of ruptures and high serum IgG4 and IgE concentration.23 The pathological features of IgG4-related IAAA or abdominal periaortitis are similar to those in other IgG4-RSD, showing an adventitia-prominent form of lymphoplasmacytic infiltrate with a high percentage of IgG4-positive plasma cells. It is important to establish pathological criteria for the diagnosis of IgG4-related aortic diseases, because some patients were reported to have normal serum IgG4 and no clinical evidence of systematic involvement.23, 24 The following three criteria proposed by Stone et al.21 were used to constitute the pathological diagnosis of IgG4-related aortitis/periaortitis: (i) an overall histology that is consistent with aortitis or periaortitis and is not readily explained by another process such as atherosclerosis; (ii) at least 50% of the plasma cells stained for IgG4; (iii) at least 50 IgG4-positive plasma cells/HPF (amplication ×400) counting at least three fields. Further efforts should be made to evaluate the sensitivity and specificity of these criteria. Since IgG4-RSD may present as aortitis or periaortitis, it is reasonable to consider the diagnosis of IgG4-RSD in any patient with aortitis or periaortitis. The background of AIP and IgG4-SC, as well as the CT finding of ring-form wall thickening of the abdominal aorta suggested our case could be diagnosed as IgG4-related abdominal periaortitis, although no pathological confirmation was acquired. In conclusion, IgG4-RSD is a newly recognized disorder. Although it has been widely accepted, much remains unknown about its natural history and prognosis. While spontaneous improvement occurs in a significant number of patients, substantial percentage of patients relapse over time or have other organ involvement. Some patients may even suffer from permanent organ dysfunction such as liver cirrhosis, portal hypertension and aortic dissection.25 It appears to be a limited window of time during which current therapies can be effective. The most important consideration for clinicians is to be aware of this new IgG4-RSD entity." @default.
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• W1600171623 title "IgG4-related sclerosing cholangitis with autoimmune pancreatitis and periaortitis: Case report and review of the literature" @default.
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