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Clinical features and management of primary sclerosing cholangitis  [cached]
Marina G Silveira, Keith D Lindor
World Journal of Gastroenterology , 2008,
Abstract: Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in cirrhosis and need for liver transplantation and reduced life expectancy. The majority of cases occur in young and middle-aged men, often in association with inflammatory bowel disease. The etiology of primary sclerosing cholangitis includes immune-mediated components and elements of undefined nature. No effective medical therapy has been identified. The multiple complications of primary sclerosing cholangitis include metabolic bone disease, dominant strictures, bacterial cholangitis, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of primary sclerosing cholangitis. Liver transplantation is currently the only life-extending therapeutic alternative for patients with end-stage disease, although recurrence in the allografted liver has been described. A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass, prominence of plasma cells within the lesions, and steroid responsiveness.
Suspected idiopathic sclerosing orbital inflammation presenting as immunoglobulin G4-related disease: a case report
Kazuki Nagai, Kazuo Andoh, Noriko Nakamura, Katsumi Sakata
Journal of Medical Case Reports , 2011, DOI: 10.1186/1752-1947-5-427
Abstract: An 82-year-old Japanese woman had a 30-year history of chronic thyroiditis. She experienced right ptosis and eyelid swelling. These symptoms gradually developed over five years. The clinical and radiographic findings suggested that our patient had idiopathic sclerosing orbital inflammation. We were unable to obtain our patient's consent to perform a biopsy. While the serum immunoglobulin G level was within the normal limits, the serum immunoglobulin G4 level was significantly elevated. The serum immunoglobulin G4 levels decreased after the administration of oral prednisolone at a daily dose of 20 mg. In addition, the swelling and ptosis of the right upper eyelid disappeared gradually after four weeks. Our patient was then suspected to have idiopathic sclerosing orbital inflammation complicated with immunoglobulin G4-related disease and chronic thyroiditis.An orbital pseudotumor of this type is indicative of idiopathic sclerosing orbital inflammation immunoglobulin G4-related disease. Immunoglobulin G4 may thus be considered a subclass of immunoglobulin G when the serum immunoglobulin G level is within normal limits.Idiopathic sclerosing orbital inflammation (ISOI) is a rare, distinct subset of orbital inflammation, which is difficult to diagnose and manage. In addition, ISOI is an ill-defined heterogeneous entity. There are many reports on ISOI [1-4]. Early intervention with immunosuppression in the form of corticosteroids can result in the control and even regression of the disease. Various diseases that cause fibrotic changes in different systemic organs have been reported in cases of ISOI [5,6]. Multifocal fibrosclerosis or systemic idiopathic fibrosis occurs when orbital pseudotumor (OPT) is complicated by idiopathic retroperitoneal fibrosis (IRF), sclerosing cholangitis (SC) or Riedel's thyroiditis [7,8]. This disease group has also been collectively known as "fibrotic overlap syndrome" due to its excellent response to steroids [9,10]. On the other hand, serum
Retroperitoneal fibrosis associated with immunoglobulin G4-related disease  [cached]
Nao Fujimori,Tetsuhide Ito,Hisato Igarashi,Takamasa Oono
World Journal of Gastroenterology , 2013, DOI: 10.3748/wjg.v19.i1.35
Abstract: Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.
Inflammatory pseudotumors of the kidney and the lung presenting as immunoglobulin G4-related disease: a case report
Genya Nishikawa, Kogenta Nakamura, Yoshiaki Yamada, Takahiko Yoshizawa, Yoshiharu Kato, Remi Katsuda, Kenji Zennami, Motoi Tobiume, Shigeyuki Aoki, Tomohiro Taki, Nobuaki Honda
Journal of Medical Case Reports , 2011, DOI: 10.1186/1752-1947-5-480
Abstract: A 54-year-old Japanese man was referred to our hospital with suspected bilateral renal cancer, multiple lung metastases and autoimmune pancreatitis. His serum immunoglobulin G4 level was high. We used computed tomography-guided biopsies and histopathological examinations of the biopsied specimens to diagnose the tumors as immunoglobulin G4-related bilateral renal and lung inflammatory pseudotumors. Our patient was treated with oral prednisolone, and after one month of treatment, contrast-enhanced computed tomography demonstrated a general improvement, as noted by a reduction in size of the masses.Renal masses that are formed due to immunoglobulin G4-related disease require comprehensive diagnosis to prevent unnecessary surgical resections from being performed. Further consideration should be paid to immunoglobulin G4-related diseases in the future.Patients with autoimmune pancreatitis often exhibit high serum immunoglobulin G4 (IgG4) levels, and/or marked infiltration of IgG4-positive plasma cells, both of which are hallmarks of IgG4-related systemic disease. In addition to the pancreas, IgG4-related mass-forming lesions have also been described in other organs. In the kidney, IgG4-related disease can also present as an inflammatory mass, and is often diagnosed after a radical or partial nephrectomy due to suspected malignancy. Here, we present a case report of a patient with IgG4-related bilateral renal and lung inflammatory pseudotumors. We also describe the diagnostic process and treatment course.A 54-year-old Japanese man presented to a Department of Internal Medicine with chief complaints of dry mouth and weight loss that he had been experiencing for two months. He had type 1 diabetes mellitus, which was being treated with insulin. Computed tomography (CT) scans showed the presence of bilateral renal and pulmonary masses, and swelling of his pancreatic parenchyma. He was referred to our hospital with suspected bilateral renal cancer, multiple lung metastases an
An aortoduodenal fistula as a complication of immunoglobulin G4-related disease  [cached]
Momir Sarac,Ivan Marjanovic,Mihailo Bezmarevic,Uros Zoranovic
World Journal of Gastroenterology , 2012, DOI: 10.3748/wjg.v18.i42.6164
Abstract: Most primary aortoduodenal fistulas occur in the presence of an aortic aneurysm, which can be part of immunoglobulin G4 (IgG4)-related sclerosing disease. We present a case who underwent endovascular grafting of an aortoduodenal fistula associated with a high serum IgG4 level. A 56-year-old male underwent urgent endovascular reconstruction of an aortoduodenal fistula. The patient received antibiotics and other supportive therapy, and the postoperative course was uneventful, however, elevated levels of serum IgG, IgG4 and C-reactive protein were noted, which normalized after the introduction of steroid therapy. Control computed tomography angiography showed no endoleaks. The primary aortoduodenal fistula may have been associated with IgG4-related sclerosing disease as a possible complication of IgG4-related inflammatory aortic aneurysm. Endovascular grafting of a primary aortoduodenal fistula is an effective and minimally invasive alternative to standard surgical repair.
Autoimmune cholangitis mimicking a klatskin tumor: a case report
Alexandra Shingina, David Owen, Charles Zwirewich, Baljinder Salh
Journal of Medical Case Reports , 2011, DOI: 10.1186/1752-1947-5-485
Abstract: We report a case of a previously healthy 65-year-old man of Middle-Eastern origin, with a history of pancreatic insufficiency of unknown etiology, evaluated for elevated liver function tests found incidentally on a routine physical examination. Imaging studies revealed an atrophic pancreas and biliary duct dilatation consistent with obstruction. Subsequent endoscopic retrograde cholangiopancreatography showed a bile duct narrowing pattern suggestive of cholangiocarcinoma, but brushings failed to reveal malignant cells. Our patient proceeded to undergo surgical resection. Histological examination of the resected mass revealed lymphoplasmacytic infiltrate with no malignant features. Our patient returned three months later with persistently high liver function tests and no evidence of biliary obstruction on imaging. A presumptive diagnosis of autoimmune cholangitis was made and our patient's symptoms resolved after a short course of an oral steroid regimen. Post factum staining of the resection specimen revealed an immunoglobulin G4 antibody positive immune cell infiltrate, consistent with the proposed diagnosis.Our case thus highlights the importance of clinician awareness of the autoimmune spectrum of biliary pathologies when confronted with atypical clinical presentations, the paucity of diagnostic measures and the benefit from long-term steroid and/or immunosuppressive treatment.Autoimmune pancreatitis (AIP) was initially described by Yoshida and colleagues in 1995 to represent a type of chronic pancreatitis with characteristic histopathologic and imaging findings [1]. Based on clinical and laboratory features, two types of AIP are described in the literature. Type I AIP is thought be a pancreatic manifestation of immunoglobulin (Ig) G4-associated systemic disease characterized by lymphoplasmacytic infiltrate and serum elevation of IgG4 levels [2]. Type II AIP shows less correlation with high IgG4 levels, occurring in a younger population and typically characterize
Primary sclerosing cholangitis
Joy Worthington, Roger Chapman
Orphanet Journal of Rare Diseases , 2006, DOI: 10.1186/1750-1172-1-41
Abstract: Primary sclerosing cholangitis (PSC).Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of intra and/or extrahepatic bile ducts. A concentric obliterative fibrosis occurs which leads to bile duct strictures, which in turn can progress to biliary cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients [1,2].Secondary sclerosing cholangitis (SSC) is a sclerosing cholangitis that occurs secondary to a known pathogenic process or injury such as obstruction of the bile ducts due to tumour or gallstones; surgical, chemical or ischaemic injury; bacterial infections or congenital abnormalities. SSC has clinical features similar to those of PSC.Inflammatory bowel disease.Coeliac disease, diabetes mellitus, rheumatoid arthritis, Sjogren's disease, systemic sclerosis, chronic pancreatitis, cystic fibrosis, retroperitoneal fibrosis, sarcoidosis, thyroiditis, systemic lupus erythematosus (SLE), lupus nephritis, autoimmune haemolytic anaemia, idiopathic thrombocytopenic purpura, Langerhans cell histiocytosis, membranous nephropathy, Peyronie's disease, vasculitis, gallbladder disease.Colorectal cancer, cholangiocarcinoma.PSC occurs with a 2:1 male predominance. Little is known about the incidence and prevalence of PSC. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000, which appears to be increasing [3-5]. This probably reflects ascertainment bias rather than a true increase. Patients usually present in the third to fifth decade but PSC is recognised as a cause of chronic liver disease in children. It is also unusual to find more than one family member affected.PSC is frequently associated with inflammatory bowel disease (IBD), especially ulcerative colitis and, less often, with colonic Crohn's disease. Approximately three quarters of Caucasian patients with PSC have IBD [6,7] and 2–7.5% of patients with ulcerative colitis have PSC [8-10]. This may be an underestimate as patients
The G4 Genome  [PDF]
Nancy Maizels ,Lucas T. Gray
PLOS Genetics , 2013, DOI: 10.1371/journal.pgen.1003468
Abstract: Recent experiments provide fascinating examples of how G4 DNA and G4 RNA structures—aka quadruplexes—may contribute to normal biology and to genomic pathologies. Quadruplexes are transient and therefore difficult to identify directly in living cells, which initially caused skepticism regarding not only their biological relevance but even their existence. There is now compelling evidence for functions of some G4 motifs and the corresponding quadruplexes in essential processes, including initiation of DNA replication, telomere maintenance, regulated recombination in immune evasion and the immune response, control of gene expression, and genetic and epigenetic instability. Recognition and resolution of quadruplex structures is therefore an essential component of genome biology. We propose that G4 motifs and structures that participate in key processes compose the G4 genome, analogous to the transcriptome, proteome, or metabolome. This is a new view of the genome, which sees DNA as not only a simple alphabet but also a more complex geography. The challenge for the future is to systematically identify the G4 motifs that form quadruplexes in living cells and the features that confer on specific G4 motifs the ability to function as structural elements.
Eosinophilic Cholangitis and Cholangiopathy: A Sheep in Wolves Clothing  [PDF]
Catherine Nashed,Sujit Vijay Sakpal,Victoria Shusharina,Ronald Scott Chamberlain
HPB Surgery , 2010, DOI: 10.1155/2010/906496
Abstract: Background. Eosinophilic cholangitis (EC) is a rare benign disorder of the biliary tract which can cause biliary obstruction. Similar to other disease processes involving the bile ducts, this disorder can pose a difficult diagnostic challenge as it can mimic cholangiocarcinoma. Methods. A systematic search of the scientific literature was carried out using PubMed to access all publications related to EC. Search keywords that were utilized included “eosinophilic cholangitis,” “etiology,” “treatment,” and “obstructive jaundice.” Results. Twenty-three cases of EC have been reported. Nineteen patients (82.6%) who presented with EC remain disease-free; 15 of these 19 patients (78.9%) with followup time remain disease-free at a mean of 9.7 months (range, 2–24 months). Conclusion. EC is a rare form of biliary obstruction which can masquerade as a malignancy. Unlike cholangiocarcinoma, EC occurs more commonly in younger patients and in men. Most patients will require surgical treatment. 1. Introduction A variety of biliary conditions can mimic cholangiocarcinoma (CCA) and the precise pathologic distinction between benign and malignant causes of common hepatic duct (CHD), and proximal biliary tract obstruction remains a challenging clinical problem. In addition to malignant causes such as hilar CCA, lymphoma, gallbladder carcinoma, and metastases, benign causes of biliary obstruction include, but are not limited to: autoimmune pancreatitis-associated sclerosing cholangitis, extrahepatic primary sclerosing cholangitis (PSC), Mirizzi syndrome, inflammatory strictures secondary to choledocholithiasis, idiopathic benign focal stricture, and acquired immune deficiency syndrome cholangitis [1–6]. Approximately 10% of patients who undergo surgery for hepatic hilar strictures are found to have benign disease [1, 2]. Hadjis and colleagues initially coined the term “malignant masquerade” in 1985 to emphasize how benign entities can be difficult to distinguish from hilar CCA, in both clinical presentation and radiological appearance [1]. Eosinophilic cholangitis (EC) is an extremely rare benign disorder of the biliary tract which can result in biliary obstruction [3–5, 7, 8]. This disorder must be distinguished from CCA, which can be difficult since it is characterized by a dense transmural eosinophilic infiltration of the bile duct. A comprehensive literature review identified only 22 cases of EC [2–4, 8–24]. In this paper, we discuss an additional case involving a 33-year-old man who presented with obstructive jaundice and a mid-bile duct stricture (Bismuth-Corlette Type
Cholangiocarcinoma with respect to IgG4 Reaction  [PDF]
Kenichi Harada,Yasuni Nakanuma
International Journal of Hepatology , 2014, DOI: 10.1155/2014/803876
Abstract: IgG4 reactions marked by infiltration of IgG4-positive plasma cells in affected organs occur in cancer patients and in patients with IgG4-related diseases. Extrahepatic cholangiocarcinomas including gall bladder cancer are often accompanied by significant IgG4 reactions; these reactions show a negative correlation with CD8-positive cytotoxic T cells, suggesting that the evasion of immune surveillance is associated with cytotoxic T cells. The regulatory cytokine IL-10 may induce IgG4-positive plasma cell differentiation or promote B cell switching to IgG4 in the presence of IL-4. Cholangiocarcinoma cells may function as nonprofessional antigen presenting cells that indirectly induce IgG4 reactions via the IL-10-producing cells and/or these may act as Foxp3-positive and IL-10-producing cells that directly induce IgG4 reactions. Moreover, IgG4-related disease is a high-risk factor for cancer development; IgG4-related sclerosing cholangitis (IgG4-SC) cases associated with cholangiocarcinoma or its precursor lesion biliary intraepithelial neoplasia (BilIN) have been reported. IgG4-positive cell infiltration is an important finding of IgG4-SC but is not a histological hallmark of IgG4-SC. For the diagnosis of IgG4-SC, its differentiation from cholangiocarcinoma remains important. 1. Introduction Inflammatory biliary diseases with periductal fibrosis are categorized as sclerosing cholangitis. In addition to the prototype of sclerosing cholangitis, primary sclerosing cholangitis (PSC), IgG4-related sclerosing cholangitis (IgG4-SC) is categorized as sclerosing cholangitis. Although IgG4-SC is characterized by the infiltration of numerous IgG4-positive cells in the wall of bile ducts, this IgG4 reaction is also found in PSC, hepatolithiasis, and cholangiocarcinoma. In particular, the differentiation between IgG4-SC and cholangiocarcinoma is an important clinical issue. Moreover, carcinogenesis in IgG4-related diseases has been noted [1] and a few cholangiocarcinoma cases arising from IgG4-SC have also been reported [2, 3]. In this review, we focus on the IgG4 reaction in cholangiocarcinoma and the pathological IgG4-SC-induced carcinogenic features of cholangiocarcinoma. 2. IgG4-Related Diseases and Clinicopathological Issues IgG4 is a minor immunoglobulin subtype that does not activate complement and comprises only 3–6% of all circulating IgG in adults [4]. Elevated serum IgG4 levels and abundant IgG4-positive plasma cell infiltration in affected organs mark IgG4-related diseases [4–6]. The physiological and pathological significance of IgG4 remains unknown in
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