scholarly journals IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

2015 ◽  
Vol 2015 ◽  
pp. 1-6
Author(s):  
Allon Kahn ◽  
Anitha D. Yadav ◽  
M. Edwyn Harrison
Keyword(s):  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Comprehensive Evaluation ◽  
Elevated Serum ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

scholarly journals Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kosuke Minaga ◽  
Tomohiro Watanabe ◽  
Akane Hara ◽  
Ken Kamata ◽  
Shunsuke Omoto ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Normal Serum ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

IgG4-related disease responder index but not serum IgG4 correlates with disease activity and the risk of 1-year relapse in type 1 autoimmune pancreatitis

Pancreatology ◽  
2019 ◽  
Vol 19 ◽  
pp. S135
Author(s):  
Filippo Vieceli ◽  
Giulia De Marchi ◽  
Antonio Amodio ◽  
Lorenzo Brozzi ◽  
Pietro Campagnola ◽  
...  
Keyword(s):  
Disease Activity ◽  
Autoimmune Pancreatitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

Immunostaining With Immunoglobulin G Subclass Antibody Cocktail for Diagnosis of Type 1 Autoimmune Pancreatitis

2020 ◽  
Vol 28 (8) ◽  
pp. 844-849
Author(s):  
Rie Nakata ◽  
Takeshi Uehara ◽  
Mai Iwaya ◽  
Shiho Asaka ◽  
Shota Kobayashi ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Tumor Formation ◽  
Mean Values ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis ◽  
Immunoglobulin G Subclass ◽  
Antibody Cocktail ◽  
Focal Tumor

Background. Immunoglobulin (Ig) G4-related diseases (RDs) are systemic diseases in which serum IgG4 levels are frequently elevated. They can cause diffuse or focal tumor formation, organ swelling, and tissue thickening in organs infiltrated by IgG4 + plasma cells. The diagnostic criteria for IgG4-RDs include an IgG4/IgG ratio >40%, but counting IgG+ cells can be difficult because of the weakness of IgG staining density. We hypothesized that an antibody cocktail of mixed IgG1, IgG2, IgG3, and IgG4 (AC-IgG) might give immunohistochemistry results comparable with those of IgG in IgG4-RD. Methods. We compared AC-IgG reactivity with IgG expression in type 1 autoimmune pancreatitis (AIP), a representative IgG4-RD. We compared immunohistochemistry results using AC-IgG and IgG-only in 10 cases of AIP. The coefficient of variation (Cv) was used to analyze differences between AC-IgG and IgG findings in AIP by 13 board-certified pathologists. Results. Although mean values for IgG+ cells did not significantly differ between AC-IgG (34.3; range = 27.4-37.1) and IgG (30.0; range = 23.0-45.6; P = .6254), Cv was lower for AC-IgG (33.4%) than for IgG (51.4%; regression equation; y[IgG] = 0.988 x + 0.982; correlation coefficient = 0.907). The data showed that the results of both methods were largely consistent. Conclusion. AC-IgG could replace IgG to count IgG+ cells because of its lower Cv.

scholarly journals Autoimmune Pancreatitis Type 1 Associated with a Pancreatic Pseudocyst

2019 ◽  
Vol 13 (1) ◽  
pp. 195-199
Author(s):  
Felix Hesse ◽  
Rickmer Braren ◽  
Roland M. Schmid ◽  
Veit Phillip
Keyword(s):  
Differential Diagnosis ◽  
Autoimmune Pancreatitis ◽  
Pancreatic Pseudocyst ◽  
Elevated Serum ◽  
Response To Treatment ◽  
Cystic Lesions ◽  
Serum Igg4 ◽  
Underlying Pathology ◽  
Tomography Scan

Pancreatic cystic lesions comprise diverse entities with different histopathological characteristics. Differential diagnosis is often challenging. Autoimmune pancreatitis (AIP) is usually not considered an underlying pathology in the differential diagnosis of peri-/pancreatic pseudo-/cystic lesions. We report the case of a 73-year-old male with diffuse pancreatic enlargement and an adjacent cystic lesion (60 × 80 mm) on computed tomography scan. Based on these imaging findings and an elevated serum IgG4 concentration, AIP complicated by a pancreatic pseudocyst was diagnosed, and treatment with glucocorticoids was started. Regular follow-ups showed a good response to treatment with regression of the pancreatic pseudocyst and remittent pancreatic swelling.

Simultaneous occurrence of autoimmune pancreatitis and sclerosing cholangitis as immune-related adverse events of pembrolizumab

2021 ◽  
Vol 14 (6) ◽  
pp. e243360
Author(s):  
Tsuyoshi Suda ◽  
Masako Kobayashi ◽  
Koji Kurokawa ◽  
Eiki Matsushita
Keyword(s):  
Bile Duct ◽  
Adverse Events ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Intrahepatic Bile Duct ◽  
Simultaneous Occurrence ◽  
Cd8 Cells ◽  
Igg4 Related Disease ◽  
Contrast Enhanced Ct ◽  
Serum Igg4

A 57-year-old man with lung cancer, previously treated with the programmed death-1 inhibitor pembrolizumab, was evaluated for liver injury and acute pancreatitis. Serum IgG4 levels were not elevated. Contrast-enhanced CT showed pancreatic swelling, contrast unevenness in the liver and thickening of the common bile duct and gall bladder. Magnetic resonance cholangial pancreatography revealed beads in the left intrahepatic bile duct and localised narrowing of the head and body of the central pancreatic duct. Endoscopic ultrasound-guided fine-needle and liver needle biopsy showed CD8+ and CD4+ T lymphocyte aggregates, whereas immunostaining revealed greater infiltration by CD8+ cells than CD4+ cells. IgG4-related disease was ruled out based on serum and pathological findings. The patient simultaneously presented with immune-related adverse events, autoimmune pancreatitis-like features and sclerosing cholangitis, which were ameliorated by steroid therapy. CD8+ lymphocytes were the dominant infiltrating cells in autoimmune pancreatitis and sclerosing cholangitis.

Intraductal Tubulopapillary Epithelial Proliferation Associated with Type 1 Autoimmune Pancreatitis

2018 ◽  
Vol 27 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Shinya Fujie ◽  
Hiroyuki Matsubayashi ◽  
Hirotoshi Ishiwatari ◽  
Hiromasa Hazama ◽  
Takaaki Ito ◽  
...  
Keyword(s):  
Pancreatic Duct ◽  
Autoimmune Pancreatitis ◽  
Fibrous Tissue ◽  
Ductal Adenocarcinoma ◽  
Epithelial Proliferation ◽  
Pancreatic Body ◽  
Gastric Type ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

A 70-year-old man was referred to our hospital with exacerbation of diabetes. His blood tests showed elevated levels of serum IgG4 and HbA1c. Computed tomography of the pancreatic body demonstrated a weakly enhanced mass, 2 cm in size, with indistinct borders. Magnetic resonance cholangiopancreatography revealed a narrowing of the main pancreatic duct (MPD) at the pancreatic body, a markedly dilated upstream duct, and a slightly dilated downstream duct. Endoscopic ultrasonography demonstrated an iso-hypoechoic heterogeneous mass, protruding and spreading in the pancreatic duct. The histology of a fine needle aspiration sample demonstrated fibrous tissue containing abundant IgG4-positive plasma cells and atypical epithelial cells. The imaging findings and histology were not typical for either pancreatic ductal adenocarcinoma or type 1 autoimmune pancreatitis (AIP), but these were not completely excluded, and a distal pancreatectomy was performed. Histological examination showed an intraductal tubulopapillary epithelial proliferation, which contained cytoplasmic mucin (MUC5AC and MUC6), and severe IgG4-positive lymphoplasmacytic infiltration in the interstitium around the MPD. Next-generation sequencing using DNA extracted from the tumor revealed no mutation of K-ras, GNAS, or TP53. The entire lesion was ultimately diagnosed as AIP with an intraductal tubular and papillary epithelial hyperplasia producing gastric-type mucin. Some recent reports have described AIP development in the background of intraductal papillary mucinous neoplasms, and some have hypothesized a paraneoplastic occurrence of IgG4-related disease. The current case indicates issues in the clinical diagnosis of rare variants of AIP, and raises questions about the relationship between AIP and pancreatic epithelial lesions.

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