Ig G4-related autoimmune pancreatitis is frequently accompanied by relevant lesions in the genitourinary tract and retroperitoneal organs, which cause various clinical problems, ranging from non-specific back pain or ...Ig G4-related autoimmune pancreatitis is frequently accompanied by relevant lesions in the genitourinary tract and retroperitoneal organs, which cause various clinical problems, ranging from non-specific back pain or bladder outlet obstruction to renal failure. The diagnosis of Ig G4-related retroperitoneal fibrosis requires a multidisciplinary approach, including serological tests, histological examination, imaging analysis, and susceptibility to steroid therapy. Radiological examinations are helpful to diagnose this condition, but surgical resection is occasionally unavoidable to exclude malignancy, particularly for patients with isolated retroperitoneal involvement. Steroid therapy is the treatment of choice for this condition, the same as for other manifestationsof Ig G4-related disease.For patients with severe ureteral obstruction,additional ureteral stenting needs to be considered prior to steroid therapy to preserve the renal function.Some papers have suggested that Ig G4-related disease can affect male reproductive organs including the prostate and testis.Ig G4-related prostatitis usually causes lower urinary tract symptoms,such as dysuria and pollakisuria.Patients sometimes state that corticosteroids given for Ig G4-related disease at other sites relieve their lower urinary tract symptoms,which leads us to suspect prostatic involvement in this condition.Because of the limited number of publications available,further studies are warranted to better characterize Ig G4-related disease in male reproductive organs.展开更多
A 61-year-old male from Northeast China presented with a 2-mo history of abdominal distension,pruritus and jaundice.Laboratory testing revealed an elevated serum Ig G4 level.A computed tomography scan showed a typical...A 61-year-old male from Northeast China presented with a 2-mo history of abdominal distension,pruritus and jaundice.Laboratory testing revealed an elevated serum Ig G4 level.A computed tomography scan showed a typical feature of autoimmune pancreatitis(AIP) and cholecystocholangitis.Early gastric cancer was incidentally discovered when endoscopic untrasound-guided fine needle aspiration(EUSFNA) of the pancreas was carried out.The patient underwent radical subtotal gastrectomy for gastric cancer combined with cholecystectomy.Helicobacter pylori(H.pylori) and Ig G4-positive plasmacytes were detected in gastric cancer tissue,pancreatic EUSFNA sample and resected gallbladder specimen by immunohistochemistry.The patient was diagnosed with H.pylori-positive Ig G4-related AIP and sclerosing cholecystocholangitis as well as H.pylori-positive gastric cancer.He responded well to steroid therapy and remains healthy with no signs of recurrence at one year follow-up.We speculate that H.pylori might act as a trigger via direct or indirect action in the initiation of onset of gastric cancer and multiorgan Ig G4-related disease.展开更多
Ig G4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected de...Ig G4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of Ig G4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the Ig G4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for Ig G4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to Ig G4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of Ig G4-related disease.展开更多
Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40...Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin(30.1 g/L, range: 35.0-55.0 g/L), serum potassium(2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose(2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h(range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG 4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.展开更多
AIM To assess the occurrence of autoimmune pancreatitis(AIP) in pancreatic resections performed for focal pancreatic enlargement.METHODS We performed a retrospective analysis of medical records of all patients who und...AIM To assess the occurrence of autoimmune pancreatitis(AIP) in pancreatic resections performed for focal pancreatic enlargement.METHODS We performed a retrospective analysis of medical records of all patients who underwent pancreatic resection for a focal pancreatic enlargement at our tertiary center from January 2000 to July 2013. The indication for surgery was suspicion of a tumor based on clinical presentation, imaging findings and laboratory evaluations. The diagnosis of AIP was based on histology findings. An experienced pathologist specialized in pancreatic disease reviewed all the cases and confirmed the diagnosis in pancreatic resection specimens suggestive of AIP. The histological diagnosis of AIP was set according to the international consensus diagnostic criteria.RESULTS Two hundred ninety-five pancreatic resections were performed in 201 men and 94 women. AIP was diagnosed in 15 patients(5.1%, 12 men and 3 women) based on histology of the resected specimen. Six of them had AIP type 1, nine were diagnosed with AIP type 2. Pancreatic adenocarcinoma(PC) was also present in six patients with AIP(40%), all six were men. Patients with AIP + PC were significantly older(60.5 vs 49 years of age, P = 0.045), more likely to have been recently diagnosed with diabetes(67% vs 11%, P = 0.09), and had experienced greater weight loss(15.5 kg vs 8.5 kg, P = 0.03) than AIP patients without PC. AIP was not diagnosed in any patients prior to surgery; however, the diagnostic algorithm was not fully completed in every case. CONCLUSION The possible co-occurrence of PC and AIP suggests that preoperative diagnosis of AIP does not rule out simultaneous presence of PC.展开更多
Type 1 autoimmune pancreatitis (AIP) or chronic sclerosing sialadenitis (Küttner’s tumour) is an uncommon disorder that has recently been confirmed as an IgG4-related disease. Here, we describe...Type 1 autoimmune pancreatitis (AIP) or chronic sclerosing sialadenitis (Küttner’s tumour) is an uncommon disorder that has recently been confirmed as an IgG4-related disease. Here, we describe a rare case of a 53-year-old male patient who primarily presented with pancreatic body mass, left neck mass and several lumps in his lower lip mimicking pancreatic cancer (PC) and neck metastasis. The patient underwent pancreatic body mass and labial gland lumps resection as well as an ultrasound-guided biopsy of the left neck mass. He was diagnosed with IgG4-related focal type of AIP (f-AIP) and Küttner’s tumour by immunohistochemistry. The patient responded well to corticosteroid therapy and remains healthy with no signs of recurrence at one year follow-up. The differentiation of f-AIP from PC is very important to avoid unnecessary pancreatic resection.展开更多
Antibody-mediated rejection(AMR) in liver transplantation has long been underestimated. The concept of the liver as an organ susceptible to AMR has emerged in recent years, not only in the context of the major histoco...Antibody-mediated rejection(AMR) in liver transplantation has long been underestimated. The concept of the liver as an organ susceptible to AMR has emerged in recent years, not only in the context of the major histocompatibility complex with the presence of HLA donor-specific antibodies, but also with antigens regarded as "minor", whose role in AMR has been demonstrated. Among them, antibodies against glutathione S-transferase T1 have been found in 100% of patients with de novo autoimmune hepatitis(dn AIH) when studied. In its latest update, the Banff Working Group for liver allograft pathology proposed replacing the term dn AIH with plasma cell(PC)-rich rejection. Antibodies to glutathione S-transferase T1(GSTT1) in null recipients of GSTT1 positive donors have been included as a contributory but nonessential feature of the diagnosis of PC-rich rejection. Also in this update, non-organ-specific anti-nuclear or smooth muscle autoantibodies are no longer included as diagnostic criteria. Although initially found in a proportion of patients with PC-rich rejection, the presence of autoantibodies is misleading since they are not diseasespecific and appear in many different contexts as bystanders. The cellular types and proportions of the inflammatory infiltrates in diagnostic biopsies have been studied in detail very recently. PC-rich rejection biopsies present a characteristic cellular profile with a predominance of T lymphocytes and a high proportion of PCs, close to 30%, of which 16.48% are Ig G4+. New data on the relevance of GSTT1-specific T lymphocytes to PC-rich rejection will be discussed in this review.展开更多
文摘Ig G4-related autoimmune pancreatitis is frequently accompanied by relevant lesions in the genitourinary tract and retroperitoneal organs, which cause various clinical problems, ranging from non-specific back pain or bladder outlet obstruction to renal failure. The diagnosis of Ig G4-related retroperitoneal fibrosis requires a multidisciplinary approach, including serological tests, histological examination, imaging analysis, and susceptibility to steroid therapy. Radiological examinations are helpful to diagnose this condition, but surgical resection is occasionally unavoidable to exclude malignancy, particularly for patients with isolated retroperitoneal involvement. Steroid therapy is the treatment of choice for this condition, the same as for other manifestationsof Ig G4-related disease.For patients with severe ureteral obstruction,additional ureteral stenting needs to be considered prior to steroid therapy to preserve the renal function.Some papers have suggested that Ig G4-related disease can affect male reproductive organs including the prostate and testis.Ig G4-related prostatitis usually causes lower urinary tract symptoms,such as dysuria and pollakisuria.Patients sometimes state that corticosteroids given for Ig G4-related disease at other sites relieve their lower urinary tract symptoms,which leads us to suspect prostatic involvement in this condition.Because of the limited number of publications available,further studies are warranted to better characterize Ig G4-related disease in male reproductive organs.
基金Supported by National Natural Scientific Foundation of China(to Gao RP),No.81070370 and No.81270544
文摘A 61-year-old male from Northeast China presented with a 2-mo history of abdominal distension,pruritus and jaundice.Laboratory testing revealed an elevated serum Ig G4 level.A computed tomography scan showed a typical feature of autoimmune pancreatitis(AIP) and cholecystocholangitis.Early gastric cancer was incidentally discovered when endoscopic untrasound-guided fine needle aspiration(EUSFNA) of the pancreas was carried out.The patient underwent radical subtotal gastrectomy for gastric cancer combined with cholecystectomy.Helicobacter pylori(H.pylori) and Ig G4-positive plasmacytes were detected in gastric cancer tissue,pancreatic EUSFNA sample and resected gallbladder specimen by immunohistochemistry.The patient was diagnosed with H.pylori-positive Ig G4-related AIP and sclerosing cholecystocholangitis as well as H.pylori-positive gastric cancer.He responded well to steroid therapy and remains healthy with no signs of recurrence at one year follow-up.We speculate that H.pylori might act as a trigger via direct or indirect action in the initiation of onset of gastric cancer and multiorgan Ig G4-related disease.
文摘Ig G4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of Ig G4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the Ig G4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for Ig G4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to Ig G4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of Ig G4-related disease.
文摘Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin(30.1 g/L, range: 35.0-55.0 g/L), serum potassium(2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose(2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h(range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG 4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.
文摘AIM To assess the occurrence of autoimmune pancreatitis(AIP) in pancreatic resections performed for focal pancreatic enlargement.METHODS We performed a retrospective analysis of medical records of all patients who underwent pancreatic resection for a focal pancreatic enlargement at our tertiary center from January 2000 to July 2013. The indication for surgery was suspicion of a tumor based on clinical presentation, imaging findings and laboratory evaluations. The diagnosis of AIP was based on histology findings. An experienced pathologist specialized in pancreatic disease reviewed all the cases and confirmed the diagnosis in pancreatic resection specimens suggestive of AIP. The histological diagnosis of AIP was set according to the international consensus diagnostic criteria.RESULTS Two hundred ninety-five pancreatic resections were performed in 201 men and 94 women. AIP was diagnosed in 15 patients(5.1%, 12 men and 3 women) based on histology of the resected specimen. Six of them had AIP type 1, nine were diagnosed with AIP type 2. Pancreatic adenocarcinoma(PC) was also present in six patients with AIP(40%), all six were men. Patients with AIP + PC were significantly older(60.5 vs 49 years of age, P = 0.045), more likely to have been recently diagnosed with diabetes(67% vs 11%, P = 0.09), and had experienced greater weight loss(15.5 kg vs 8.5 kg, P = 0.03) than AIP patients without PC. AIP was not diagnosed in any patients prior to surgery; however, the diagnostic algorithm was not fully completed in every case. CONCLUSION The possible co-occurrence of PC and AIP suggests that preoperative diagnosis of AIP does not rule out simultaneous presence of PC.
基金Supported by National Natural Scientific Foundation,No.81070370,and No.81270544 to Gao RPNational Institutes of Health,No.5R01AA016003 to Brigstock DR
文摘Type 1 autoimmune pancreatitis (AIP) or chronic sclerosing sialadenitis (Küttner’s tumour) is an uncommon disorder that has recently been confirmed as an IgG4-related disease. Here, we describe a rare case of a 53-year-old male patient who primarily presented with pancreatic body mass, left neck mass and several lumps in his lower lip mimicking pancreatic cancer (PC) and neck metastasis. The patient underwent pancreatic body mass and labial gland lumps resection as well as an ultrasound-guided biopsy of the left neck mass. He was diagnosed with IgG4-related focal type of AIP (f-AIP) and Küttner’s tumour by immunohistochemistry. The patient responded well to corticosteroid therapy and remains healthy with no signs of recurrence at one year follow-up. The differentiation of f-AIP from PC is very important to avoid unnecessary pancreatic resection.
基金Supported by Andalusian government,Proyecto de Excelencia,No.CTS-7846Spanish Ministry of Economy,Instituto de Salud Carlos Ⅲ,No.11/857 and No.17/1403
文摘Antibody-mediated rejection(AMR) in liver transplantation has long been underestimated. The concept of the liver as an organ susceptible to AMR has emerged in recent years, not only in the context of the major histocompatibility complex with the presence of HLA donor-specific antibodies, but also with antigens regarded as "minor", whose role in AMR has been demonstrated. Among them, antibodies against glutathione S-transferase T1 have been found in 100% of patients with de novo autoimmune hepatitis(dn AIH) when studied. In its latest update, the Banff Working Group for liver allograft pathology proposed replacing the term dn AIH with plasma cell(PC)-rich rejection. Antibodies to glutathione S-transferase T1(GSTT1) in null recipients of GSTT1 positive donors have been included as a contributory but nonessential feature of the diagnosis of PC-rich rejection. Also in this update, non-organ-specific anti-nuclear or smooth muscle autoantibodies are no longer included as diagnostic criteria. Although initially found in a proportion of patients with PC-rich rejection, the presence of autoantibodies is misleading since they are not diseasespecific and appear in many different contexts as bystanders. The cellular types and proportions of the inflammatory infiltrates in diagnostic biopsies have been studied in detail very recently. PC-rich rejection biopsies present a characteristic cellular profile with a predominance of T lymphocytes and a high proportion of PCs, close to 30%, of which 16.48% are Ig G4+. New data on the relevance of GSTT1-specific T lymphocytes to PC-rich rejection will be discussed in this review.
