IgG4-related sclerosing disease

Based on histological and immunohistochemical exami-nation of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by ex-tensive IgG4-positive plasma cells and T-lymphocyte inf iltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, sali-vary gland, retroperitoneum, kidney, lung, and pros-tate, in which tissue fi brosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fi brosis, tubulointerstitial nephritis, intersti-tial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be as-sociated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in oth-ers, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related scleros-ing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

MRCP and MRI findings in 9 patients with autoimmune pancreatitis

瞄准：为了评估磁性的回声 cholangiopancreatography (MRCP ) ，与磁性的回声(先生) 一起的调查结果在自体免疫的胰腺炎(AIP ) 想象病人。方法：有 AIP 的九个病人经历了 MRI， MRCP，内视镜后退 cholangiopancreatography (ERCP ) ，计算断层摄影术，和 ultrasonography。在类固醇治疗前后拿的 MRCP 和先生图象被考察并且与另外的成像形式相比。AIP 盒子的 MRCP 调查结果与有胰的头的癌的 10 个盒子的那些相比。结果：在 MRCP 上，在 ERCP 上注意的主要的胰腺的管的缩小的部分没被设想，当主要的胰腺的管的非包含的片断被设想时。近似的主要的胰腺的管的在上游的膨胀的度比在胰腺的癌的情况下看温和。更低的胆汁管的狭窄或阻塞在 8 个病人被检测。先生图象在 T1 加权的先生图象上与减少的信号紧张显示出胰的增大， T2 加权的先生图象上的增加的信号紧张，并且，在 3 个病人，低亚硫酸钠强烈像囊的边界。在类固醇治疗以后，以前没设想主要的胰腺的管的部分被看见，与胆汁管狭窄的改进一起。胰腺的增大减少了，并且 T1 加权、 T2 加权的先生图象上的反常信号紧张成为了 isointense。结论：MRCP 不能区分从与胰腺的癌看见的主要的胰腺的管的狭窄与 AIP 看见的主要的胰腺的管的不规则的变窄。然而，与在 T1 加权、 T2 加权的先生图象上显示出反常信号紧张的胰腺的增大的先生成像一起的 MRCP 调查结果在支持 AIP 的诊断是有用的。

Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis:A prospective study using IgG4-immunostaining

瞄准：检验组织学并且免疫从自体免疫的胰腺炎(AIP ) 的主要十二指肠的乳头状的小突起拿的活体检视标本的组织化学的调查结果病人们。方法：在有 AIP 的 3 个病人并且 5 个控制病人的 resected 胰的主要十二指肠的乳头状的小突起[胰腺的癌(n = 3 ) 并且长期的含酒精的胰腺炎(n = 2 )] 免疫用 anti-CD4-T 房间， CD8-T 房间和 IgG4 抗体被染色。与怀疑的视神经乳头炎与 AIP 和 5 个控制病人从 2 个病人的主要十二指肠的乳头状的小突起拿的钳活体检视标本有希望地在组织化学地检验的十二指肠镜检查和免疫期间被拿。结果：包括许多 CD4 积极或 CD8 积极的 T 淋巴细胞和 IgG4 积极的血浆房间(】or=10/HPF ) 的中等或严重的 lymphoplasmacytic 渗入，与 AIP 在所有 3 个病人的主要十二指肠的乳头状的小突起被观察。一样的调查结果也在与 AIP 从 2 个病人的主要十二指肠的乳头状的小突起拿的活体检视标本，但是在控制被检测，仅仅有一些(【or=3/HPF ) 渗入主要十二指肠的乳头状的小突起的 IgG4 积极的血浆房间。结论：IgG4 积极的血浆房间的丰富的渗入明确地与 AIP 在病人的主要十二指肠的乳头状的小突起被检测。尽管这是初步的研究，从主要十二指肠的乳头状的小突起拿的活体检视标本的 IgG4-immunostaining 可以支持 AIP 的诊断。

Pancreatic cancer with a high serum IgG4 concentration

Differentiation between autoimmune pancreatitis and pancreatic cancer is sometimes difficult. It has been reported that serum IgG4 concentrations are significantly elevated and particularly high (>135 mg/dL) in autoimmune pancreatitis. Measurement of serum IgG4 has become a useful tool for differentiating between autoimmune pancreatitis and pancreatic cancer. However, we present a 74-year-old female with a markedly elevated serum IgG4 (433 mg/dL) who underwent pancreaticoduodenectomy for pancreatic cancer. Elevated serum IgG4 levels continued after the resection. On histology, adenocarcinoma of the pancreas accompanied with moderate lymphoplasmacytic infiltration infiltrated the lower bile duct and duodenum, but there were no findings of autoimmune pancreatitis. Although a small metastasis was detected in one parapancreatic lymph node, regional lymph nodes were swollen. Abundant IgG4-positive plasma cells infiltrated the cancerous areas of the pancreas, but only a few IgG4-positive plasma cells were detected in the noncancerous areas. Pancreatic cancer cells were not immunoreactive for IgG4. An abundant infiltration of IgG4-positive plasma cells was detected in the swollen regional lymph nodes and in the duodenal mucosa. We believe that the serum IgG4 level was elevated in this patient with pancreatic cancer as the result of an IgG4-related systemic disease that had no clinical manifestations other than lymphadenopathy.

Malignancies associated with intraductal papillary mucinous neoplasm of the pancreas

AIM: As intraductal papillary mucinous neoplasm (IPMN)has a favorable prognosis, associated malignancies have potential significance in these patients. We examined the incidence and characteristics of pre-existing, coexisting and subsequent malignancies in patients with IPMN. METHODS: Seventy-nine cases of IPMN were diagnosed by detection of mucous in the pancreatic duct during endoscopic retrograde pancreatography. Histological diagnosis was confirmed in 30 cases (adenoma (n = 19)and adenocarcinoma (n = 11). Other primary malignancies associated with IPMN, occurring in the prediagnostic or postdiagnostic period, were investigated. Postdiagnostic follow-up period was 3.3±0.5 years (range, 0.2-20 years).RESULTS: Other 40 malignancies occurred in 28 patients (35%). They were found before (n = 15), at (n = 19) and after (n = 6) the diagnosis of IPMT. Major associated malignancies were gastric cancer (n = 12), colonic cancer (n = 7), esophageal cancer (n = 4), pulmonary cancer (n = 4), and independent pancreatic cancer (n = 3).Pancreatic cancer was synchronous with IPMN in two patients and metachronous in one (3 years after diagnosis of IPMN). Thirty-one lesions were treated surgically or endoscopically. Fourteen patients died of associated cancers. Development of other malignancies was related to age (71.9±8.2 vs66.8±9.3, P＜0.05), but not to gender or site of the tumor.CONCLUSION: IPMN is associated with a high incidence of other malignancies, particularly gastric and colonic cancers. Common genetic mechanisms between IPMN and other associated malignancies might be present. Clinicians should pay attention to the possibility of associated malignancies in preoperative screening and follow-up of patients with IPMN.

Sclerosing cholecystitis associated with autoimmune pancreatitis

瞄准：与自体免疫的胰腺炎(AIP ) 在病人评估胆囊的组织病理学说、放射学的调查结果。方法：19 个 AIP 病人的胆囊的放射学的调查结果回顾地被考察。8 个 AIP 病人的 Resected 胆囊组织学地被检验，免疫与 anti-IgG4 抗体被染色。控制为征兆的胆石由胆囊 resected 组成了(n = 10 ) 并且那些为胰腺的癌在胰头十二指肠切除术期间搬迁了(n = 10 ) ，以及额外的肝的胆汁管和胰为胰腺的癌由胰头十二指肠切除术搬迁了(n = 10 ) 。结果：胆囊墙变厚被超声检测或与 AIP 在 10 个病人计算了断层摄影术(3 严重并且 7 中等) ；在这些病人，额外的肝的胆汁管的严重狭窄也被注意。组织学地，胆囊变厚在 8 中的 6 个被检测(75%) 有 AIP 的病人；4 个盒子与纤维变性有透壁 lymphoplasmacytic 渗入，并且 2 个盒子有基于 mucosal 的 lymphoplasmacytic 渗入。与稠密的纤维变性和弥漫的 lymphoplasmacytic 渗入的额外的肝的胆汁管墙的可观的透壁的变厚在 7 个病人被检测。Immunohistochemically， IgG4 积极的浆细胞的严重或中等的渗入在所有 8 个病人的胆囊，胆汁管，和胰被检测，但是没在控制被检测。结论：胆囊墙与纤维变性和 IgG4 积极的血浆房间的丰富的渗入变厚经常与 AIP 在病人被检测。我们建议一个新术语的使用，致硬化的胆汁，为这些盒子，那被象致硬化的胰腺炎或致硬化的胆管炎的一样的机制在 AIP 导致。

Thermo-chemo-radiotherapy for advanced bile duct carcinoma

AIM: Complete resection of the bile duct carcinoma is sometimes difficult by subepithelial spread in the duct wall or direct invasion of adjacent blood vessels. Nonresected extrahepatic bile duct carcinoma has a dismal prognosis,with a life expectancy of about 6 mo to 1 year. To improve the treatment results of locally advanced bile duct carcinoma, we have been conducting a clinical trial using regional hyperthermia in combination with chemoradiation therapy.METHODS: Eight patients complaining of obstructive jaundice with advanced extrahepatic bile duct underwent thermo-chemo-radiotherapy (TCRT). All tumors were located in the upper bile duct and involved hepatic bifurcation, and obstructed the bile duct completely.Radiofrequency capacitive hyperthermia was administered simultaneously with chemotherapeutic agents once weekly immediately following radiotherapy at 2 Gy.We administered heat to the patient for 40 min after the tumor temperature had risen to 42 ℃. The chemotherapeutic agents employed were cis-platinum (CDDP,50 mg/m2) in combination with 5-fluorouracil (5-FU,800 mg/m2) or methotrexate (MTX, 30 mg/m2) in combination with 5-FU (800 mg/m2). Number of heat treatments ranged from 2 to 8 sessions. The bile duct at autopsy was histologically examined in three patients treated with TCRT.RESULTS: In respect to resolution of the bile duct, there were three complete regression (CR), two partial regression (PR), and three no change (NC). Mean survival was 13.2±10.8 mo (mean±SD). Four patients survived for more than 20 mo. Percutaneous transhepatic biliary drainage (PTBD) tube could be removed in placement of self-expandable metallic stent into the patency-restored bile duct after TCRT. No major side effects occurred. At autopsy, marked hyalinization or fibrosis with necrosis replaced extensively bile duct tumor and wall, in which suppressed cohesiveness of carcinoma cells and degenerative cells were sparsely observed.CONCLUSION: Although the number of cases is rather small, TCRT in the treatment of locally advanced bile duct carcinoma is promising in raising local control and thus,long-term survival.