Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012

Background

IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progressive or malignant diseases.

Methods

The Research Committee of IgG4-related Diseases and the Research Committee of Intractable Diseases of Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare, Japan and the Japan Biliary Association have set up a working group consisting of researchers specializing in IgG4-SC, and established the new clinical diagnostic criteria of IgG4-SC 2012.

Results

The diagnosis of IgG4-SC is based on the combination of the following 4 criteria: (1) characteristic biliary imaging findings, (2) elevation of serum IgG4 concentrations, (3) the coexistence of IgG4-related diseases except those of the biliary tract, and (4) characteristic histopathological features. Furthermore, the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm accurate diagnosis of IgG4-SC.

Conclusion

These diagnostic criteria for IgG4-SC are useful in practice for general physicians and other nonspecialists.     

Autoimmune pancreatitis that developed over a 3-month observation period for IgG4-related dacryoadenitis and sialadenitis

Autoimmune pancreatitis (AIP) that developed in a short period in a patient with IgG4-related dacryoadenitis and sialadenitis is reported. A 67-year-old man was diagnosed as having IgG4-related dacryoadenitis and sialadenitis histologically, serologically, and radiologically. At that time, the pancreas was normal on computed tomographic scanning, magnetic resonance imaging, and 18F-fluorodeoxyglucose positron emission tomography. However, AIP occurred in the pancreatic body and tail during the 3-month period of observation of the initial diseases. All IgG4-related lesions improved promptly after steroid therapy. This report emphasizes that, since IgG4-related disease can develop in other organs within a short period in a patient with IgG4-related disease, periodic imaging follow-up is necessary.     

Chronic pancreatitis in the elderly in Japan.

BACKGROUND/AIM: Although the elderly comprise an increasingly large segment of the population, little has been written about chronic pancreatitis in this age group in Japan. In this study, we analyzed the clinical features of elderly Japanese patients with chronic pancreatitis and compared them with those of late-onset chronic pancreatitis patients in Western countries. METHODS: Subjects were 182 patients (162 males and 20 females) with chronic pancreatitis. They were divided into two groups: early-onset group (onset <65 years of age, n = 119) and late-onset group (onset > or =65 years of age, n = 63). Clinical findings and follow-up data were examined for each group. RESULTS: Alcohol abuse was the most common etiological factor in early-onset pancreatitis patients. In the late-onset group, the frequencies of idiopathic and autoimmune pancreatitis increased. Furthermore, the age at onset of autoimmune pancreatitis was >60 years in 94% of the cases. The late-onset group was more likely to have painless disease, and calcification of the pancreas and steatorrhea were rare in this group. Major causes of death were malignancy and malnutrition in each group. CONCLUSIONS: Early- and late-onset chronic pancreatitis showed different clinical features. Clinicians should consider autoimmune pancreatitis in the differential diagnosis in elderly patients with obstructive jaundice.     

Morphological changes after steroid therapy in autoimmune pancreatitis

BACKGROUND: Although many patients with autoimmune pancreatitis undergo steroid therapy, detailed evaluation of morphological changes in the pancreas and bile duct following therapy has not been performed in this disease. In this study serological and morphological changes occurring during steroid treatment of autoimmune pancreatitis are comparatively examined. METHODS: Ten patients with autoimmune pancreatitis were treated with corticosteroids. Morphological findings were: pancreatic enlargement (n = 9), irregular narrowing of the main pancreatic duct (n = 10), and biliary stenosis (n = 9). An initial dose of prednisolone was 40-30 mg/day, and this was tapered by 5 mg every 1-2 weeks. All patients underwent ultrasound and serological testing 1-2 weeks after commencing medication, followed by weekly serological testing and by CT and endoscopic retrograde cholangiopancreatography after 1-2 months. Radiological and serological changes were compared. RESULTS: All 10 patients were responsive to steroid therapy. Pancreatic size normalized within 1 month; however, irregularity of the pancreatic duct remained in 6 patients. Rigidity or lateral deformity of the bile duct remained in 3 patients and biliary stenosis persisted in 5. Four patients in whom elevated serum IgG4 failed to normalize also showed incomplete morphological improvement. Three patients with complete improvement of the pancreatic duct stopped medication, but recurrence of pancreatitis did not occur. CONCLUSIONS: Although steroid therapy was morphologically and serologically effective in patients with autoimmune pancreatitis, cholangiopancreatographic abnormalities remained in many patients. Morphological improvement on cholangiopancreatography and normalization of serum IgG4 after steroid therapy appeared to be good indicators for discontinuing medication in patients with autoimmune pancreatitis.     

Primary sclerosing cholangitis complicated with idiopathic thrombocytopenic purpura

We present a 66-year-old woman with primary sclerosing cholangitis (PSC) complicated with idiopathic thrombocytopenic purpura (ITP). Both PSC and ITP are considered to reflect an immunological disturbance. However, their coexistence is very rare and to the best of our knowledge this is only the second reported case. In Japan, PSC patients are rarely treated with liver transplantation. Fortunately, the present patient underwent successful hepatic transplantation from a brain-dead donor and simultaneous splenectomy. This case emphasizes the importance of liver transplantation as an effective treatment for primary sclerosing cholangitis.     

Recent Advances in the Diagnosis and Management of Autoimmune Pancreatitis: Similarities and Differences in Japan and Korea

Two subtypes (types 1 and 2) of autoimmune pancreatitis (AIP) are currently recognized. Type 1 AIP is related to immunoglobulin G4 (lymphoplasmacytic sclerosing pancreatitis), and type 2 AIP is characterized by neutrophilic infiltration into the epithelium of the pancreatic duct (idiopathic duct-centric pancreatitis). Although type 2 AIP is sometimes observed in the United States and Europe, most cases of AIP in Japan and Korea are type 1. The international consensus diagnostic criteria for AIP were created to be applicable worldwide and to distinguish between the two types of AIP. AIP is diagnosed based on the presence of at least one of the five cardinal features (i.e., imaging, serology, other organ involvement, histology, and response to steroid therapy). Oral steroids are the standard therapy for AIP, but immunomodulatory drugs or rituximab have been successfully used for patients with relapsed AIP in the United States and Europe. Generally, the clinical manifestations and demography of AIP are similar between Japan and Korea. However, there are differences in some aspects of the disease, including the proportion of other organ involvement, the prevalence of type 2 AIP, diagnostic criteria and maintenance therapy between the two countries.     

Therapeutic basis of glycyrrhizin on chronic hepatitis B

Glycyrrhizin, a major component of a herb (licorice), has been intravenously used for the treatment of chronic hepatitis B in Japan and improves liver function with occasional complete recovery from hepatitis. This substance modifies the intracellular transport and suppresses sialylation of hepatitis B virus (HBV) surface antigen (HBsAg) in vitro. This study was designed to clarify the pharmacological basis for its effectiveness. The structure-bioactivity relationship of glycyrrhizin, glycyrrhetic acid 3-O-monoglucuronide and glycyrrhetic acid was determined, and glycyrrhetic acid was found to be the most active of them. The amounts of three substances bound to the liver were evaluated in guinea pigs after intravenous administration of glycyrrhizin. Glycyrrhizin and glycyrrhetic acid 3-O-monoglucuronide were detected at concentrations of 31.8-1.3 μg/g of liver, but glycyrrhetic acid was not detected. When glycyrrhizin attained these concentrations in the cellular fraction of the PLC/PRF/5 cell culture, it suppressed the secretion of HBsAg as reported previously. These results indicated that glycyrrhizin administered intravenously might bind to hepatocytes at the concentration at which glycyrrhizin could modify the expression of HBV-related antigens on the hepatocytes and suppress sialylation of HBsAg.     

Invited. Ossification of posterior longitudinal ligaments: Evaluation with MRI

Ossification of the posterior longitudinal ligament is a special subcategory of degenerative disease responsible for compression of the spinal cord. On MR images, T2-weighted sequences are the most effective to evaluate both spinal cord compression due to the ossification and abnormal signal intensity of the cord. Although ossification of the ligaments is well demonstrated on CT and plain radiographs, MRI noninvasively provides useful information about the degree and extent of spinal cord compression, as well as the character of the ossification.     

A case of systemic lupus erythematosus with various central and peripheral neurological disorders developed with motor paralytic bladder as a major manifestation

A 29-year-old female with systemic lupus erythematosus who developed accerelated hypertension, motor paralytic bladder and various other neurological abnormalities is described. Cystometry demonstrated flaccid atonic neuropathic bladder. Elevated protein level and albuminocytologic dissociation were recognized in her cerebral spinal fluid. Magnetic resonance imaging study detected high signal intensities in themedulla oblongata andcauda equina. Her clinical symptoms and laboratory abnormalities were resolved after two courses of methyl-prednisolone pulse therapy. Possible roles of antiphospholipid antibodies were considered in the pathogenesis of her neurologic abnormalities.