Diagnosis and treatment of autoimmune pancreatitis: experience with 100 patients

BACKGROUND： Autoimmune pancreatitis （ALP） is increasingly recognized as a unique subtype of pancreatitis. This study aimed to analyze the diagnosis and treatment of AIP patients from a tertiary care center in China. METHODS： One hundred patients with AIP who had been treated from January 2005 to December 2012 in our hospital were enrolled in this study. We retrospectively reviewed the data of clinical manifestations, laboratory tests, imaging examinations, pathological examinations, treatment and outcomes of the patients. RESULTS： The median age of the patients at onset was 57 years （range 23-82） with a male to female ratio of 8.1：1. The common manifestations of the patients included obstructive jaundice （49 patients, 49.0%）, abdominal pain （30, 30.0%）, and acute pancreatitis （11, 11.0%）. Biliary involvement was one of the most extrapancreatic manifestations （64, 64.0%）. Fifty-six （56.0%） and 43 （43.0%） patients were classified into focal- type and diffuse-type respectively according to the imaging examinations. The levels of serum IgG and IgG4 were elevated in 69.4% （43/62） and 92.0% （69/75） patients. Pathological analysis of specimens from 27 patients supported the diagnosis of lymphoplasmacytic sclerosing pancreatitis, and marked （〉10 ceUs/HPF） IgG4 positive cells were found in 20 （74.1%） patients.Steroid treatment and surgery as the main initial treatments were given to 41 （41.0%） and 28 （28.0%） patients, respectively. The remission rate after the initial treatment was 85.0%. Steroid was given as the treatment after relapse in most of the patients and the total remission rate at the end of follow-up was 96.0%. CONCLUSIONS： Clinical manifestations, laboratory tests, imaging and pathology examinations in combination could increase the diagnostic accuracy of AIP. Steroid treatment with an initial dose of 30 or 40 mg prednisone is effective and safe in most patients with AIP.

Steroids reduce local inflammatory mediator secretion and mucosal permeability in collagenous colitis patients

AIM：. To study the effect of oral steroids upon clinical response and rectal mucosa secretion of eosinophil cationic protein （ECP）, myeloperoxidase （NPO）, basic fibroblast growth factor （bFGF）, vascular endothelial growth factor （VEGF） and albumin in patients with collagenous colitis （CC）. METHODS： A segmental perfusion technique was used to collect perfusates from rectum of CC patients once before and twice （one and four weeks） after the start of steroid treatment. Clinical data was monitored and ECP, MPO, bFGF, VEGF and albumin concentrations were analyzed by immunochemical methods in perfusates and in serum. RESULTS： Steroids reduced the number of bowel movements by more than five times within one week and all patients reported improved subjective wellbeing at wk 1 and 4. At the same time, the median concentrations of ECP, bFGF, VEGF and albumin in rectal perfusates decreased significantly. MPO values were above the detection limit in only 3 patients before treatment and in none during treatment. VEGF, bFGF, ECP and albumin concentrations correlated with each other with the exception of ECP and albumin. A decrease of serum ECP and VEGF concentrations was also seen even if the overtime reduction was not significant.CONCLUSION： Oral steroid treatment in CC patients induced a simultaneous reduction of bowel movements and rectal release of ECP, bFGF, VEGF and albumin, suggesting that these polypeptides and increased mucosal permeability are important components of the pathophysiology in collagenous colitis.

Advances in the diagnosis and treatment of Ig G4-related sclerosing cholangitis: a review

Immunoglobulin G4-related sclerosing cholangitis （IgG4-SC） is an IgG4-related disease characterized by bile duct fibroinflammatory wall-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif- ferent regions of the bile duct can be involved, with the distal region being the most common. IgG4-SC can also have other organ involvement, such as the pancreas, urinary tract, salivary glands and lacrimal glands. In clinical practice, the manifestation of IgG4-SC is very similar to cholangiocarcinoma （CC） and primary sclerosing cholangiUs （PSC）, as well as pancreatic malignancies, while the treatment and prognosis are totally different. Japanese researchers ever established the clinical diagnostic criteria in 2012： （1） charac- teristic biliary imaging findings; （2） elevated serum IgG4 concentrations; （3） the coexistence of IgG4-related diseases except those of the biliary tract; and （4） characteristic histopathological features. According to our observations, IgG4-SC can be distinguished from CC with 100% specificity only at a cutoff of six times the upper normal limit. Imaging findings have low specificity for diagnosis, with the exception of intraductal ultrasonography, which can reflect the lesion with relatively high specificity. IgG4 plasma cell infiltration can be found in bile duct biopsy tissue, although this procedure is difficult. According to recent studies, the treatment of IgG4-SC relies mainly on corticosteroids. Following steroid treatment, most IgG4-SC patients can recover and their symptoms are resolved although a few patients relapse after steroid withdrawal. Maintenance of steroid therapy or alternative drugs is necessary in such cases. There is, however, no stronq evidence for malignant transformation in I qG4-SC.

Steroid receptors in endometrium and their dynamics after RU486 treatment

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Case Report:Granulomatous hypophysitis:two case reports and literature review

Granulomatous hypophysitis （GRH） is extremely rare and commonly presents with chronic inflammatory of the enlarged pituitary gland. In our study, 66-year-old and 57-year-old women, both Chinese, were diagnosed with GRH presenting preoperatively definite imageology characters as pituitary adenoma. The 66-year-old woman presented with a year of headache, half a year of gradual decrease of visual acuity, and one month of right ptosis. Serum prolaetin level was slightly elevated. Screening computed tomography （CT） scanning revealed typical low density mass found on the enlarged sella, which demonstrated invasive extension from the sella to the right cavernous sinus by contrast enhanced magnetic resonance imaging （MRI）. Consequently, the patient was diagnosed with probable invasive pituitary adenoma. The other 57-year-old woman complained a light headache and had been previously treated as nonfunctional pituitary adenoma in other hospital. Finally these two patients underwent transsphenoidal microsurgery and were diagnosed with GRH according to postoperative histopathology. They then were treated with steroid. During the follow-up, the clinical symptoms such as headache, visual damage, and ptosis vanished, and the mass of the sellae dramatically shrank on repeated MR images. Clinically and radiologically, GRH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Trans-sphenoidal surgery can decompress the optical nerve or oculomotornerve as a therapeutic strategy, and support biopsy or further pathological diagnosis. However, the hormonal therapy should be emphasized both as diagnostic and therapeutic strategies. Conservative and tentative steroid treatment should be performed in preoperative period without acute nerve damage.