AUTOIMMUNE PANCREATITIS

Autoimmune Pancreatitis (AIP) is a new nosological entity that was first reported by Sarles et al. in 1961 and then named by Yoshida et al. in 1995 in Japan. It was then ignored by many Western researchers and now, in the last decade; it appears to have been recognized worldwide. AIP is a distinct form a chronic pancreatitis with an immune mediated fibroinflammatory process that has unique histopathologic features that makes it distinguishable from other forms of pancreatitis. Moreover, AIP is the only type of pancreatitis that responds to steroid administration. The Honolulu consensus document that has recently been published by Chari et al. described the histopathologic and clinical subtypes of AIP. Indeed, it appears that there are two forms of AIP, with different prevalence in Europe and Asia and distinct clinical profiles. The first subtype, the most common type in Asia, has recently been named Lymphoplasmocytic sclerosing pancreatitis (LPSP) or type I AIP because of its histological features and its association with elevated IgG serum levels and various autoantibodies. The second one is called idiopathic duct centric pancreatitis, IDCP, or type II AIP, that barely exists in Japan, but more accounted in Caucasian people. IDCP is recognized by its particular histology that is a granulocytic epithelial lesion (GEL) which makes some people call it AIP with GEL. Still nowadays, the diagnosis of AIP is a challenge. AIP can only be definitively diagnosed by histological examination. The main differential diagnosis of AIP is, except chronic pancreatitis, pancreatic cancer. That explains why there are still some unnecessary resections. Several groups have proposed diagnostic criteria for AIP as in Japan, Korea, Germany, Italy and the United States. Thus, it is important to find an international consensus. Above all, it is important to find new criteria as specific markers in the serum and the pancreatic tissues, for example using proteomics, to be able to diagnosis both types of AIP, and distinguish AIP from pancreatic cancer in order to avoid surgical resection in patients with AIP. The aim of this project is to review all relevant studies about AIP and to document all the available diagnostic tools.

Pediatric morphea (localized scleroderma): review of 136 patients.

BACKGROUND: Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement. OBJECTIVES: We sought to determine the clinical features of morphea in a large pediatric cohort. METHODS: We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. RESULTS: Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease. LIMITATIONS: Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations. CONCLUSIONS: These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.

Tumeurs fibroblastiques et myofibroblastiques de la tête et du cou [Fibroblastic and myofibroblastic tumors of the head and neck.]

Fibroblastic and myofibroblastic tumors of the head and neck are numerous and may develop either in adults or in childhood. They can be benign and nonrecurring, benign but locally recurring, of low-grade of malignancy or fully malignant. The diagnosis and treatment of these lesions can be difficult. This review focuses on several (myo)fibroblastic lesions of the head and neck, including nodular fasciitis and related neoplasms, hemangiopericytoma-like tumor (glomangiopericytoma) of sinonasal passages, nasopharyngeal angiofibroma, desmoid fibromatosis, Gardner-associated fibroma, extrapleural solitary fibrous tumor, inflammatory myofibroblastic tumor, low-grade myofibroblastic sarcoma, and adult-type fibrosarcoma.

Prevalence and significance of anti-HILA antibodies after liver transplantation : P236

Background: The pathogenic role of anti-HLA antibodies (AHA) after kidney transplantation is well established. However, its significance after liver transplantation remains unclear. The aim of our study was to determine the prevalence and significance of AHA after liver transplantation. Methods: Between January 2007 and November 2007, all liver transplant recipients who were greater than 6 months posttransplantation and followed regularly at our transplant outpatient clinic (n = 95) were screened for AHA. All clinical and electronic records were reviewed. Serum samples were tested using multiplex technology (Luminex). A liver biopsy had been performed in 55 out of the 95 patients based on clinical grounds but no routine protocol biopsies were performed. Immunosuppression was calcineurin inhibitor-based in 90 patients, sirolimus-based in 4 patients and one patient had no anti-rejection therapy (operationally tolerant recipient). Results: The mean time from transplantation to study was 85 months (range 6-248 months). Overall, AHA were found in 23/95 (24.2%) of patients (5 had anti-class I alone, 13 anti-class II alone, and 4 had both anti-class I and II). However, only 4/95 patients (4.2%) had donor-specific antibodies (DSA) (one anti-class I and 3 anti-class II). Twenty-one out of 95 patients (22.1%) had a history of past or current biopsy-proven or radiological biliary complications (chronic rejection, ischemic cholangitis, ischemic type biliary lesions or biliary anastomosis stricture). Among patients with AHA, 4/23 (17,4%) had biliary complications, while it was 17/72 (23.6%) in patients without AHA (NS). Among patients with DSA, 3/4 (75%) had biliary complications (two with biopsy-proven chronic rejection in association with biliary strictures and one with ischemic cholangitis following hepatic artery thrombosis), versus 1/19 (5.3%) patients with AHA but no DSA (p = 0.009), versus 16/72 (22.2%) patients without AHA (p = 0.046). In patients with DSA, immunosuppression was not different than in patients without DSA. Conclusions: We found a 24% AHA prevalence. The presence of DSA, but not of AHA, was significantly associated with an increased incidence of biliary complications including chronic liver allograft rejection. The exact mechanisms and possible causal relationship linking DSA to biliary complications remain to be studied. Larger prospective trials are thus needed to further define the role of AHA and in particular of DSA after liver transplantation.

Place actuelle de la papillotomie chirurgicale transduodénale [Current status of surgical transduodenal papillotomy].

The treatment of biliary lithiasis has changed during the past 20 years. Cholecystectomy remains the gold standard for cholelithiasis, but many options are available for calculi of the common bile duct. Among them are surgical open or laparoscopic choledochotomy, biliary-enteric anastomosis, transduodenal sphincterotomy (TDS), endoscopic sphincterotomy. With the aim to describe the current place of TDS, we reviewed the patients operated on in our department between 1976 and 1992. We found 78 patients with a mean age of 58 years (26-89 years). 34 (43%) of them had acute cholecystitis, with 26 being operated on urgently. 47 (60%) were jaundiced, 15 (19%) had pancreatitis and 12 (15%) had cholangitis before operation. Indications for TDS have been impacted stone or absence of progression of the contrast medium on intraoperative cholangiography in 71 patients (91%). 3 patients died (1 pulmonary embolism, 1 sepsis of pulmonary origin, 1 MOF syndrome complicating preoperative necrotizing pancreatitis). 30 patients (38%) had complications, of which 20 were directly related to TDS. Hemorrhage occurred in 4 cases, and resolved spontaneously without transfusion. Hyperamylasemia occurred in 17 instances, but clinical pancreatitis developed in only 1 case, with complete resolution. 1 duodenal fistula healed after conservative therapy. No death is attributable directly to TDS. Today, the importance of endoscopic sphincterotomy is increasing. This retrospective study shows that TDS, if performed with caution, does not increase the operative risks even in emergent operations. During surgical exploration of the common bile duct, TDS is indicated to remove an impacted stone, or as a bilio-enteric anastomosis if multiple stones are present with a thin common duct.(ABSTRACT TRUNCATED AT 250 WORDS)

Survival after surgical drainage of malignant pericardial effusion.

OBJECTIVES: Management of malignant pericardial effusion (PE) is complex. Cardiac surgeons are not necessarily familiar with or are challenged by the many underlying etiologies. Analyzing risk factors for mortality may help to estimate the benefit of surgery in high-risk patients. METHODS: Patients undergoing a surgical pericardiotomy for malignant PE, between 2001 and 2011, were included. The influence of tumor type, disease extension, intra-pericardial tumor infiltration on early mortality and long-term survival as well as freedom from symptoms after drainage, and the use of sclerosing agents on PE recurrence rates was analyzed. RESULTS: PE drainage was performed on 46 patients 12 ± 30 months after tumor diagnosis. Malignant diseases were lung cancers (50 %), breast cancers (15 %), lymphoma and leukemia (13 %), cancers of the digestive tract (13 %), and others (9 %). 80 % of patients were symptomatic and symptom relief was achieved in 65 %. Nobody died during surgery. Recurrence rate was 4 %. Early in-hospital mortality was 22 %. After 1 year, 29 % of patients were alive. Eleven patients (24 %) had a complete tumor regression. Metastatic spread (p < 0.001), pericardial infiltration (p = 0.02), and intra-pericardial Bleomycin (p = 0.01) injection were associated with increased mortality. Hematological malignancies had a better prognosis for survival. CONCLUSION: Surgical pericardiotomy is safe, associated with a low recurrence rate and symptom relief in the majority of dyspneic patients. Intra-pericardial Bleomycin may reduce recurrent effusion but does not ameliorate survival. Long-term survival rate was low with an increased mortality in cases of metastatic spreading, pericardial infiltration, and as the tumor of origin: breast cancers. Leukemic and lymphatic tumors have better prognosis.