Guest guest Posted October 17, 2008 Report Share Posted October 17, 2008 Dear Elisabetta; There are several groups in Italy working on PSC and other autoimmune liver diseases. Here are some of the groups as listed in their recent publications: _____________________________________________ Mol Aspects Med. 2008 Feb-Apr;29(1-2):96-102. Autoimmune liver disease 2007. Muratori P, Granito A, Pappas G, Muratori L, Lenzi M, Bianchi FB Department of Internal Medicine, Cardioangiology, Hepatology, Alma Mater Studiorum-University of Bologna, Policlinico Sant'Orsola- Malpighi, via Massarenti 9, 40138 Bologna, Italy. Autoimmune liver disease (ALD) includes a spectrum of diseases which comprises both cholestatic and hepatitic forms: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and the so called " overlap " syndromes where hepatitic and cholestatic damage coexists. All these diseases are characterized by an extremely high heterogeneity of presentation, varying from asymptomatic, acute (as in a subset of AIH) or chronic (with aspecific symptoms such as fatigue and myalgia in AIH or fatigue and pruritus in PBC and PSC). The detection and characterization of non organ specific autoantibodies plays a major role in the diagnostic approach of autoimmune liver disease; anti nuclear reactivities (ANA) and anti smooth muscle antibodies (SMA) mark type 1 AIH, liver kidney microsomal antibody type 1 (LKM1) and liver cytosol type 1 (LC1) are the serological markers of type 2 AIH; antimitochondrial antibodies (AMA) are associated with PBC, while no specific marker is found in PSC, since anticytoplasmic neutrophil antibodies with perinuclear pattern (atypical p-ANCA or p-ANNA) are also detected in a substantial proportion of type 1 AIH cases. Treatment options rely on immunosoppressive therapy (steroids and azathioprine) in AIH and on ursodeoxycholic acid in cholestatic conditions; in all these diseases liver transplantation remains the only therapeutical approach for the end stage of liver disease. PMID: 18067956 _____________________________________________ Curr Med Chem. 2007;14(19):2081-94. Current treatments of primary sclerosing cholangitis. Vacca M, Krawczyk M, Petruzzelli M, Sasso RC, van Erpecum KJ, Palasciano G, van Berge-Henegouwen GP, Moschetta A, Portincasa P Clinica Medica " A. Murri " , Department of Internal Medicine and Public Medicine (DIMIMP), University of Bari, Italy. Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic disease characterized by hepatic inflammation and obliterative fibrosis, resulting in both intra- and extra-hepatic bile duct strictures. End- stage liver disease and bile duct carcinoma represent frequent complications. Incidence and prevalence of PSC in USA have been recently estimated as 0.9 per 100,000 person-years, and 1-6 per 100,000 person-years, respectively. Major diagnostic criteria include the presence of multifocal strictures, beadings of bile ducts, and compatible biochemical profile, once excluded secondary causes of cholangitis. Since the aetiology of PSC remains poorly defined, medical therapy is currently limited to symptom improvement and prolonged survival. Ursodeoxycholic acid (UDCA), corticosteroids and immunosuppressants have been proposed alone or in combination to improve the clinical outcome. In selected cases, surgical or endoscopic procedures need to be considered. Orthotopic liver transplantation (OLT) is at the moment the only definitive approach although disease relapse has been reported. In this article the state of the art in PSC treatment and future promises in this field are reviewed. PMID: 17691949. _____________________________________________ Am J Gastroenterol. 2005 Jul;100(7):1516-22. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L, Baldo V Department of Surgical and Gastroenterological Sciences, University of Padova, Padova, Italy. Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC. AIM: To evaluate the natural history of AIH/PSC overlap syndrome compared to a group of " classical " PSC. METHODS: Forty-one consecutive PSC patients, with a regular follow-up of at least 2 years, were prospectively included in the study. Among these, 7 fulfilled the criteria for AIH/PSC overlap syndrome. RESULTS: The AIH/PSC overlap group significantly differed from the " classical " PSC group in the following parameters: mean age at presentation (21.4 +/- 5.0 vs 32.3 +/- 10 years, p < 0.01), AST 191.0 +/- 14.8 vs 48.9 +/- 34.5 U/L, p < 0.005), ALT (357.0 +/- 26.5 vs 83.7 +/- 60.7 U/L, p < 0.005) and serum IgG (25.6 +/- 4.7 vs 12.9 +/- 6.0 mg/dl, p < 0.0001). The mean follow-up was similar in the 2 groups (93.3 +/- 65.9 vs 98.1 +/- 65.9 months respectively). Treatment included immunosuppression + ursodeoxycholic acid (UDCA) in the AIH/PSC overlap patients, and UDCA in the " classical " PSC group. Deaths were recorded only in the classical PSC group. The median survival in the latter group was 207 months (95% C.I. 87.6-326.4). The major events during the follow-up included: OLTx (1/7 vs 6/34), and neoplasms (only in the group of " classical " PSC). The new Mayo score prognostic index only increased significantly during follow-up in the " classical " PSC group (r2 0.8117, p < 0.01) CONCLUSION: Patients with AIH/PSC overlap syndrome seem to benefit from immunosuppression + UDCA therapy, survival is apparently better than in " classical " PSC condition. PMID: 15984974. _____________________________________________ Best regards, Dave (father of (23); PSC 07/03; UC 08/03) > > Dear PSC'ers, > do you know an italian hepatologist interesting in PSC? > Thank you > Elisabetta > Quote Link to comment Share on other sites More sharing options...
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