Re: information Italy

[Guest guest]

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:

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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

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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.

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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.

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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

>