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Panminerva Medica 2002 December;44(4):313-23

lingua: Inglese

Primary sclerosing cholangitis

Cecere A., Tancredi L., Gattoni A.

Department of Clinical and Experimental Internal Medicine “F. Magrassi” 2nd Division General Medicine 2nd University of Naples, Naples, Italy


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To dis­cuss exhaus­tively: clin­ical, ser­o­log­ical and path­o­logic ­aspects of primary sclerosing cholangitis (PSC); ­recent advan­tages in the knowl­edge of the ­disease’s path­o­gen­esis, ther­a­peutic ­approaches ­that ­still ­appear ­today ­less pref­er­able ­than ­liver trans­plan­ta­tion. We ­have ­reviewed the ­most impor­tant ­researches on PSC of ­recent ­years. PSC is char­ac­ter­ized by ­chronic inflam­ma­tion of the ­main ­bile ­ducts, ­except for the gall­bladder. HLA-B8 and HLA-DR3 hap­lo­types ­have ­been asso­ciated ­with PSC. It is prob­able ­that PSC is an ­organ-spe­cific dis­ease. Some ­studies ­have ­shown the pres­ence of anti­bodies ­against the cyto­plasm of neu­trophils (­ANCA) in the ­serum of ­more ­than 50% of ­patients, and the pres­ence of anti­cat­a­lase anti­bodies in 60% of ­patients. ANCAs ­induce leu­ko­cyte meta­bolic acti­va­tion, ­that ­forms H2O2 and the ­anion super­oxide O-2, ­which in ­turn ­impair com­po­nents of ­both ­cell and cel­lular ­matrix. Catalase is ­mainly ­found in the ­liver; it is ­active in the “anti­ox­i­da­tive ­defense ­system” ­against ­toxic O2 metab­olites. Anticatalase anti­bodies are ­directed ­against an essen­tial ­region of cata­lases, by inhib­iting ­their enzy­matic func­tions. In ­these con­di­tions, a ­state of oxi­da­tive ­stress is deter­mined by imbal­anced ­ratio of oxi­da­tive to anti­ox­i­da­tive fac­tors, due to effec­tive pro­duc­tion of rad­ical spe­cies as ­well as to simul­ta­neous ­failure of anti­rad­ical ­defenses. Radical ­stress ­results in irre­ver­sible impair­ment of tis­sues. In PSC, pri­mary ­lesions ­seem to be sec­on­dary to the pro­duc­tion of rad­i­cals by ANCAs, depos­ited immu­no­com­plex, com­ple­men­tery pro­teins and bac­te­rial ­toxins. The sub­se­quent ­lysis of ­bile epi­the­lial ­cells ­seems to ­release cata­lases, ­that are ­thought to act as “non­self” ­once rec­og­nized by the immu­no­com­pe­tent ­system. Since the neo­an­tigen cat­a­lase ­seems to be ­highly ­expressed in PSC ­patients, it may ­easily asso­ciate ­itself ­with MHC mole­cules for pres­en­ta­tion to the ­immune ­system. Thus, in ­turn, B lym­pho­cytes ­would be ­forced to pro­duce anti­cat­a­lase anti­bodies. Since anti­cat­a­lase anti­bodies ­inhibit cata­lases, it can be hypoth­e­sized ­that ­they ­play a ­role in the path­o­gen­esis of PSC. From a ther­a­peutic view­point, the ­only effec­tive ­cure is trans­plan­ta­tion.

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