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A Journal on Internal Medicine

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

language: English

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


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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