Pbc and Ama—What Is the Connection?
1999; Lippincott Williams & Wilkins; Volume: 29; Issue: 1 Linguagem: Inglês
10.1002/hep.510290126
ISSN1527-3350
Autores Tópico(s)Pediatric Hepatobiliary Diseases and Treatments
ResumoPrimary Biliary Cirrhosis (PBC) is very closely associated with the presence of antimitochondrial antibodies (AMA); indeed, the presence of these antibodies is virtually disease specific.Despite the major advances made during the last decade in identifying the antigens with which these antibodies react, the reason for the close association remains uncertain.In this review, we examine the clinical correlates of AMA with features of PBC and discuss the possible implications of the association. CLINICAL CORRELATES Epidemiology of PBCThere is geographical variation in the prevalence of PBC, being highest in areas such as the north of England and parts of North America but rare in Africa and the Indian subcontinent. 1Studies from the north of England suggest that the incidence of PBC is increasing.Clustering of cases is well described. 2PBC may occur in families, and up to 4% of first-degree relatives may have PBC.Where PBC does occur in families, it may be related to maternally inherited factors and tends to present earlier in the second generation. 3 AMA Are Detectable Before the Clinical and Histological Features of PBC AMA Persist After Liver Transplantation But Do Not Correlate With Disease RecurrenceAfter transplantation, titers of AMA fall but soon return to pretransplant levels. 21The pattern of reactivity of the AMA
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