Year: 2024
Type: article
Abstract: syndrome To the Editor:We have read with great interest the article by Stoelinga et al. 1 on the diagnostic criteria and long-term outcomes in autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) variant syndrome under combination therapy, published in JHEP Reports.We are grateful to the authors for this novel piece of evidence on a quite enigmatic condition.We would like to offer some comments to highlight the challenges in studying such a difficult clinical phenotype.One of the most debated aspects of AIH-PBC management is whether Paris criteria should be used in clinical practice.2 Although the Paris criteria are highly specific, they may exclude patients with milder presentations.In other words, the relatively high cut-offs for AST, ALT, and IgG in these criteria tend to identify patients with severe PBC and AIH, potentially overlooking those with mild overlap manifestations, e.g.AIH with mild biliary damage or PBC with mild hepatitis, who would actually benefit from an effective combined treatment.3 In their work, Stoelinga et al. report that 55 out of 83 patients (76%) did not meet the Paris criteria; yet, clinicians still prescribed ursodeoxycholic acid along with immunosuppression to all these patients outside Paris criteria.Paris-positive and Parisnegative individuals were different on baseline histology, since the former had more liver inflammation and fibrosis.However, when evaluating treatment response at 12 months (i.e.complete biochemical response for the AIH component, Paris-II criteria for the PBC component), the rates were similar regardless of the Paris criteria status.This finding prompted the authors to conclude that, regardless of disease onset, biochemical response might not be affected by the Paris status.To us, these data seem to suggest that Paris criteria capture a more inflamed group of cases within a spectrum of disease, but without a significant consequence on treatment response.Notably, the authors recognize that the retrospective nature of the study only allows for inference of an association, since they could not exclude that differences in the treatment regimens were present.To further explore the phenotypic landscape of this syndrome, the authors presented a comparison between the previously mentioned 83 patients with AIH-PBC and 74 with a diagnosis of PBC associated with elevated ALT and/or IgG levels who were not considered as having AIH-PBC overlap by clinicians, and therefore not treated with a combination regimen.Unfortunately, no histological data were available on the latter cohort.We might speculate that the 74 patients with PBC and elevated IgG/ALT, who were not classified as having AIH-PBC overlap by clinicians, have de facto Paris criteria-negative AIH-PBC.
Source: JHEP Reports
Language: English
Topic: Liver Diseases and Immunity
Subfield: Hepatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,900
OpenAlex ID: W4402737582