Letter: reversibility of hepatic fibrosis and immunosuppression withdrawal in patients with autoimmune hepatitis/primary biliary cirrhosis overlap syndrome - author's reply.

Abstract

The experience of Drs Yilmaz and Dayanan1 in patients with features of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) is similar to that in patients with classical AIH.2, 3 Liver inflammation is probably the principal driver of fibrosis in each of these clinical syndromes.4 As Drs Yilmaz and Dayanan suggest, the suppression of inflammation can be an effective anti-fibrotic strategy even when the inflammation is accompanied by features of PBC. Other studies of PBC support these observations.5, 6 The inflammatory features associated with AIH lack disease-specificity, and their occurrence in PBC has identified patients whose outcomes may be improved by management strategies that combine corticosteroids (with or without azathioprine) and ursodeoxycholic acid.7, 8 The experience of Drs Yilmaz and Dayanan suggests that immunosuppressive therapy alone may be effective as an anti-fibrotic regimen in some patients. Furthermore, outcomes may be better than in those with classical AIH or PBC. The overlap syndromes are clinical descriptions based largely on clinical judgments, and the AIH/PBC overlap syndrome described by Drs Yilmaz and Dayanan exists within a clinical spectrum that is bounded by patients with mainly AIH and weak manifestations of PBC and by patients with mainly PBC and inflammatory features reminiscent of AIH.8 Immunosuppressive therapy has a role across the spectrum, but outcomes may differ depending on the predominant component.8 The absence of relapse after drug withdrawal suggests that PBC may have been the predominant disease in this experience. The observations of Drs Yilmaz and Dayanan indicate that immunosuppressive therapy can have anti-fibrotic effects in immune-mediated liver diseases outside the classical phenotype of AIH. Future objectives must be to characterise those patients who are likely to respond, validate non-invasive methods to monitor fibrosis, and rigorously evaluate site-specific anti-fibrotic interventions that might supplement or replace current strategies.9 The author's declarations of personal and financial interests are unchanged from those in the original article.9