Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

Abstract

Vanishing bile duct syndrome (VBDS) is a rare disorder characterized by loss of interlobular bile ducts and progressive worsening cholestasis. The acute presentation of this disease is typically associated with a drug hypersensitivity and Stevens-Johnson syndrome/toxic epidermal necrolysis (TEN). The mainstay of treatment has been ursodeoxycholic acid with mixed results from immunosuppressive regimens. Anti–tumor necrosis factor-α and plasmapheresis have been speculated to be of potential benefit. It is hoped that early identification and intervention in VBDS secondary to Stevens-Johnson syndrome/TEN with continued reporting will lead to better regimens and outcomes. Our case report details the first reported use of infliximab and plasmapheresis, in addition to steroids, in a patient with VBDS secondary to TEN, as well as a literature review that supports a mechanism for why these modalities could be effective treatments. Unfortunately, our patient died, and the use of these therapies had an unclear benefit on his liver and skin disease. We hope that additional work can be published to confirm or refute their utility in the treatment of these diseases.

Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up

“…Cholestasis is a recognized complication of drug toxicity. However, there are few case reports of a chronic cholestatic complication of SJS/TEN known as vanishing bile duct syndrome (VBDS) . This is characterized by prolonged, progressive cholestasis associated histologically with the loss of more than 50% of interlobular bile ducts.…”

Successful liver transplantation for drug-induced vanishing bile duct syndrome

“…Ultimately, identification, withdrawal and avoidance of the offending drugs is key in the management of such cases. Some reports indicated the use of immunosuppression, tacrolimus, infliximab or even plasmapheresis may also be helpful 14–16. This could be an acceptable approach if the immune-allergic features dominate, but the evidence is scarce.…”

Clinical and pathogenic aspects of the severe cutaneous adverse reaction epidermal necrolysis (EN)

“…This syndrome is thought to be caused by hepatocellular necrosis and ischaemic hepatitis induced by fluid loss, but involvement of a TCR‐HLA interaction involving an antigen present in/on bile duct epithelium has also been hypothesized 133 . Hence, immunosuppression and TNFα blockers have been suggested as therapeutic options, but results have been inconsistent 170–172 . Of patients with acute kidney injury, 5% require long‐term dialysis 11 .…”