Vanishing bile duct and Stevens-Johnson syndrome associated with ciprofloxacin treated with tacrolimus

Abstract

Stevens-Johnson syndrome (SJS) is a serious and potentially life-threatening disease. Vanishing bile duct syndrome (VBDS) is a rare cause of progressive cholestasis. Both syndromes are mostly related with drugs. We report a case of a patient with ciprofloxacin-induced SJS and acute onset of VBDS, and reviewed the related literature. It is the first case of ciprofloxacin-induced VBDS successfully treated with tacrolimus. This case reminds physicians of the importance of drug reactions, their severity, techniques for diagnosis and methods of management.

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

“…Tacrolimus has had mixed results. 1,3,4 We concluded that the risk/ benefit ratio for tacrolimus in our patient was too high, given his kidney and lung injury. Intravenous immunoglobulin has also been used in adults that developed VBDS secondary to graftversus-host disease and for a patient with VBDS secondary to TEN but was unable to prevent progression to liver transplant in those cases.…”

“…Refractory cases have used immunosuppression, most commonly the calcineurin inhibitor tacrolimus, with mixed results. 1,3,4 With increased knowledge of the pathophysiology, it has been suggested that tumor necrosis factor-a (TNF-a) inhibitors may represent an alternative therapy. 4 Our patient, a 6-year-old boy, represents the first reported use of a TNF-a inhibitor and plasmapheresis for treatment of VBDS associated with TEN.…”

“…1,3,4 With increased knowledge of the pathophysiology, it has been suggested that tumor necrosis factor-a (TNF-a) inhibitors may represent an alternative therapy. 4 Our patient, a 6-year-old boy, represents the first reported use of a TNF-a inhibitor and plasmapheresis for treatment of VBDS associated with TEN. We also summarized the presentation, management, and response to other therapies of patients with VBDS secondary to TEN.…”

“…These cases led to the hypothesis that VBDS can be caused by the same hyperimmune response that causes SJS/TEN. 1,3,4,7 Because of the limited cases, therapeutic interventions vary. Refractory cases have used immunosuppression, most commonly the calcineurin inhibitor tacrolimus, with mixed results.…”

“…2 Adult patients typically have concurrent liver diseases. [1][2][3][4][5][6] Pediatric case reports associate the development of VBDS with Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN; see Table 1). These cases led to the hypothesis that VBDS can be caused by the same hyperimmune response that causes SJS/TEN.…”