ABSTRACT
BACKGROUND: Primary sclerosing cholangitis is an uncommon chronic cholestatic liver disease with a poor prognosis in symptomatic cases. Genetic and immunologic alterations have been identified, and many possible etiologies have been entertained. Most treatments have limited benefit, and primary sclerosing cholangitis is a common cause for liver transplantation. OBJECTIVE: To describe a patient with documented primary sclerosing cholangitis associated with chronic ulcerative colitis, who developed hepatic toxicity following ingestion of metabisulfite. RESULTS: A placebo-controlled oral challenge suggested metabisulfite hypersensitivity with liver toxicity. He was treated with cobalamin (to prevent sulfite toxicity), low-sulfite diet, steroids, and antibiotics and has had an unusually benign course for 19 years. CONCLUSIONS: The hypersensitivity to oral metabisulfite in our patient appeared to be a significant trigger to flare-ups of his disease. Controlling the response to metabisulfite (along with recurrent antibiotic and steroid therapy) may have contributed significantly to the remarkably good outcome in this patient.
