Hepatic metabisulfite sensitivity in a patient with sclerosing cholangitis.
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.
Stormont
James M
JM
University of Rochester School of Medicine and Dentistry, Rochester, New York, USA. stormymd@aol.com
Flaherty
Morgan
M
Condemi
John
J
D000284:Administration, Oral; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D015209:Cholangitis, Sclerosing / Q000209:etiology* / Q000628:therapy; D004035:Diet Therapy; D006505:Hepatitis / Q000097:blood / Q000150:complications*; D006801:Humans; D008099:Liver / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010919:Placebos; D013447:Sulfites / Q000008:administration & dosage / Q000633:toxicity*; D014805:Vitamin B 12 / Q000008:administration & dosage / Q000627:therapeutic use
D010919:Placebos; D013447:Sulfites; C030981:metabisulfite; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase; D014805:Vitamin B 12