Dubin-Johnson Syndrome Treatment & Management

Updated: Dec 15, 2022
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: BS Anand, MD  more...
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Approach Considerations

Dubin-Johnson syndrome (DJS) is a benign disorder and does not require any specific therapy, although patients should be warned that pregnancy, oral contraceptive use, administration of estrogens as a component of gender reassignment, and an intercurrent illness can exacerbate the associated jaundice.

In the past, patients were treated with phenobarbital, which was used primarily to reduce the serum bilirubin levels. This treatment is no longer recommended.

Rifampicin and ursodeoxycholic acid (UDCA) therapy have beneficial effects in chronic cholestatic diseases. These may result, in part, from the induction of MRP2 expression in the liver and kidney. However, neither an indication nor a general role for these agents in Dubin-Johnson syndrome has been defined. [2]

These drugs, in fact, should be used with caution in patients with Dubin-Johnson syndrome. Reporting on a patient with the disease who had a novel 974C→G nonsense mutation of the MRP2 gene, Corpechot et al found a rise in conjugated bilirubinemia following the chronic administration of rifampicin, as well as a sharp increase in serum bile acid concentration following the concomitant chronic administration of rifampicin and UDCA. [46] These adverse effects may result from an increased expression of MRP3 at the basolateral membrane of hepatocytes as a direct consequence of drug induction.

This observation suggests that these drugs should be used with caution in situations in which MRP2 expression may be decreased, as observed in the late stage of cholestasis. [46]

Patient education

Once diagnosed with Dubin-Johnson syndrome, patients should be informed of the disease process and its benign nature, and they should understand that no further investigative workup is required in the future.


The following consultations are indicated in Dubin-Johnson syndrome:

  • Gastroenterologist

  • Geneticist