We thank Dr Efe and Associates for their comments regarding our report, “Uveitis in Patients With Autoimmune Hepatitis,” which describes a consistent uveitis phenotype—chronic persistent bilateral uveitis—in 7 patients who also suffer from autoimmune hepatitis (AIH).

Efe and associates suggest the possibility that other immune-mediated diseases, in which hepatitis may also occur, might have explained uveitis in our patients. In particular, they suggest systemic lupus erythematosus or Sjogren syndrome should be considered as an underlying diagnosis in the patients in our series. We are confident that our patients have been correctly diagnosed, and that additional diagnoses have not been overlooked. As discussed in our report, AIH is a difficult diagnosis, and diagnostic criteria—relating to clinical features and results of investigations including liver histopathology—have been developed by an international consortium. We used these criteria to establish the diagnosis in 4 cases. Interestingly, 1 patient, in whom AIH was “definite” by the diagnostic criteria, was diagnosed with systemic lupus erythematosus prior to recognition that she suffered from AIH. In routine clinical practice, exhaustive testing may not be practical, and for 3 additional cases, information required to apply these criteria was not available. However, the authors of our report included 1 hepatologist (J.M.S.), who reviewed all cases and agreed with the diagnosis of AIH that had been made by the internist or pathologist involved in the management of these 3 patients. We excluded 2 cases because the diagnosis of AIH could not be substantiated.

The possibility of drug toxicity is raised by Efe and associates as an alternative explanation for the hepatitis observed in some of our patients. As highlighted in our paper, drug history is an important consideration when diagnosing AIH, but we do not believe that our patients suffer from hepatotoxicity related to the use of methotrexate or infliximab. According to the diagnostic criteria described above, negative points are applied in cases with a history of relevant drug use. Methotrexate does not attract these negative points because, although it causes hepatotoxicity, the presentation differs from that of AIH. The issue of methotrexate use in 3 individuals is discussed in our paper. The patient who was treated with infliximab received a single infusion for treatment of cystoid macular edema.

Efe and associates conclude that an association of AIH and uveitis “seems neither real nor coincidental,” and state that “it is difficult to draw a definitive conclusion” from our report. As acknowledged in the original publication, our study was small and retrospective, with selection bias, and without a population of reference. Factors supporting a real association are: the rare occurrence of both uveitis and AIH in the same individual; the consistent phenotype of the uveitis; and the immune-mediated basis of the 2 diseases. However, to quote from our publication: “Further studies are required to confirm this putative relationship.”