A 64-year-old woman was diagnosed by her ophthalmologist with acute zonal occult outer retinopathy (AZOOR) and referred for rheumatologic consultation.
The patient presented with blurred vision, worsening night vision, sensitivity to light and decreasing visual fields. Her ophthalmologist documented an “abnormal full-field electroretinogram with significant cone dysfunction and mild rod dysfunction in both eyes.” A detailed history and physical examination revealed no extraocular symptoms or signs. Extensive evaluation, including acute phase reactants and autoantibody testing, revealed no other abnormalities. Her ophthalmologist established the diagnosis of AZOOR and prescribed prednisone 50 mg daily. No comments on steroid-sparing agents or a prednisone taper were found in the ophthalmologist’s notes. The reason for referral was documented as “immunosuppression management.”
Dilemma
Patients with autoimmune or inflammatory ocular disease are often referred for rheumatologic consultation by their treating ophthalmologist. Ophthalmologists are often unfamiliar with the immunosuppressive medications used to manage these patients, and rheumatologists are often unfamiliar with the primary ocular diseases requiring immunosuppressive treatment. Managing these patients is difficult when the condition is rare and the medical literature provides minimal guidance. As of 2011, fewer than 150 cases of AZOOR had been reported in the English medical literature.1 Although AZOOR is described as an autoimmune retinopathy, the pathogenesis and the specific role of the immune system in this condition are unclear.
A lack of familiarity with these disorders makes members of both subspecialties hesitant to take ownership of such diseases as AZOOR. This raises a number of issues, both ethical and practical. The most pressing: How do we, as rheumatologists, define our moral and professional obligations to help a patient when their condition is not clearly within the scope of our discipline? Is it ethically appropriate to send the patient back to the referring physician without rheumatologic follow-up or even decline to see the patient if their condition is not one we consider to be “ours”? How can we best meet the needs of our patients when therapeutic roles are in question?
Discussion
Beyond the question of how to treat this patient, there is the question of who is ultimately responsible for making treatment decisions. This is especially challenging when there is no known effective treatment (as with AZOOR), the stakes are high (vision is on the line) and the physicians involved may never have seen a similar case. If there were a proven therapy, the providers’ roles might be clearer: The ophthalmologist could recommend a well-accepted treatment and monitor the ocular response, while the rheumatologist could monitor the safety of the chosen treatment.
